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5-10-2014
1
Parkinson’s disease: (differential) diagnosis
Professor Bastiaan R. BloemParkinson Center Nijmegen (ParC)
Radboud University Medical Center
@BasBloem
My fascination
Snijders et al., Neurologist 2012;18:404‐405
The patient as the teacher
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And now look at this!
Take home message
Parkinson’s disease cannot be
managed by neurologists alone
Take home message
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What beast are we fighting?
It seemed clear initially
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A recognizable (motor) syndrome
But what about this?
Cerebral MRI
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“Parkinson” is not a disease,it is a syndrome
Take home message
Complex manifestations
Motor symptoms & signs
Non‐motor manifestations
The explanation
Braak et al., Neurobiol Aging 2003;24:197‐211
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Even in early disease stages
• 159 newly diagnosed Parkinson patients
•Median disease duration 4.4 months
• 12% still untreated
• 159 newly diagnosed Parkinson patients
•Median disease duration 4.4 months
• 12% still untreated
Even in early disease stages
Take home message
Parkinson syndrome includes both motor and non‐motor
manifestations
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At the other end of the spectrum
Weerkamp et al., JAGS 2012;60:2277‐2282Weerkamp et al., JAGS (2013) in press
Example of wonderful results
Other concurrent diseases
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Importance of compensation
Disease
signs
Primary disease process Compensatory strategies
Compensation
Medical management Allied health care
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Medical management and allied
health interventions are
complementary approaches
Take home message
How do I recognize
Parkinson SYNDROME?
STN stimulators ON STN stimulators OFF
ESSENTIAL: bradykinesia
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STN stimulators ON STN stimulators OFF
ESSENTIAL: bradykinesia
Symptoms ≠ par cipa on
Take home message
Diagnosing Parkinson syndromeis a multidisciplinary enterprise!
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Look alikes
TRAP #1: is this bradykinesia?
Courtesy of Niall Quinn
TRAP #2: behind the mask …
Courtesy of Louise van der Valk (Papaver)
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TRAP #3: thumb extension tremor
Courtesy of Niall Quinn
TRAP #4: reduced arm swing
What do you see here?
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SWEDD’s
How can I suspect it might
be Parkinson DISEASE?
What is this?
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But what about this?
Or this?
Parkinson “disease”
Symptoms that SHOULD be present
Symptoms that should NOT be present
This is how it works
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Parkinson’s disease
Atypical parkinsonism
An important difference
Atypical parkinsonism is not a diagnosis
Parkinson Atypical parkinsonism
Marked overlap (particularly in early stages)
Early disease stages
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Parkinson Atypical parkinsonism
Marked overlap (particularly in early stages)
With disease progression
Parkinson Atypical parkinsonism
Marked overlap (particularly in early stages)
Post‐mortem examination
Why the difference is so important
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So when is it TRULY
Parkinson DISEASE?
This is definitive Parkinson’s disease
Healthy
Parkinson
The only exception during life
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What goes wrong in the brain?
Dopaminergic system
Frontal lobeStriatum
Substantia nigra
Ventral tegmental area
Where in the brain?
‐
‐
+
Dopaminergic system
Frontal lobeStriatum
Substantia nigra
Ventral tegmental area
Where in the brain?
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Much like a garden hose
The problem in atypical parkinsonism …
BUT: Parkinson’s also causes diffuse lesions!
Braak et al., Neurobiol Aging 2003;24:197‐211
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A brain on fire …
When can I expect it to be
Parkinson DISEASE?
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A common, well‐known disease
= Parkinson
= parkinsonism
Incidence
55‐65 65‐75 75‐85 85+
Age
Influence of age
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55‐65 65‐75 75‐85 85+
Age
= women
= men
Incidence
Influence of gender
1994 1998 2001
A progressive disease
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Severity of symptoms
Patient A
Highly variable rates of progression
Time
Patient B
Patient C
Important: patients tend to follow their
OWN course
Asymmetry
Good response to levodopa
Slow progression
Supportive features
Bloem’s “quick and dirty’ diagnosis:
Parkinson’s disease is:
1. Asymmetrical
2. Slowly progressive
3. Responds well to medication
Take home message
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Could this be Parkinson’s disease?
What do you see here?
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Red flags
Red flags, part 1
Abnormal posture
Recent summary
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Pisa syndrome
What do you see here?
Red flags, part 2
Gait and balance disability
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Gait & balance
disorders – please see
my other lecture
Autonomic dysfunction
Red flags, part 3
The “ cold hands” sign
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Extra diagnostic weapons
The weapon MEDICATION
Severity of symptoms
Time
Spontaneous course Parkinson
Treatment effect
Medication in atypical parkinsonism
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Spontaneous course parkinsonism
Treatment effectX
XCease treatment
Spontaneous course Parkinson
Treatment effect
Severity of symptoms
Time
Medication in atypical parkinsonism
Thank you!
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