SUBACUTE SCLEROSING PANENECPHALITIS Dr Amit Vatkar MBBS DCH

SUBACUTE SCLEROSING PANENECPHALITIS Dr. Amit Vatkar MBBS, DCH, DNB Pediatrics Fellow in Pediatric Neurology, Mumbai Trained in Neurophysiology & Epilepsy, USA Contact No. : +91 -8767844488 Email: vatkaramit@yahoo. com

HISTORY 10 year old male child Siva Normal child till 1 year back, • Had poor school performance for past 1 year • H/O difficulty in walking with frequent falls for past 1 month • c/o sudden jerky movements involving both upper limbs & lower limbs & bedridden for 7 days. • H/O jerks decreased in frequency during sleep. • Received treatment for above with anticonvulsants & referred here.

• No H/O fever / vomiting / altered sensorium. • No H/O dog bite/ recent vaccination. • No H/O visual or hearing impairment. • Not able to eat because of jerks. • He obeys & carried out simple commands & talks few words. • No H/O bowel & bladder disturbances.

• No previous H/O measles/ exanthematous illness. • Immunisation H/O details not known. • No H/o prior developmental delay. • No H/O similar illness in the family. 19 yr 10 yr

EXAMINATION child conscious afebrile no neurocutaneous markers vitals: PR : 100 / min RR : 20/ min BP : 100/70 mm. Hg Anthropometry Wt : 25 Kg/32. 5 Kg Ht : 130/132 cm HC : 50 cm

CNS EXAMINATION - posture : lying in bed flexed at thigh & knee Sudden jerky movements of both UL & LL speaks few words, respond to simple commands no cranial nerve palsy or dysfunction - motor system: tone increased in all 4 limbs Power > 3 in all 4 limbs DTR increased in all 4 limbs plantar B/L extensor - able to perceive pain - cerebellar system & gait could not be elicited No signs of meningeal irritation Other systems: normal

PROVISONAL DIAGNOSIS Myoclonic jerks for evaluation probably SSPE

DIFFERENTIAL DIAGNOSIS • Juvenile myoclonic epilepsy( Janz syndrome) – Onset between 12 - 16 yrs, on awakening, yrs later with GTCS, EEG- 4 – 6/sec irregular spike & wave pattern enhanced by photic stimulation • Lafora disease – 10 – 18 yrs with GTCS, with mental deterioration with myoclonic jerks ultimately with cerebellar and extrapyramidal signs, EEG- polyspike wave discharges in occipital region • MERRF – Myoclonic epilepsy, mitochondrial myopathy & cerebellar signs, associated with optic atrophy, SNHL, dementia

INVESTIGATION • Hb : 8. 4 g • TC : 10, 800/ mm 3 • DC : P 69 L 28 E 3 • Blood sugar : 80 mg/dl • Blood urea : 46 mg/dl • Sr creatinine : 1. 6 mg/dl • CT brain : normal

SLOW WAVE PERIODIC BURST

EEG : Periodic complexes with sharp wave & spike discharges seen. S/O SSPE.

FINAL DIAGNOSIS SSPE SUBACUTE SCLEROSING PANENCEPHALITIS

SSPE • Most common form of chronic encephalitis. • Incidence: 1 in 1, 000 • First described by Dawson in 1934 as “Inclusion body encephalitis” • Van Bogaert named it as SSPE. • M: F 2: 1 • Hispanic origin

SSPE • Measles at an early age (2 - 4 yr ) favors development of SSPE. • persistent infection - altered measles virus that is harbored intracellularly in CNS & altered host response to virus. • Interval : 7 – 10 yr • Insidious onset of neurological symptoms – juvenile period. • Leading to an inexorable neurodegenerative process & death within 1 – 3 yr of onset.

PATHOLOGY • Inflammation , necrosis and gliosis of brain • Microscopy : - perivascular infiltration - Neuronophagia - Proliferation of microglia & astrocytes - Coudry type A and B inclusion bodies • Cytokines are detected in brain lesions

STAGES OF SSPE Stages I II IV Parts of brain involved Cortex + subcortical white matter Deep nuclear structures Entire CNS

JABBOUR’ S Clinical stages of SSPE (1969) Stage 1: subtle features • • • Behavioural changes Decreased scholastic performance Irritability Hyperactivity Negativism Psychosis

STAGE 2 • • • Myoclonic jerks Choreo-athetosis Tremor Ballismus – dystonic posture Ataxia Spasticity • Ocular changes: 50% of patients choreoretinitis, reduced visual acuity, optic atrophy, papilledema, uveitis, retrobulbar neuritis

STAGE 3 • • • Increased spasticity Myoclonic jerks may decrease or disappear Decorticate or decerebrate posture Noisy breathing Autonomic disturbances May die of hyperthermia , cvs collapse, hypothalamic disturbances

STAGE 4 • • • Mutism Generalised hypotonia Loss of cortical function Random eye movements Vegetative stage

DIAGNOSTIC CRITERIA • Compatible clinical course & atleast one of the following 1) measles antibody detected in CSF 2) characteristic EEG 3) typical histological findings and / or isolation of virus/ viral antigen in brain tissue • Elevated measles Ab titres using complement fixation technique. CSF > 1: 8 dilution serum > 1: 24 dilution

CT & MRI non-specific for SSPE • CT : - variable cortical atrophy and ventricular dilatation - focal and multi focal whitematter abnormalities. • MRI : hypodense in T 1 weighted images in white matter.

EEG • Described by Radermecker and Cobb and Hill. • Normal in early stages. • High amplitude 300 -1500 microvolts repetitive polyphasic sharp and slow wave complexes of 0. 5 – 2 sec in duration , that occur every 4 -15 seconds and are syncronous with myoclonic jerks. • Terminal stages – disorganisation of background.

TREATMENT • Primarily supportive. • Amanitide : anti-RNA agent , prolongs life. • Isoprinosine (inosiplex ) - purine derivative - beneficial drug for reducing morbidity & mortality - 100 mg/kg/day • Plasmapheresis : not beneficial • Others : interferon α 2 b , pyroncopolymer , 5 -bromo-2 deoxyuridine.

TO CONCLUDE PREVENTION IS THE BEST TREATMENT.

About Dr. Amit Vatkar is a Pediatric Neurologist from Mumbai, India. He has completed his fellowship in Pediatric Neurology with specialising in Epilepsy surgery workup from Hinduja hospital under the guidance of Vrajesh Udani, top neurologist in India. He has also been trained in Epilepsy & neurophysiology at Case Western Reserve University at Cleveland under the guidance of Dr. Hans Luders. He specialises in Clinical Neurophysiology (EEG, EMG and NCV). He also provides portable EEG services in Mumbai. Currently, He is supporting many schools for children with special needs. He is attached to major hospitals in Mumbai where he consults pediatric neurological cases. His areas of expertise are 1. Epilepsy, Seizure disorders 2. Developmental Disorders including delayed speech, motor milestones, and coordination issues 3. Autism and other Behavioural disorders, including attention-deficit/hyperactivity disorder (ADHD), school failure and sleep problems 4. Movement Disorders, 5. Cerebral palsy, muscular dystrophy, and nerve muscle disorders 6. Headaches, including migraines

Dr. Amit Vatkar Pediatric Neurologist, Navi Mumbai MBBS, DNB Email: vatkaramit@yahoo. com Contact No. : +91 -8767844488 Visit us at: http: //pediatricneurology. in/ THANK YOU !