1 Typical Optic Neuritis Optic Neuropathy Lots of

1 Typical Optic Neuritis Optic Neuropathy Lots of ways to divvy these up…

2 Typical Optic Neuritis Optic Neuropathy Inflammatory Lots of ways to divvy these up… this is just one. Noninflammatory

3 Typical Optic Neuritis Optic Neuropathy Inflammatory Lots of ways to divvy these up… Noninflammatory

4 Typical Optic Neuritis Optic Neuropathy Inflammatory Lots of ways to divvy these up… this is just one. Typical (demyelinating) Atypical Noninflammatory

5 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory

6 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune

7 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune

8 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic

9 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What is far-and-away the most common type of optic neuropathy? Hint… It’s not listed on this slide! Hint…It’s so common, it gets its own ophthalmic subspecialty! Glaucoma

10 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What is far-and-away the most common type of optic neuropathy? Hint…It’s not listed on this slide! Hint… It’s so common, it gets its own ophthalmic subspecialty! Glaucoma

11 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What is far-and-away the most common type of optic neuropathy? Hint…It’s not listed on this slide! Hint…It’s so common, it gets its own ophthalmic subspecialty! It’s… Glaucoma

12 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What is far-and-away the most common type of optic neuropathy? Hint…It’s not listed on this slide! Hint…It’s so common, it gets its own ophthalmic subspecialty! It’s…Glaucoma (don’t forget—glaucoma is an optic neuropathy!)

13 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What exam finding is the sine qua non of unilateral or asymmetric bilateral optic neuropathy? A relative afferent pupillary defect (RAPD)

14 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What exam finding is the sine qua non of unilateral or asymmetric bilateral optic neuropathy? A relative afferent pupillary defect (RAPD)

15 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What exam finding is the sine qua non of unilateral or asymmetric bilateral optic neuropathy? A relative afferent pupillary defect (RAPD) What should you do if a presumptive unilateral/asymmetric bilateral ON pt doesn’t have an RAPD? You should question the diagnosis

16 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What exam finding is the sine qua non of unilateral or asymmetric bilateral optic neuropathy? A relative afferent pupillary defect (RAPD) What should you do if a presumptive unilateral/asymmetric bilateral ON pt doesn’t have an RAPD? You should question the diagnosis

17 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What functional abnormalities are likely to be found in a pt with an optic neuropathy? two words --Decreased central acuity two diff words --Abnormal visual fields two diff words --Impaired color vision

18 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic What functional abnormalities are likely to be found in a pt with an optic neuropathy? --Decreased central acuity --Abnormal visual fields --Impaired color vision

19 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Name 3 infectious causes of atypical ON: 1) Lues (syphilis) 2) TB 3) Lyme (There are many others, of course) Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic

20 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Name 3 infectious causes of atypical ON: 1) Lues (syphilis) 2) TB 3) Lyme (There are many others, of course) Immune Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic

21 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Ischemic Compressive Atypical Infectious Immune Toxic/nutritional Congenital/hereditary Traumatic Name 3 immune-related causes of atypical ON: 1) Sarcoid 2) SLE 3) Wegener’s (There are many others, of course)

22 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Ischemic Compressive Atypical Infectious Immune Toxic/nutritional Congenital/hereditary Traumatic Name 3 immune-related causes of atypical ON: 1) Sarcoid 2) SLE 3) Wegener’s (There are many others, of course)

23 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Ischemic Who is the typical ‘typical optic neuropathy’ patient? Compressive A female age 15 - 45 Atypical Toxic/nutritional What percentage of typical/demyelinating ON pts are female? Congenital/hereditary About 75% Immune Infectious Traumatic

24 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Ischemic Who is the typical ‘typical optic neuropathy’ patient? Compressive A female age 15 - 45 Atypical Toxic/nutritional What percentage of typical/demyelinating ON pts are female? Congenital/hereditary About 75% Immune Infectious Traumatic

25 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Ischemic Who is the typical ‘typical optic neuropathy’ patient? Compressive A female age 15 - 45 Atypical Toxic/nutritional What should you do if a presumptive demyelinating ON pt doesn’t fit this profile? What percentage of typical/demyelinating ON pts are female? You should question the diagnosis Congenital/hereditary About 75% Infectious Immune Traumatic

26 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Ischemic Who is the typical ‘typical optic neuropathy’ patient? Compressive A female age 15 - 45 Atypical Toxic/nutritional What should you do if a presumptive demyelinating ON pt doesn’t fit this profile? What percentage of typical/demyelinating ON pts are female? You should question the diagnosis Congenital/hereditary About 75% Infectious Immune Traumatic

27 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Ischemic Who is the typical ‘typical optic neuropathy’ patient? Compressive A female age 15 - 45 Atypical Toxic/nutritional What percentage of typical/demyelinating ON pts are female? Congenital/hereditary About 75% Immune Infectious Traumatic

28 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Ischemic Who is the typical ‘typical optic neuropathy’ patient? Compressive A female age 15 - 45 Atypical Toxic/nutritional What percentage of typical/demyelinating ON pts are female? Congenital/hereditary About 75% Immune Infectious Traumatic

