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Synucleinopathies
- PD (Ch379)
- DLB (Ch381)
- MSA
Other Parkinsonism
- Vascular
- Drug-induced
- Wilson, NBIA, Huntington
- Mass lesions
MSA (Multiple System Atrophy)
Features
- Mean onset 55-65
- Mean survival 6-10 years
- Severe autonomic failure early:
- Orthostatic hypotension (drop ⥠30/15 within 3 min)
- Urinary incontinence/retention
- Sexual dysfunction
- Movement features:
- MSA-P (parkinsonian): parkinsonism more prominent
- MSA-C (cerebellar): ataxia more prominent
- Inspiratory stridor (laryngeal dystonia â characteristic, can be fatal)
- REM sleep behavior disorder (common)
- Poor/early-failing levodopa response
- Pyramidal signs
- Bulbar dysfunction
- Cold/discolored hands
Pathology
- α-synuclein in glial cytoplasmic inclusions (GCIs)
- Different from PD/DLB (neuronal)
Diagnosis
- Clinical (consensus criteria)
- MRI:
- âHot cross bun signâ (pons cruciate hyperintensity on T2 â MSA-C)
- Putaminal atrophy + hyperintensity rim (MSA-P)
- Cerebellar atrophy (MSA-C)
- Autonomic testing
- MIBG-cardiac scintigraphy (preserved in MSA, decreased in PD)
Treatment
- No disease-modifying
- Supportive:
- Orthostatic hypotension (midodrine, fludrocortisone, droxidopa)
- Catheterization for urinary
- PEG for dysphagia
- CPAP for stridor (life-saving)
- PT, OT, speech therapy
- Levodopa trial (may help motor briefly)
- Multidisciplinary care
PSP (Progressive Supranuclear Palsy)
Features
- Mean onset 60-65
- Mean survival 5-8 years
- Vertical supranuclear gaze palsy (especially downgaze) â hallmark
- Early falls (within 1 year) â backward falls
- Axial rigidity > limb
- Frontal cognitive impairment (apathy, dysexecutive)
- Pseudobulbar
- Dysphagia, dysarthria
- Apraxia of eyelid opening
- Square wave jerks
- Poor levodopa response
Variants
- Richardson syndrome (classic â gaze palsy + falls + cognitive)
- PSP-P (parkinsonism predominant, mimics PD)
- PSP-PGF (progressive gait freezing)
- PSP-CBS (corticobasal syndrome)
- PSP-SL (speech and language)
Pathology
- 4R tau deposits
- Globose tangles
- Tufted astrocytes
Diagnosis
- Clinical (MDS criteria 2017)
- MRI:
- âHummingbird signâ (midbrain atrophy, sagittal)
- âMickey Mouse signâ (midbrain on axial)
- Midbrain : pons area ratio reduced
- Tau PET (research)
Treatment
- No disease-modifying (multiple negative trials of anti-tau antibodies â gosuranemab, tilavonemab, BIIB092)
- Supportive
- Falls prevention
- Levodopa trial (limited)
- Botulinum toxin for blepharospasm/dystonia
- SSRIs for pseudobulbar affect
- Amantadine sometimes
CBD (Corticobasal Degeneration)
Features
- Mean onset 60-70
- Mean survival 6-8 years
- Asymmetric parkinsonism + cortical features:
- Alien limb phenomenon â involuntary movements feel ânot theirsâ
- Cortical sensory loss (astereognosis, agraphesthesia)
- Apraxia (ideomotor)
- Myoclonus
- Dystonia (often arm)
- Limb rigidity asymmetric
- Cognitive impairment
- Aphasia (variants)
- Poor levodopa response
Variants
- CBS (corticobasal syndrome) â clinical
- CBD â pathological diagnosis
- Can be CBS-PSP, CBS-AD overlap
Pathology
- 4R tau
- Astrocytic plaques
- Ballooned neurons
Diagnosis
- Clinical (Armstrong criteria)
- MRI: asymmetric perirolandic atrophy
- DAT-scan reduced
Treatment
- No disease-modifying
- Supportive
- Levodopa rarely helps
- Botulinum toxin for dystonia
- Multidisciplinary
𩺠åºééæ¥
- Atypical parkinsonism = parkinsonism + atypical features + poor levodopa response often
- MSA: autonomic failure + cerebellar OR parkinsonian + stridor + RBD + âhot cross bunâ MRI
- PSP: vertical supranuclear gaze palsy + early falls + axial rigidity + âhummingbirdâ MRI
- CBD: asymmetric + alien limb + cortical sensory + apraxia + myoclonus
- No disease-modifying therapy
- Supportive care multidisciplinary