Atypical Parkinsonism (Parkinson Mimics, Parkinson Plus)

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

2. Atypical Parkinsonism=
•PD Mimics
•PD Plus

3. James Parkinson (1817)

5. 3 CASES

6. 3 CASES
with
1. Features of Parkinsonism
2. Diagnosed & Treated as Parkinson’s Disease

7. Case 1: ‘C. Dickens’ 58 yrs

8. 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

9. CASE 1:
Dickens; 58 yrs

10. Case 1

11. MRI Case 1:

12. Case 2: Robert 61 yrs

13. 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

14. Case 2: Robert. Age 61

15. MRI Case 2

16. Case 3: Alicia ‘64 years’

17. 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)

18. Case 3:

19. MRI Case 3:

20. 1st Diagnosis:
Parkinson’s Disease
Unique
Clinical
Findings
Unique
Imaging
Findings
Rapid
Progression
Poor Response
to Levadopa

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

22. PD Atypical
Parkinsonism
Parkinsonism
Sporadic Familial FamilialSporadic Secondary
PSP
MSA
CBD

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

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

25. BASIC PATHOLOGY

26. Synucleino-pathy Tau-pathy

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

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

29. Average Cases in Daily practice
85
10
5
0 20 40 60 80 100
PD
Secondary PD
Atypical Parkinsonism

30. Percentage of Atypical Parkinsonism varients
70
25
5
0 10 20 30 40 50 60 70 80
PSP
MSA
CBD

31. Average years of response to Levadopa
12
3
1.5
0.4
0 2 4 6 8 10 12 14
PD
MSA
PSP
CBD

32. 15
8
7
6
0 5 10 15 20
PD
MSA
PSP
CBD
Average survival years from symptom onset

33. PSP
(Progressive Supra-nuclear Palsy)

34. 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

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

36. MRI findings in PSP

37. MRI findings in PSP

38. MRI findings in PSP

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

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

41. PSP Case

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

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

44. MSA
(Multiple System Atrophy)

45. 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

46. MRI findings in MSA

47. MRI findings in MSA

48. Case 2: Robert: 61 yrs

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

50. CASE 2:
Diagnosis:
MSA-p

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

52. CBD
(Cortico-Basal Degeneration)

53. 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)

54. 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 %

55. MRI findings in CBD

56. Case 3:
Alicia, 58 yrs

57. 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

58. Case 3:
Diagnosis:
CBD
(Cortico-Basal Degeneration)

59. Hand posture in CBD

60. CBD Case

61. CBD Case

62. Alien Limb Phenomena
Example

63. CBD Case (Alien Limb with Myoclonus)

64. Approach to
Atypical Parkinsonism

65. 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

66. 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

67. 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 + ++ ++ +++

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

69. 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

70. 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

71. 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)

72. Actor ‘Kader Khan’: Diagnosed as PSP
Diagnosed: 2013; Died: 2017

73. Thank You