29 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune With what disease is demyelinating optic neuropathy associated? Multiple sclerosis (MS) Is it always accompanied or followed by MS? No, but the lifetime risk of developing MS is high Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic

30 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune With what disease is demyelinating optic neuropathy associated? Multiple sclerosis (MS) Is it always accompanied or followed by MS? No, but the lifetime risk of developing MS is high Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic

31 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune With what disease is demyelinating optic neuropathy associated? Multiple sclerosis (MS) Is it always accompanied or followed by MS? No, but the lifetime risk of developing MS is high Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic

32 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Infectious Immune With what disease is demyelinating optic neuropathy associated? Multiple sclerosis (MS) Is it always accompanied or followed by MS? No, but the lifetime risk of developing MS is high Ischemic Compressive Toxic/nutritional Congenital/hereditary Traumatic

33 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Unilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional How profound is the vision loss? Congenital/hereditary The vision can be anywhere from 20/20 to NLP; however, most Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

34 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Unilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional How profound is the vision loss? Congenital/hereditary The vision can be anywhere from 20/20 to NLP; however, most Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

35 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional Howpercent profound is the vision loss? What of typical optic neuritis pts present with bilateral vision loss? Congenital/hereditary Thethan vision can be optic anywhere 20/20 bilaterally to NLP; however, most Less 1% of adult neuritisfrom pts present Immune Infectious are in the 20/40 – 20/200 range Traumatic There is a subpopulation of optic neuritis pts for whom bilateral presentation is the long-term visual prognosis? is What somewhat more common--what is that population? Very good—about 95% will be 20/40 or better at one year Children What should you do if spontaneous recovery fails to occur? You should question the diagnosis

36 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional Howpercent profound is the vision loss? What of typical optic neuritis pts present with bilateral vision loss? Congenital/hereditary Thethan vision can be optic anywhere 20/20 bilaterally to NLP; however, most Less 1% of adult neuritisfrom pts present Immune Infectious are in the 20/40 – 20/200 range Traumatic There is a subpopulation of optic neuritis pts for whom bilateral presentation is the long-term visual prognosis? is What somewhat more common--what is that population? Very good—about 95% will be 20/40 or better at one year Children What should you do if spontaneous recovery fails to occur? You should question the diagnosis

37 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional Howpercent profound is the vision loss? What of typical optic neuritis pts present with bilateral vision loss? Congenital/hereditary Thethan vision can be optic anywhere 20/20 bilaterally to NLP; however, most Less 1% of adult neuritisfrom pts present Immune Infectious are in the 20/40 – 20/200 range Traumatic There is a subpopulation of optic neuritis pts for whom bilateral presentation is the long-term visual prognosis? is What somewhat more common--what is that population? Very good—about 95% will be 20/40 or better at one year Children What should you do if spontaneous recovery fails to occur? You should question the diagnosis

38 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional Howpercent profound is the vision loss? What of typical optic neuritis pts present with bilateral vision loss? Congenital/hereditary Thethan vision can be optic anywhere 20/20 bilaterally to NLP; however, most Less 1% of adult neuritisfrom pts present Immune Infectious are in the 20/40 – 20/200 range Traumatic There is a subpopulation of optic neuritis pts for whom bilateral presentation is the long-term visual prognosis? is What somewhat more common--what is that population? Very good—about 95% will be 20/40 or better at one year Children What should you do if spontaneous recovery fails to occur? You should question the diagnosis

39 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Unilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional How profound is the vision loss? Congenital/hereditary The vision can be anywhere from 20/20 to NLP; however, most Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

40 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Unilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional How profound is the vision loss? Congenital/hereditary The vision can be anywhere from 20/20 to NLP; however, most Immune Infectious are in the 20/40 –to 20/200 range Snellen Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

41 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Unilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional How profound is the vision loss? Congenital/hereditary The vision can be anywhere from 20/20 to NLP; however, most Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

42 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Unilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional How profound is the vision loss? Congenital/hereditary The vision can be anywhere from 20/20 to NLP; however, most Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

43 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Unilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional How profound is the vision loss? Congenital/hereditary The vision can be anywhere from 20/20 to NLP; however, most Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

44 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Unilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional How profound is the vision loss? Congenital/hereditary The vision can be anywhere from 20/20 to NLP; however, most Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

45 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Unilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical Toxic/nutritional How profound is the vision loss? Congenital/hereditary The vision can be anywhere from 20/20 to NLP; however, most Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

46 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

47 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

48 Typical Optic Neuritis Optic Neuropathy NMO involves two specific disease processes occurring together. One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of the optic What isimportantly, the pattern other of vision loss in neuritis? Ischemic nerves and spinal cord (although aspects oftypical the optic Bilateral vision loss developing over a period of a few days, CNS an be affected—more shortly) Compressive with spontaneous recovery beginning a week or two later Typical Atypical (demyelinating) ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

49 Typical Optic Neuritis Optic Neuropathy NMO involves two specific disease processes occurring together. One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, the CNS an be affected—more shortly) Compressive with spontaneous recovery beginning a week or two later Typical Atypical (demyelinating) ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

50 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, the CNS an be affected—more shortly) Compressive with spontaneous recovery beginning a week or two later Typical Atypical (demyelinating) ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

