1. Atypical Parkinsonian Syndromes
Sara Radmard, MD, PGY-2

2. Parkinsonism definition
Hypokinetic syndrome
Bradykinesia, akinesia
Rigidity
Postural instability
Tremor (typically resting tremor)

3. Atypical Parkinsonian Syndromes
Lewy Body Dementia
Multiple System Atrophy
Progressive supranuclear palsy
Cortical basal degeneration

4. Other diseases that can have Parkinsonian features
Spinocerebellar ataxias (2,3,17)
Huntington disease
Wilson disease
Neuronal brain iron accumulation disorders
Mitochondrial disorders
X-linked dystonia-parkinsonism

5. Atypical Parkinsonian Syndrome Features
Multisystem degeneration
Early falls/postural instability
Early dementia
Marked dysautonomia
Decreased responsiveness to dopamine
Ocular findings or bulbar signs
Ataxia
Myoclonus
Alien limb phenomenon

6. Prognosis Atypical Parkinsonian Syndromes
More rapid decline
Time of death from diagnosis typically <7 years (depending on syndrome)

7. Tauopathies Synucleinopathies
PSP
FTLD
CBD PD
MSA
LBD

8. Progressive Supranuclear Palsy
Average age onset in 60s
Mean survival from diagnosis 5-8 years
Features: Early falls/gait instability, axial rigidity, supranuclear gaze palsy, progressive dementia
Other typical features: Dysphagia, voice changes (dysarthria, dysphonia)
Marked midbrain atrophy

9. Subcortical Dementia PSP generally presents with subcortical dementia
Bradyphrenia
Decreased processing speed
Verbal fluency problems (like primary progressive aphasia)
Executive dysfunction

10. Corticobasal Degeneration
Cortico=cortex, basal=basal ganglia
Key features: Asymmetric rigidity (arm), dystonia, ideomotor apraxia, frontal/striatal/parietal dementia, alien limb phenomenon, myoclonus
Other typical features: gait difficulty, action or rest tremor, bulbar impairment, hyperreflexia, extensor plantar response
Ideomotor apraxia: inability to perform a skilled motor task despite having intact language, motor and sensory function
-Cannot mime certain tasks (brushing teeth, using screwdriver, cutting with scissors
Alien limb: abnormal grasping, posturing, or spontaneous leveitation of arm or leg. Can go against contralateral limb

11. CBD dementia: Fronto-striatal-parietal
Frontal: decreased concentration, attention, verbal fluency, executive dysfunction
Parietal: visuospatial dysfunction, apraxia

12. Multiple System Atrophy
Median age of onset late 50s
Mean survival from time of diagnosis 6-9 years
Triad: Parkinsonism, marked dysautonomia, and cerebellar/ pyramidal signs
-Two main types: Parkinsonian versus cerebellar ataxia
Symptoms more symmetric
Resting tremor not typical

13. Lewy Body Dementia Most common cause of dementia after AD
Dementia must precede Parkinsonism by 1 year
Visual hallucination
Fluctuating consciousness throughout the day
Increased propensity for REM behavioral sleep disorder
Diffuse Lewy Bodies that starts in brainstem then goes throughout cortex