Extrapyramidal Tutor Information PULSE Preparation for Finals Tutor

Extra-pyramidal – Tutor Information PULSE: Preparation for Finals Tutor name

Resource summary • Common viva questions/topics • Case-based additional information (Cases 1 – 5)

Common questions • Things you might pick up and questions you will get asked…

What is the function of the extrapyramidal system? Involuntary reflexes and movement, and co-ordination of movements

What are the different patterns of increased tone? • ‘Clasp knife’ • ‘Lead pipe’ • Cogwheeling

What are the different patterns of increased tone? • Spasticity = UMN. ‘Clasp knife’ • Rigidity = extrayramidal. ‘Lead pipe’ • Cogwheeling = increased tone with superimposed tremor

What are the causes of Parkinsonism? • • • IPD - asymmetric tremor, good response to L-dopa Vascular - Hx strokes, early falls, no tremor PSP - failure of up gaze, early falls MSA - cerebellar and pyramidal signs, postural drop, bladder dysfunction DLB - hallucinations, cognitive decline CBD - subcortical dementia, unilateral clumsiness/rigidity/bradykinesia Wilsons - AR, liver disease, KF rings Pugilistic - Hx head trauma e. g. boxing Drugs - metoclopramide, domperidone, antipsychotics Normal pressure hydrocephalus - urine incontinence, feet to floor gait, cognitive decline

What are the essential features of Parkinsonism? • Tremor • Rigidity • Akinesia • Postural instability • TRAP!

What are the other features of IPD? • Depression • Speech and swallow problems • Incontinence • REM sleep disturbance • Turning over in bed difficulty • Dementia • Anosmia • Handwriting - micrografia

What are the indications and side effects of L-dopa therapy? • If <70 yo first line is dopamine agonists, if >70 yo start L-dopa • 75% develop motor complications at 5 y • Given with peripheral decarboxylase inhibitor • Side effects: DOPAMINE - dyskinesia, on-off motor fluctuations, psychosis, ABP drop, mouth dryness, insomnia, N/V, excessive day time sleepiness

What are the other treatments of IPD? • • • Physiotherapy, Occupational therapy support Dopamine agonists (ropinirole, pramopexole) COMT inhibitors (entacapone) Apomorphine Surgery (pallidotomy) Deep brain stimulation

DDx of a tremor? Extrapyramidal Cerebellar Benign essential Physiological Medication related Worse on rest, better on movement Intention, worse on movement Postural, on action, positive FHx. Rx Bblockers. Hyperthyroidism, anxiety, ETOH withdrawal B agonists, lithium, antidepressants, valproate

Case 1

What are the causes of parkinsonism…. ? Idiopathic PD - Due to degeneration of dopaminergic neurons in the pars compacta of the substantia nigra Secondary Parkinsonism - Drug induced - MPTD toxicity - Post-infective (Von Economo’s encephalitis, encephalitis lethargica Parkinsonian plus

To complete my examination… • Cranial nerve examination • Full neurological Hx including drug history • Assess for and exclude parkinsonian-plus syndromes…

Case 2

Parkinson’s PLUS • Active stand reveals a postural drop Multi-system atrophy

Case 3

Parkinson’s PLUS • Visual fields – unable to look up Progressive Supra-nuclear palsy (PSP)

Case 4

Parkinson’s PLUS • Reduced score on MMSE; poor cognitive function Fronto-temporal or Lewy-body Dementia (FTD/LBD)

Case 5

• Abdominal examination: • Dupytren’s contracture • Palmar erythema • LOSS of axillary hair • 8 spider naevi on anterior chest • Gynaecomastia Wilson’s Disease

Special Tests for Parkinson’s • Gait – shuffling, poor initiation (hesitation), lack of normal arm swing, difficulty turning • Bradykinesia – decrease in speed and amplitude of complex movements • Tapping – ask pt to tap fingers in turn onto surface repeatedly, quickly and with both hands at once • Twiddling – rotating hands around each other in front of body • Tremor – resting, pill-rolling tremor (4 -6 Hz); facilitated by distraction (e. g. serial 7 s from 100, or moving the contralateral limb e. g. rapidly opposing contralateral thumb and fingers) • Tone – lead pipe rigidity (increased tone) or cogwheel (exaggerated stretch reflex interrupted by tremor) • Face • Absence of blinking • Lack of facial expression • Glabellar tap – keeping finger out of pt’s line of sight, tap middle of forehead (glabella) with middle finger – normal people blink a few times then stop; in PD blinking persists • Speech - Typically monotonous, soft and faint, lacking intonation; Palilalia sometimes present (repetition of end of word) • Writing - micrographia