51 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, the CNS an be affected—more shortly) Compressive with spontaneous recovery beginning a week or two later Typical Atypical (demyelinating) ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

52 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, How does transverse myelitis present radiologically? the CNS an be affected—more shortly) Compressive As longitudinally extensive spinal lesions withcord spontaneous recovery beginning a week or two later Typical Atypical ^ no Toxic/nutritional What diagnosis How (demyelinating) long are we talking about? How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary 2 -3 vertebral segments or so The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

53 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, How does transverse myelitis present radiologically? the CNS an be affected—more shortly) Compressive As longitudinally extensive spinal lesions withcord spontaneous recovery beginning a week or two later Typical Atypical ^ no Toxic/nutritional What diagnosis How (demyelinating) long are we talking about? How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary 2 -3 vertebral segments or so The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

54 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, How does transverse myelitis present radiologically? the CNS an be affected—more shortly) Compressive As longitudinally extensive spinal lesions withcord spontaneous recovery beginning a week or two later Typical Atypical ^ no Toxic/nutritional What diagnosis How (demyelinating) long are we talking about? How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary 2 -3 vertebral segments or so The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

55 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, How does transverse myelitis present radiologically? the CNS an be affected—more shortly) Compressive As longitudinally extensive spinal lesions withcord spontaneous recovery beginning a week or two later Typical Atypical ^ no Toxic/nutritional What diagnosis How (demyelinating) long are we talking about? How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary 2 -3 vertebral segments or so The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

56 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? So, the spinal cord lesions in NMO are longitudinally extensive…Perchance, Transverse myelitis. NMO isisprimarily disease of ‘the optic the same a true of the optic nerve in NMO? What is importantly, the pattern of vision loss lesions inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Indeed it is! Further, optic nerve lesions in NMO tend more posterior Bilateral vision loss developing over a period oftoabe few days, How does transverse myelitis present radiologically? the CNS an be affected—more shortly) than those found in typical optic neuritis. Compressive As longitudinally extensive spinal cord lesions Inflammatory Noninflammatory with spontaneous recovery beginning a week or two later Typical ^no Atypical Toxic/nutritional How posterior arediagnosis we talking about here? What How (demyelinating) long are we talking about? How profound is the visionshould loss? be considered if optic neuritis They can extend to the optic chiasm, which isrecover almost unheard of in typical presents bilaterally and/or fails to spontaneously? Congenital/hereditary 2 -3 vertebral segments or so The vision can be anywhere from 20/20 to NLP; however, most optic neuritis Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

57 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? So, the spinal cord lesions in NMO are longitudinally extensive…Perchance, Transverse myelitis. NMO isisprimarily disease of ‘the optic the same a true of the optic nerve in NMO? What is importantly, the pattern of vision loss lesions inoftypical optic neuritis? anterior vs Ischemic nerves and spinal cord’ (although other aspects Indeed it is! Further, optic nerve lesions in NMO tend to be more posterior Bilateral vision loss developing over a period of a few days, posterior How does transverse myelitis present radiologically? the CNS an be affected—more shortly) than those found in typical optic neuritis. Compressive As longitudinally extensive spinal cord lesions Inflammatory Noninflammatory with spontaneous recovery beginning a week or two later Typical ^no Atypical Toxic/nutritional How posterior arediagnosis we talking about here? What How (demyelinating) long are we talking about? How profound is the visionshould loss? be considered if optic neuritis They can extend to the optic chiasm, which isrecover almost unheard of in typical presents bilaterally and/or fails to spontaneously? Congenital/hereditary 2 -3 vertebral segments or so The vision can be anywhere from 20/20 to NLP; however, most optic neuritis Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

58 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? So, the spinal cord lesions in NMO are longitudinally extensive…Perchance, Transverse myelitis. NMO isisprimarily disease of ‘the optic the same a true of the optic nerve in NMO? What is importantly, the pattern of vision loss lesions inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Indeed it is! Further, optic nerve lesions in NMO tend more posterior Bilateral vision loss developing over a period oftoabe few days, How does transverse myelitis present radiologically? the CNS an be affected—more shortly) than those found in typical optic neuritis. Compressive As longitudinally extensive spinal cord lesions Inflammatory Noninflammatory with spontaneous recovery beginning a week or two later Typical ^no Atypical Toxic/nutritional How posterior arediagnosis we talking about here? What How (demyelinating) long are we talking about? How profound is the visionshould loss? be considered if optic neuritis They can extend to the optic chiasm, which isrecover almost unheard of in typical presents bilaterally and/or fails to spontaneously? Congenital/hereditary 2 -3 vertebral segments or so The vision can be anywhere from 20/20 to NLP; however, most optic neuritis Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

59 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? So, the spinal cord lesions in NMO are longitudinally extensive…Perchance, Transverse myelitis. NMO isisprimarily disease of ‘the optic the same a true of the optic nerve in NMO? What is importantly, the pattern of vision loss lesions inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Indeed it is! Further, optic nerve lesions in NMO tend more posterior Bilateral vision loss developing over a period oftoabe few days, How does transverse myelitis present radiologically? the CNS an be affected—more shortly) than those found in typical optic neuritis. Compressive As longitudinally extensive spinal cord lesions Inflammatory Noninflammatory with spontaneous recovery beginning a week or two later Typical ^no Atypical Toxic/nutritional How posterior arediagnosis we talking about here? What How (demyelinating) long are we talking about? How profound is the visionshould loss? be considered if optic neuritis They can extend to the optic chiasm, which isrecover almost unheard of in typical presents bilaterally and/or fails to spontaneously? Congenital/hereditary 2 -3 vertebral segments or so The vision can be anywhere from 20/20 to NLP; however, most optic neuritis Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

