Parkinson s disease: (differential) diagnosis Professor Bastiaan R. Bloem Parkinson Center Nijmegen (ParC) Radboud University Medical Center @BasBloem My fascination The patient as the teacher Snijders et al., Neurologist 2012;18:404 405 1
And now look at this! Take home message Take home message Parkinson s disease cannot be managed by neurologists alone 2
What beast are we fighting? It seemed clear initially 3
A recognizable (motor) syndrome But what about this? Cerebral MRI 4
Take home message Parkinson is not a disease, it is a syndrome Complex manifestations Motor symptoms & signs Non motor manifestations The explanation Braak et al., Neurobiol Aging 2003;24:197 211 5
Even in early disease stages 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 6
At the other end of the spectrum Weerkamp et al., JAGS 2012;60:2277 2282 Weerkamp et al., JAGS (2013) in press Example of wonderful results Other concurrent diseases 7
Importance of compensation Compensation Disease signs Primary disease process Compensatory strategies Medical management Allied health care 8
Take home message Medical management and allied health interventions are complementary approaches How do I recognize Parkinson SYNDROME? ESSENTIAL: bradykinesia STN stimulators ON STN stimulators OFF 9
ESSENTIAL: bradykinesia STN stimulators ON STN stimulators OFF Symptoms par cipa on Take home message Diagnosing Parkinson syndrome is a multidisciplinary enterprise! 10
Look alikes TRAP #1: is this bradykinesia? Courtesy of Niall Quinn TRAP #2: behind the mask Courtesy of Louise van der Valk (Papaver) 11
TRAP #3: thumb extension tremor Courtesy of Niall Quinn TRAP #4: reduced arm swing What do you see here? 12
SWEDD s How can I suspect it might be Parkinson DISEASE? What is this? 13
But what about this? Or this? This is how it works Parkinson disease Symptoms that SHOULD be present Symptoms that should NOT be present 14
Parkinson s disease An important difference Atypical parkinsonism Atypical parkinsonism is not a diagnosis Marked overlap (particularly in early stages) Parkinson Atypical parkinsonism Early disease stages 15
Marked overlap (particularly in early stages) Parkinson Atypical parkinsonism With disease progression Marked overlap (particularly in early stages) Parkinson Atypical parkinsonism Post mortem examination Why the difference is so important 16
So when is it TRULY Parkinson DISEASE? This is definitive Parkinson s disease Healthy Parkinson The only exception during life 17
What goes wrong in the brain? Where in the brain? Frontal lobe Dopaminergic system Striatum Substantia nigra Ventral tegmental area Where in the brain? Frontal lobe Dopaminergic system Striatum Substantia nigra Ventral tegmental area + 18
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 19
A brain on fire When can I expect it to be Parkinson DISEASE? 20
A common, well known disease Incidence Influence of age = Parkinson = parkinsonism 55 65 65 75 75 85 85+ Age 21
Incidence Influence of gender = women = men 55 65 65 75 75 85 85+ Age A progressive disease 1994 1998 2001 22
Highly variable rates of progression Severity of symptoms Important: patients tend to follow their OWN course Patient C Patient B Patient A Time Supportive features Asymmetry Slow progression Good response to levodopa Take home message Bloem s quick and dirty diagnosis: Parkinson s disease is: 1. Asymmetrical 2. Slowly progressive 3. Responds well to medication 23
Could this be Parkinson s disease? What do you see here? 24
Red flags Red flags, part 1 Abnormal posture Recent summary 25
Pisa syndrome What do you see here? Red flags, part 2 Gait and balance disability 26
Gait & balance disorders please see my other lecture Red flags, part 3 Autonomic dysfunction The cold hands sign 27
Extra diagnostic weapons The weapon MEDICATION Medication in atypical parkinsonism Severity of symptoms Spontaneous course Parkinson Treatment effect Time 28
Medication in atypical parkinsonism Severity of symptoms Spontaneous course parkinsonism X Cease treatment X Treatment effect Spontaneous course Parkinson Treatment effect Time Thank you! 29