Extra-pyramidal

1. Extra-pyramidal PULSE: Preparation for finals Tutor name

2. TuBS attendance • https://tutorialbooking.com/

3. Session overview • Common extra-pyramidal conditions for the OSCE • How to present your findings • Case presentations and OSCE questions

4. What is the purpose of an OSCE? “This station tests a student’s ability to perform an appropriate focussed physical examination, demonstrating consideration for the patient, and to report back succinctly describing the relevant findings. It also tests a student’s clinical judgement i.e. the ability to decide the differential diagnosis, choose investigations and formulate a management plan.”

5. Common extra-pyramidal conditions in the OSCE • Tremor • Parkinsonism • Gait problems

6. Presenting your findings • What were you asked to do? • What were your key positive findings? • What were the important negative findings? • What does this mean? • How would you complete your examination, and what investigations would you do?

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

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

9. 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

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

11. 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

12. Example case presentation • Asked to examine the hands and face of this 75 year old lady

13. Extrapyramidal • Upper Limb neuro examination • Face: CN, speech • WINDEC • STAND BACK INSPECT • If impression of PD focus of eliciting EP signs: tremor, rigidity, speech • FUNCTION: button, hadnwriting

14. 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 7s 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

15. On examination she had a resting tremor which was more obvious on the right than the left hand. This tremor improved on movement. • She had increased tone in both upper limbs with some evidence of cogwheeling. • Examination of the face was unremarkable however I did note that she had a very quiet voice. Her eye movements were normal.

16. Completing your examination • Cranial nerve examination • Full neurological Hx including drug history • Assess for and exclude parkinsonian-plus syndromes…

17. Investigations • Bloods; FBC, U&E, LFTs, caeruloplasmin, INR, antibodies, glucose, B12/folate • LP; cells, oligoclonal bands, xanthochromia • Nerve conduction studies • Imaging; CXR (paraneoplastic), CT, MRI (posterior lesions, cord), DAT scan • Other; nerve biopsy, urine dip (glucose, blood), MMSE/ACER

18. Case 1

19. 70 year old gentleman Short shuffling steps, slow turn. Pill rolling tremor

20. Case 1 - continued • Mask like facies • Tone – lead pipe and cog-wheel rigidity • Power – 5/5 throughout • Coordination – tremor, difficult dysdiadochokinesis testing • Reflexes – normal, symmetrical • Sensation is normal Please present your findings.

21. Case 2

22. 70 year old gentleman Short shuffling steps, slow turn. Pill rolling tremor

23. Case 2 - continued • Mask like facies • Tone – lead pipe and cog-wheel rigidity • Power – 5/5 throughout • Coordination – tremor, difficult dysdiadochokinesis testing • Reflexes – normal, symmetrical • Sensation is normal

24. Parkinson’s PLUS • Active stand reveals a postural drop Please present your findings.

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

26. Case 3

27. 70 year old gentleman Short shuffling steps, slow turn. Pill rolling tremor

28. Case 3 - continued • Mask like facies • Tone – lead pipe and cog-wheel rigidity • Power – 5/5 throughout • Coordination – tremor, difficult dysdiadochokinesis testing • Reflexes – normal, symmetrical • Sensation is normal

29. Parkinson’s PLUS • Visual fields – unable to look up Please present your findings.

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

31. Case 4

32. 70 year old gentleman Short shuffling steps, slow turn. Pill rolling tremor

33. Case 4 - continued • Mask like facies • Tone – lead pipe and cog-wheel rigidity • Power – 5/5 throughout • Coordination – tremor, difficult dysdiadochokinesis testing • Reflexes – normal, symmetrical • Sensation is normal

34. Parkinson’s PLUS • Reduced score on MMSE; poor cognitive function Please present your findings.

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

36. Case 5

37. 70 year old gentleman Short shuffling steps, slow turn. Pill rolling tremor

38. Case 5 - continued • Mask like facies • Tone – lead pipe and cog-wheel rigidity • Power – 5/5 throughout • Coordination – tremor, difficult dysdiadochokinesis testing • Reflexes – normal, symmetrical • Sensation is normal

39. Parkinson’s PLUS • Abdominal examination: • Dupytren’s contracture • Palmar erythema • LOSS of axillary hair • 8 spider naevi on anterior chest • Gynaecomastia Please present your findings.

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

41. What are the indications and side effects of L-dopa therapy? • If <70yo first line is dopamine agonists, if >70yo start L-dopa • 75% develop motor complications at 5y • 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

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

43. Summary • Common extra-pyramidal conditions for the OSCE • How to present your findings • Case presentations and viva questions • Parkinson’s PLUS Syndromes • Multi-system atrophy (MSA) • Progressive supra-nuclear palsy (PSP) • Fronto-temporal/Lewy-body dementia (FTD/LBD) • Wilson’s Disease

44. Please complete TuBS feedback • Tutor details • For more information on Examining for Finals sessions: • examiningforfinals@gmail.com • www.sefce.net/pulse • Resource updated 2017: Dr A Swan • With thanks to previous contributors: • Dr Emma Claire Phillips (FY2) • Dr Kristina Lee (FY2) • Dr Russell Hewett (Neurology Consultant)