60 Typical Optic Neuritis Optic Neuropathy How does transverse myelitis present clinically? As a symmetric para- or quadriparesis, often with sensory loss NMO involves two specific disease processes occurring together. (note that symmetric deficits are distinctly uncommon in MS) One is optic neuritis; what is the other? So, the spinal cord lesions in NMO are longitudinally extensive…Perchance, Transverse myelitis. NMO isisprimarily disease of ‘the optic the same a true of the optic nerve in NMO? What is importantly, the pattern of vision loss lesions inoftypical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Indeed it is! Further, optic nerve lesions in NMO tend more posterior Bilateral vision loss developing over a period oftoabe few days, How does transverse myelitis present radiologically? the CNS an be affected—more shortly) than those found in typical optic neuritis. Compressive As longitudinally extensive spinal cord lesions Inflammatory Noninflammatory with spontaneous recovery beginning a week or two later Typical ^no Atypical Toxic/nutritional How posterior arediagnosis we talking about here? What How (demyelinating) long are we talking about? How profound is the visionshould loss? be considered if optic neuritis They can extend to the optic chiasm, which isrecover almost unheard of in typical presents bilaterally and/or fails to spontaneously? Congenital/hereditary 2 -3 vertebral segments or so The vision can be anywhere from 20/20 to NLP; however, most optic neuritis Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

61 Typical Optic Neuritis Optic Neuropathy NMO involves two specific disease processes occurring together. One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss in of typical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, the CNS an be affected—more shortly) Compressive with spontaneous recovery beginning a week or two later Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range --Intractable… Traumatic --Intractable… What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

62 Typical Optic Neuritis Optic Neuropathy NMO involves two specific disease processes occurring together. One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss in of typical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, the CNS an be affected—more shortly) Compressive with spontaneous recovery beginning a week or two later Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups --Intractable…daytime somnolence What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

63 Typical Optic Neuritis Optic Neuropathy NMO involves two specific disease processes occurring together. One is optic neuritis; what is the other? Inflammatory Noninflammatory Transverse myelitis. NMO is primarily a disease of ‘the optic What is importantly, the pattern of vision loss in of typical optic neuritis? Ischemic nerves and spinal cord’ (although other aspects Bilateral vision loss developing over a period of a few days, the CNS an be affected—more shortly) Compressive with spontaneous recovery beginning a week or two later Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious note of these--they’re very important! are in the. Make 20/40 a–mental 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups They will clue you in to the fact you’re dealing with --Intractable…daytime somnolence What is the long-term visual prognosis? NMO rather than typical optic neuritis. Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

64 Typical Optic Neuritis Optic Neuropathy Does it really matter that much whether a pt has NMO vs typical optic neuritis? Very much so, for two reasons: Inflammatory Noninflammatory 1) The treatment regimens for the two diseases are different What is the pattern of NMO, vision loss in typical optic neuritis? 2) By correctly identifying and treating you might preclude the dreaded Ischemic Bilateral vision lossparalysis developing over adeath periodowing of a few days, sequelae of the disease, those being or even to the Compressive with(untreated spontaneous week or two later transverse myelitis NMOrecovery carries abeginning very higharisk of both) Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious note of these--they’re very important! are in the. Make 20/40 a–mental 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups They will clue you in to the fact you’re dealing with --Intractable…daytime somnolence What is the long-term visual prognosis? NMO rather than typical optic neuritis. Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

65 Typical Optic Neuritis Optic Neuropathy Does it really matter that much whether a pt has NMO vs typical optic neuritis? Very much so, for two reasons: Inflammatory Noninflammatory 1) The treatment regimens for the two diseases are different What is the pattern of NMO, vision loss in typical optic neuritis? 2) By correctly identifying and treating you might preclude the dreaded Ischemic Bilateral vision lossparalysis developing over adeath periodowing of a few days, sequelae of the disease, those being or even to the Compressive with(untreated spontaneous week or two later transverse myelitis NMOrecovery carries abeginning very higharisk of both) Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious note of these--they’re very important! are in the. Make 20/40 a–mental 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups They will clue you in to the fact you’re dealing with --Intractable…daytime somnolence What is the long-term visual prognosis? NMO rather than typical optic neuritis. Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

66 Typical Optic Neuritis Optic Neuropathy Does it really matter that much whether a pt has NMO vs typical optic neuritis? Very much so, for two reasons: Inflammatory Noninflammatory 1) The treatment regimens for the two diseases are different What is the pattern of NMO, vision loss in typical optic neuritis? 2) By correctly identifying and treating you might preclude the dreaded Ischemic Bilateral vision lossparalysis developing over adeath periodowing of a few days, sequelae of the disease, those being or even to the Compressive with(untreated spontaneous week or two later transverse myelitis NMOrecovery carries abeginning very higharisk of both) Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious note of these--they’re very important! are in the. Make 20/40 a–mental 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups They will clue you in to the fact you’re dealing with --Intractable…daytime somnolence What is the long-term visual prognosis? NMO rather than typical optic neuritis. Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

