Atypical Parkinsonism

Atypical Parkinsonism (Parkinson Mimics, Parkinson Plus)

1. A TYPICAL PARKINSONISM Presented By: Dr. Ahmed Tanjimul Islam MD ( Neurology) Rajshahi Medical College Hospital, Bangladesh.

2. A typical Parkinsonism= PD Mimics PD Plus

3. James Parkinson (1817)

4. 3 CASES

5. 3 CASES with 1. Features of Parkinsonism 2. Diagnosed & Treated as Parkinson’s Disease

6. Case 1: ‘C. Dickens’ 58 yrs

7. CASE 1: (Dickens) Findings: History 2005 Jan: Slowness of movement. 2005 July : Rigidity (axial). 2006 Jan: Difficulty in walking, using stairs. 2006 March: Faint attack 2 times. Examination Tremor: Absent Gait: Wide based (not parkinsonian) Eye: Vertical eye movement: Absent. BP: Postural drop: absent Focal signs : absent Investigation MRI: Midbrain Atrophy

8. CASE 1: Dickens; 58 yrs

9. Case 1

10. MRI Case 1:

11. Case 2: Robert 61 yrs

12. Case 2 (Robert) Findings History 2008 Jan: Diagnosed Parkinson’s disease 2009 Dec: Frequent Falls, Incontinence 2010 Dec : Severe Dementia 2011 July: Wheel chair bound Examination Tremor: Mild Severe Axial Rigidity MMS : Severe Dementia Eye: Stare look (Gaze palsy: absent) BP: Postural Drop Investigation MRI: Putamen Atrophy Hot Cross Bun Sign

13. Case 2: Robert. Age 61

14. MRI Case 2

15. Case 3: Alicia ‘64 years’

16. CASE 3 (Alicia) Findings History 2004 Jan: Unilateral upper limb difficulty 2005 Feb: Fixed dystonic hand posture 2007 Jan: Severe slow, stooped posture 2008 July: Wheel chair bound 2008 Dec: Severe dementia Examination Tremor: Absent Rigidity: Present (unilateral) Bradykinesia (Axial): Present BP: Postural drop: Absent Investigation MRI: Cortical Atrophy (Unilateral)

17. Case 3:

18. MRI Case 3:

19. 1 st Diagnosis: Parkinson’s Disease Unique Clinical Findings Unique Imaging Findings Rapid Progression Poor R esponse to Levadopa

20. PD Atypical Parkinsonism Parkinsonism Sporadic Familial Familial Sporadic Secondary A= Accidents B= Blow C= CVD D= Drugs, Toxins E= Encephalopathy

21. PD Atypical Parkinsonism Parkinsonism Sporadic Familial Familial Sporadic Secondary PSP MSA CBD

22. Atypical Parkinsonism Parkinsonism PSP MSA CBD Steele Richardson Olszewski Syndrome Shy Drager Syndrome 1996

23. Atypical Parkinsonism Parkinsonism MSA Shy Drager Syndrome 1996 MSA P: Striatonigral Degeneration MSA C: Olivopontocerebellar Degeneration MSA A: Shy Drager Syndrome

24. BASIC PATHOLOGY

25. Synucleino-pathy Tau- pathy

26. Synucleino-pathy Tau- pathy Parkinsonism Cognitive impairment MSA CBD PSP Cognitive impairment Synucleino-pathy Tau- pathy Parkinsonism

27. Synucleino-pathy Tau- pathy Parkinsonism Cognitive impairment MSA PD CBD PSP Cognitive impairment Synucleino-pathy Tau- pathy Parkinsonism

28. Average Cases in Daily practice

29. Percentage of Atypical Parkinsonism varients

30. Average years of response to Levadopa

31. Average survival years from symptom onset

32. PSP (Progressive Supra-nuclear Palsy)

33. Diagnostic Criteria of PSP Clinically Possible Clinically Definite 1. Age> 40 1. Onset after 40 2. Slow Vertical saccades 2. Rapid Progressive 3. Vertical Gaze Palsy 4. Postural instability/ Falls

34. PSP Neuro-Ophthalmological features Vertical Supranuclear gaze Plasy Downward > Upward Horizontal Slow saccade Convergence: Lost Total Gaze palsy

35. MRI findings in PSP

36. MRI findings in PSP

37. MRI findings in PSP

38. CASE 1: Findings Case Findings Age Yes 2. Rapid Progression Yes 3. Gaze palsy (Vertical/ Horizontal) Yes 4. Postural Instability NO 5. Axial Rigidity Yes

39. Case 1: Diagnosis: PSP (Progressive Supra-Nuclear Palsy)

40. PSP Case

41. PD Vs PSP Signs PD PSP Eye signs - +++ Postural Instability - (late) +++ Axial rigidity + +++ Resting Tremor +++ - Levadopa response +++ +

