Atypical Parkinsonian Syndromes

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Presentation transcript:

Atypical Parkinsonian Syndromes Sara Radmard, MD, PGY-2

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

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

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

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

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

Tauopathies Synucleinopathies PSP FTLD CBD PD MSA LBD

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

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

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 https://www.youtube.com/watch?v=G3JceitLE2g&t=42s

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

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

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