67 Typical Optic Neuritis Optic Neuropathy In general terms, what is the key difference in the treatment regimens? Typical optic neuritis is treated with immunomodulatory meds, whereas NMO is treated with immunosuppressive regimens So, it NMO to immunomodulatory meds? Does reallydoesn’t matterrespond that much whether a pt has NMO vs typical optic neuritis? Oh it responds all right--it gets worse Very much so, for two reasons: Inflammatory Noninflammatory 1) The treatment regimens for the two diseases are different What is the pattern of NMO, vision loss in typical optic neuritis? 2) By correctly identifying and treating you might preclude the dreaded Ischemic Bilateral vision lossparalysis developing over adeath periodowing of a few days, sequelae of the disease, those being or even to the Compressive with(untreated spontaneous week or two later transverse myelitis NMOrecovery carries abeginning very higharisk of both) Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious note of these--they’re very important! are in the. Make 20/40 a–mental 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups They will clue you in to the fact you’re dealing with --Intractable…daytime somnolence What is the long-term visual prognosis? NMO rather than typical optic neuritis. Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

68 Typical Optic Neuritis Optic Neuropathy In general terms, what is the key difference in the treatment regimens? Typical optic neuritis is treated with immunomodulatory meds, whereas NMO is treated with immunosuppressive regimens So, it NMO to immunomodulatory meds? Does reallydoesn’t matterrespond that much whether a pt has NMO vs typical optic neuritis? Oh it responds all right--it gets worse Very much so, for two reasons: Inflammatory Noninflammatory 1) The treatment regimens for the two diseases are different What is the pattern of NMO, vision loss in typical optic neuritis? 2) By correctly identifying and treating you might preclude the dreaded Ischemic Bilateral vision lossparalysis developing over adeath periodowing of a few days, sequelae of the disease, those being or even to the Compressive with(untreated spontaneous week or two later transverse myelitis NMOrecovery carries abeginning very higharisk of both) Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious note of these--they’re very important! are in the. Make 20/40 a–mental 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups They will clue you in to the fact you’re dealing with --Intractable…daytime somnolence What is the long-term visual prognosis? NMO rather than typical optic neuritis. Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

69 Typical Optic Neuritis Optic Neuropathy In general terms, what is the key difference in the treatment regimens? Typical optic neuritis is treated with immunomodulatory meds, whereas NMO is treated with immunosuppressive regimens So, it NMO to immunomodulatory meds? Does reallydoesn’t matterrespond that much whether a pt has NMO vs typical optic neuritis? Oh it responds all right--it gets worse Very much so, for two reasons: Inflammatory Noninflammatory 1) The treatment regimens for the two diseases are different What is the pattern of NMO, vision loss in typical optic neuritis? 2) By correctly identifying and treating you might preclude the dreaded Ischemic Bilateral vision lossparalysis developing over adeath periodowing of a few days, sequelae of the disease, those being or even to the Compressive with(untreated spontaneous week or two later transverse myelitis NMOrecovery carries abeginning very higharisk of both) Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious note of these--they’re very important! are in the. Make 20/40 a–mental 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups They will clue you in to the fact you’re dealing with --Intractable…daytime somnolence What is the long-term visual prognosis? NMO rather than typical optic neuritis. Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

70 Typical Optic Neuritis Optic Neuropathy In general terms, what is the key difference in the treatment regimens? Typical optic neuritis is treated with immunomodulatory meds, whereas NMO is treated with immunosuppressive regimens So, it NMO to immunomodulatory meds? Does reallydoesn’t matterrespond that much whether a pt has NMO vs typical optic neuritis? Oh it responds all right--it gets worse Very much so, for two reasons: Inflammatory Noninflammatory 1) The treatment regimens for the two diseases are different What is the pattern of NMO, vision loss in typical optic neuritis? 2) By correctly identifying and treating you might preclude the dreaded Ischemic Bilateral vision lossparalysis developing over adeath periodowing of a few days, sequelae of the disease, those being or even to the Compressive with(untreated spontaneous week or two later transverse myelitis NMOrecovery carries abeginning very higharisk of both) Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious note of these--they’re very important! are in the. Make 20/40 a–mental 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups They will clue you in to the fact you’re dealing with --Intractable…daytime somnolence What is the long-term visual prognosis? NMO rather than typical optic neuritis. Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