42. PSP P= Progressive, Postural Instability S= Symmetrical, Slow Saccade (Vertical gaze palsy) P= Penguin sign

43. MSA (Multiple System Atrophy)

44. Diagnostic criteria of MSA Possible MSA P: Parkinsonism Possible MSA C: Cerebellar Dysfunction Probable MSA P: Autonomic failure + Poor Levadopa Response Probable MSA C: Autonomic Failure + Poor Levadopa Response Definite: Pathological Confirmation

45. MRI findings in MSA

46. MRI findings in MSA

47. Case 2: Robert: 61 yrs

48. Case 2 Findings Age, Rapid progression Yes Parkinsonism predominant Yes 2. Cerebellar predominant NO 3. Autonomic involvement Yes 4. Poor Levadopa Response Yes Case 2: Robert

49. CASE 2: Diagnosis: MSA-p

50. PD vs MSA Signs PD MSA Autonomic + +++ Cerebellar - +++ Postural instability + +++ Dementia - ++ Levadopa response +++ + (Transient)

51. CBD ( Cortico -Basal Degeneration)

52. Diagnostic points of CBD 1. Unilateral Onset 2. Dystonia in arm 4. Rigidity & Bradykinesia 5. Limb apraxia / Useless arm 6. Alien Limb Phenomenon 7. Myoclonus (Stimulus Sensitive)

53. Diagnostic points of CBD % 1. Unilateral Onset 95 % 2. Dystonia in arm 80 % 4. Rigidity & Bradykinesia 80 % 5. Limb apraxia / Useless arm 50 % 6. Alien Limb Phenomenon 50 % 7. Myoclonus (Stimulus Sensitive) 50 %

54. MRI findings in CBD

55. Case 3: Alicia, 58 yrs

56. Case 3: Alicia 1. Unilateral Onset Yes 2. Dystonia in arm Yes 4. Rigidity & Bradykinesia Yes 5. Limb apraxia / Useless arm No 6. Alien Limb Phenomenon No 7. Myoclonus (Stimulus Sensitive) No

57. Case 3: D iagnosis: CBD ( Cortico -Basal Degeneration)

58. Hand posture in CBD

59. CBD Case

60. CBD Case

61. Alien Limb Phenomena Example

62. CBD Case (Alien Limb with Myoclonus)

63. Approach to A typical Parkinsonism

64. When to suspect Atypical Parkinsonism Motor Ocular Behavioral Autonomic Early Falls Cerebellar Signs Pyramidal Signs Axial Rigidity Orthostatic Hypotension Incontinence Impotency Early Dementia Hallucination Vertical Gaze Palsy Slow Saccades Imaging (MRI, PET Scan findings) Response to Levadopa Rapid Progression of disease

65. When to suspect Atypical Parkinsonism Motor Ocular Behavioral Autonomic Imaging (MRI, PET Scan findings) Response to Levadopa Rapid Progression of disease A= Autonomic, Axial B= Bowel Bladder C= Cerebellar D= Dementia E= Eye Signs F= Falls

66. Signs PD PSP MSA CBD Symmetry + +++ +++ _ Axial Rigidity + +++ ++ + Palsy Vertical Gaze + +++ + + Postural instability + +++ +++ + Autonomic features +- + +++ - L Dopa response Early +++ + + - L Dopa response late ++ - + - Limb dystonia Alien limb - - - +++ MRI findings + ++ ++ +++

67. Cause of Death: 1. Infections 2. Aspiration Pneumonia 3. Respiratory Failure 4 . Cachexia, Wasting Syndrome

68. Treatment of Atypical Parkinsonism: Levadopa / Dopamine Dopamin agonist Supportive Therapy Rehabilitation Palliative Care 1-3 years 3-6 years 6-9 years 9-12 years

69. Treatment of Atypical Parkinsonism: Supportive Therapy 1-3 years 3-6 years 6-9 years 9-12 years Physical Therapy Speech therapy Occupational Therapy Percuteneous gastrostomy Botox Anti- depressents Anti-psychotics Anticholinergics

70. Summery Main Findings MRI PSP P= Postural instability S= Symmetrical Parkinsonism P= Palsy (Vertical Gaze) P= Penguin Sign Humming bird sign MSA M= Mean BP drop S= Symmetrical Parkinsonism A= Ataxia MSA c: Cross bun sign MSA p: Putamen Atrophy CBD A= Asymmetrical A= Akinetic Rigidity A= Apraxia A= Alien Limb Cortex & Basal Ganglia atrophy (Frontal , Parietal lobe)

71. Actor ‘Kader Khan’: Diagnosed as PSP D iagnosed: 2013 ; Died: 2017

72. Thank You