71 Typical Optic Neuritis Optic Neuropathy In general terms, what is the key difference in the treatment regimens? Typical optic neuritis is treated with immunomodulatory meds, whereas NMO is treated with immunosuppressive regimens So, it NMO to immunomodulatory meds? Does reallydoesn’t matterrespond that much whether a pt has NMO vs typical optic neuritis? Oh it responds all right--it gets worse Very much so, for two reasons: Inflammatory Noninflammatory 1) The treatment regimens for the two diseases are different What is the pattern of NMO, vision loss in typical optic neuritis? 2) By correctly identifying and treating you might preclude the dreaded Ischemic Bilateral vision lossparalysis developing over adeath periodowing of a few days, sequelae of the disease, those being or even to the Compressive with(untreated spontaneous week or two later transverse myelitis NMOrecovery carries abeginning very higharisk of both) Typical Atypical ^no affects ‘other aspects Time(demyelinating) for the ‘more shortly. ’ When NMO Toxic/nutritional What diagnosis should be considered if optic neuritis How profound is the vision loss? of the CNS, ’ one of three classic (but not pathognomonic!) presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary vision be anywhere 20/20 torecover NLP; however, most ‘intractable’ presentations may. The arise. What can are they? Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious note of these--they’re very important! are in the. Make 20/40 a–mental 20/200 range --Intractable…nausea and vomiting Traumatic --Intractable…hiccups They will clue you in to the fact you’re dealing with --Intractable…daytime somnolence What is the long-term visual prognosis? NMO rather than typical optic neuritis. Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

72 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic What is the target of the antibodies? Neuropathy The protein aquaporin-4 What does this protein do? It is the main water channel protein in astroglial cells. Damage to this protein interferes with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

73 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic What is the target of the antibodies? Neuropathy The protein aquaporin-4 What does this protein do? It is the main water channel protein in astroglial cells. Damage to this protein interferes with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

74 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic What is the target of the antibodies? Neuropathy The protein aquaporin-4 What does this protein do? It is the main water channel protein in astroglial cells. Damage to this protein interferes with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

75 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic What is the target of the antibodies? Neuropathy The protein aquaporin-4 word-# What does this protein do? It is the main water channel protein in astroglial cells. Damage to this protein interferes with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

76 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic What is the target of the antibodies? Neuropathy The protein aquaporin-4 What does this protein do? It is the main water channel protein in astroglial cells. Damage to this protein interferes with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

77 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic What is the target of the antibodies? Neuropathy The protein aquaporin-4 What does this protein do? It is the main water channel protein in astroglial cells. Damage to this protein interferes with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

78 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic What is the target of the antibodies? Neuropathy The protein aquaporin-4 What does this protein do? CNS cell type cells. Damage to this protein interferes It is the main water channel protein in astroglial with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

79 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic What is the target of the antibodies? Neuropathy The protein aquaporin-4 What does this protein do? It is the main water channel protein in astroglial cells. Damage to this protein interferes with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

80 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic Is lab testing available to detect these antibodies? What is the target of the antibodies? Neuropathy Yes, and they form part of the diagnostic criteria The protein aquaporin-4 for NMO What does this protein do? It is the main water channel protein in astroglial cells. Damage to this protein interferes with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

81 Typical Optic Neuritis In a few words, how would you characterize the pathological process in NMO? As an antibody-mediated autoimmune condition Optic Is lab testing available to detect these antibodies? What is the target of the antibodies? Neuropathy Yes, and they form part of the diagnostic criteria The protein aquaporin-4 for NMO What does this protein do? It is the main water channel protein in astroglial cells. Damage to this protein interferes with CNS fluid homeostasis. Inflammatory Typical (demyelinating) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

82 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) (next question) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis in NMO? Very good—about 95% will be 20/40 or better at one year What should you do if spontaneous recovery fails to occur? You should question the diagnosis

83 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) (next question) Noninflammatory What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Atypical ^no Toxic/nutritional What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis in NMO? Very good—about 95% will be 20/40 or better at one year ^ poor—vision <20/200 in at least one eye is the rule! What should you do if spontaneous recovery fails to occur? You should question the diagnosis

84 Typical Optic Neuritis (Warning: Soapbox speech ahead) Can typical optic neuritis present bilaterally? Yes. Can it be chronic? Yes. But you Optic (speaking to errbody who isn’t a fellowship-trained neuro-oph) shouldn’t make that call, Neuropathy because such cases are zebras, if not unicorns (or even zebracorns). So, don’t select ‘bilateral typical optic neuritis’ or ‘chronic typical optic neuritis’ as answers on the OKAP or WQEs, don’t utter those words when taking the Boards, and most importantly, Inflammatory Noninflammatory don’t write them on a pt’s chart! What is the pattern of vision loss in typical optic neuritis? Ischemic Bilateral vision loss developing over a period of a few days, Compressive with spontaneous recovery beginning a week or two later Typical Atypical ^no Toxic/nutritional (demyelinating) What diagnosis How profound is the visionshould loss? be considered if optic neuritis presents bilaterallyfrom and/or fails to spontaneously? Congenital/hereditary The vision can be anywhere 20/20 torecover NLP; however, most Neuromyelitis optica (NMO), aka Devic’s disease Immune Infectious are in the 20/40 – 20/200 range Traumatic What is the long-term visual prognosis in NMO? Very good—about 95% will be 20/40 or better at one year ^ poor—vision <20/200 in at least one eye is the rule! What should you do if spontaneous recovery fails to occur? You should question the diagnosis

85 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary What is the usual appearance of the ONH in typical optic neuritis? Immune Infectious Traumatic Benign—no edema is the rule. What should you do if a typical optic neuritis pt has florid disc edema? You should question the diagnosis

86 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary What is the usual appearance of the ONH in typical optic neuritis? Immune Infectious Benign—no edema is the rule. Or if present, mild edema w/o heme. Traumatic What should you do if a typical optic neuritis pt has florid disc edema? You should question the diagnosis

87 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary What is the usual appearance of the ONH in typical optic neuritis? Immune Infectious Benign—no edema is the rule. Or if present, mild edema w/o heme. Traumatic What should you do if a typical optic neuritis pt has florid disc edema? You should question the diagnosis

88 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary What is the usual appearance of the ONH in typical optic neuritis? Immune Infectious Benign—no edema is the rule. Or if present, mild edema w/o heme. Traumatic What should you do if a typical optic neuritis pt has florid disc edema? You should question the diagnosis

89 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary Is typical optic neuritis associated with ocular pain? Immune Infectious Yes. >90% will complain of pain, especially during ocular rotations. Traumatic What should you do if a typical optic neuritis pt does not c/o pain? You should question the diagnosis

90 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary Is typical optic neuritis associated with ocular pain? Immune Infectious Yes. >90% will complain of pain, especially during ocular rotations. Traumatic What should you do if a typical optic neuritis pt does not c/o pain? You should question the diagnosis

91 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary Is typical optic neuritis associated with ocular pain? Immune Infectious Yes. >90% will complain of pain, especially during ocular rotations. Traumatic What should you do if a typical optic neuritis pt does not c/o pain? You should question the diagnosis

92 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary Is typical optic neuritis associated with ocular pain? Immune Infectious Yes. >90% will complain of pain, especially during ocular rotations. Traumatic What should you do if a typical optic neuritis pt does not c/o pain? You should question the diagnosis

93 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary Is NMO optic neuritis associated with ocular pain? Immune Infectious Yes. >90% will complain of pain, especially during ocular rotations. Traumatic What about optic neuritis due to NMO--is it associated with pain? Yes, but much less so--only 1/3 will c/o ocular pain What should you do if a typical optic neuritis pt does not c/o pain? You should question the diagnosis

94 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary Is NMO optic neuritis associated with ocular pain? Immune Infectious Yes. >90% will complain of pain, especially during ocular rotations. Traumatic What about optic neuritis due to NMO--is it associated with pain? Yes, but much less so--only 1/3 will c/o ocular pain What should you do if a typical optic neuritis pt does not c/o pain? You should question the diagnosis

95 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Infectious With respect to treatment, what is the key difference between typical and atypical ON? Noninflammatory In atypical ON, treatment can influence final visual outcome; whereas in typical Ischemic ON, treatment has no effect on final Compressive visual outcome Toxic/nutritional Huh? What about the Optic Neuritis Treatment Trial. Congenital/hereditary (ONTT)? Didn’t it show Immune a benefit of IV steroids (and a deleterious Traumatic effect of PO steroids)? True and true. But the effect of IV steroids was to 1) hasten visual recovery and 2) delay onset of MS; the effect of PO steroids was to increase the risk of recurrent optic neuritis. Neither treatment had any effect on final visual outcome, or on the likelihood of developing MS.

96 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Infectious With respect to treatment, what is the key difference between typical and atypical ON? Noninflammatory In atypical ON, treatment can influence final visual outcome; whereas in typical Ischemic ON, treatment has no effect on final Compressive visual outcome Toxic/nutritional Huh? What about the Optic Neuritis Treatment Trial. Congenital/hereditary (ONTT)? Didn’t it show Immune a benefit of IV steroids (and a deleterious Traumatic effect of PO steroids)? True and true. But the effect of IV steroids was to 1) hasten visual recovery and 2) delay onset of MS; the effect of PO steroids was to increase the risk of recurrent optic neuritis. Neither treatment had any effect on final visual outcome, or on the likelihood of developing MS.

97 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Infectious With respect to treatment, what is the key difference between typical and atypical ON? Noninflammatory In atypical ON, treatment can influence final visual outcome; whereas in typical Ischemic ON, treatment has no effect on final Compressive visual outcome Toxic/nutritional Huh? What about the Optic Neuritis Treatment Trial. Congenital/hereditary (ONTT)? Didn’t it show Immune a benefit of IV steroids (and a deleterious Traumatic effect of PO steroids)? True and true. But the effect of IV steroids was to 1) hasten visual recovery and 2) delay onset of MS; the effect of PO steroids was to increase the risk of recurrent optic neuritis. Neither treatment had any effect on final visual outcome, or on the likelihood of developing MS.

98 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Infectious With respect to treatment, what is the key difference between typical and atypical ON? Noninflammatory In atypical ON, treatment can influence final visual outcome; whereas in typical Ischemic ON, treatment has no effect on final Compressive visual outcome Toxic/nutritional Huh? What about the Optic Neuritis Treatment Trial. Congenital/hereditary (ONTT)? Didn’t it show Immune a benefit of IV steroids (and a deleterious Traumatic effect of PO steroids)? True and true. But the effect of IV steroids was to 1) hasten visual recovery and 2) delay onset of MS; the effect of PO steroids was to increase the risk of recurrent optic neuritis. Neither treatment had any effect on final visual outcome, or on the likelihood of developing MS.

99 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical With respect to treatment, what is the key difference between typical and atypical ON? Noninflammatory In atypical ON, treatment can influence final visual outcome; whereas in typical Ischemic ON, treatment has no effect on final Compressive visual outcome Huh? What about the Optic Neuritis Treatment Trial (ONTT)? Didn’t it show Immune a benefit of IV steroids (and a deleterious Infectious effect of PO steroids)? True and true. But the effect of IV On the other hand, most infectious and immune steroidsprocesses was to 1) hasten visual recovery can be either cured or controlled via proper andtreatment! 2) delay onset of MS; the effect of PO steroids was to increase the risk of recurrent optic neuritis. Neither treatment had any effect on final visual outcome, or on the likelihood of developing MS.

100 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS What about lumbar puncture? It is not indicated

101 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS What about lumbar puncture? It is not indicated

102 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS What about lumbar puncture? It is not indicated

103 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS What about lumbar puncture? It is not indicated

104 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS What about lumbar puncture? It is not indicated

105 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Atypical Noninflammatory Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS What about lumbar puncture? It is not indicated

106 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: It is not indicated

107 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated

108 Typical Optic Neuritis Optic Neuropathy Inflammatory Noninflammatory With regard to the 25% of ON patients who went on to develop MS despite having no white-matter changes… Ischemic Which of the following characteristics were associated Compressive of developing MS? Typical with an increased risk. Atypical Severe ONH edema? Toxic/nutritional (demyelinating) Lack of pain? Congenital/hereditary VA = NLP? If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Macular exudates? Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated

109 Typical Optic Neuritis Optic Neuropathy Inflammatory Noninflammatory With regard to the 25% of ON patients who went on to develop MS despite having no white-matter changes… Ischemic Which of the following characteristics were associated Compressive of developing MS? Typical with an increased risk. Atypical Severe ONH edema? Toxic/nutritional (demyelinating) Lack of pain? All of these were associated with Congenital/hereditary VA = NLP? decreased risk of be developing MS If a pt presents with typical optic neuritis, what sort of workup should done? Immune Infectious Macular exudates? Traumatic NO! MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated a

110 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

111 Typical Optic Neuritis Optic Neuropathy Inflammatory Typical (demyelinating) Noninflammatory Atypical Ischemic Compressive Toxic/nutritional Congenital/hereditary If a pt presents with typical optic neuritis, what sort of workup should be done? Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

112 Typical Optic Neuritis Optic Neuropathy Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

113 Typical Optic Neuritis Optic Neuropathy Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

114 Typical Optic Neuritis Optic Neuropathy Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

115 Typical Optic Neuritis Optic Neuropathy Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

116 Typical Optic Neuritis Optic Neuropathy Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention S tudy What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

117 Typical Optic Neuritis Optic Neuropathy Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

118 Typical Optic Neuritis What is the generic name of Avonex? IFNb-1 a Optic Neuropathy In general terms, what sort of drug is Avonex? An immunomodulator What is its therapeutic benefit in ON patients with MRI changes? It reduces the risk of developing MS Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

119 Typical Optic Neuritis What is the generic name of Avonex? IFNb-1 a Optic Neuropathy In general terms, what sort of drug is Avonex? An immunomodulator What is its therapeutic benefit in ON patients with MRI changes? It reduces the risk of developing MS Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

120 Typical Optic Neuritis What is the generic name of Avonex? IFNb-1 a Optic Neuropathy In general terms, what sort of drug is Avonex? An immunomodulator What is its therapeutic benefit in ON patients with MRI changes? It reduces the risk of developing MS Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

121 Typical Optic Neuritis What is the generic name of Avonex? IFNb-1 a Optic Neuropathy In general terms, what sort of drug is Avonex? An immunomodulator What is its therapeutic benefit in ON patients with MRI changes? It reduces the risk of developing MS Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

122 Typical Optic Neuritis What is the generic name of Avonex? IFNb-1 a Optic Neuropathy In general terms, what sort of drug is Avonex? An immunomodulator What is its therapeutic benefit in ON patients with MRI changes? It reduces the risk of developing MS Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one

123 Typical Optic Neuritis What is the generic name of Avonex? IFNb-1 a Optic Neuropathy In general terms, what sort of drug is Avonex? An immunomodulator What is its therapeutic benefit in ON patients with MRI changes? It reduces the risk of developing MS Inflammatory Noninflammatory Ischemic Typical (demyelinating) Why is it important to know the probability of developing MS? Because it may influence the pt’s decision to. Compressive receive MS prophylaxis Atypical with the drug Avonex Toxic/nutritional What landmark study confirmed the ability of. Congenital/hereditary Avonex to reduce the If a pt presents with typical optic neuritis, what sort of workupevent? should be done? risk of developing MS after a demyelinating Immune Infectious Traumatic MRI brain and orbits, with contrast. That’s it. for Controlled High-risk Avonex Multiple The CHAMPS, which stands sclerosis Prevention Study What is the purpose of the MRI? To look for white-matter changes, the presence of which increases the likelihood of developing MS How many white matter lesions Probability of MS by 15 years if… …no white matter changes on MRI: 25% What about lumbar puncture? …white matter changes present: 72% It is not indicated have to be present to convey an increased risk of MS? Just one