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. 2010 Jan 1;3(1):1–23.

Table 1.

Classification of major neurodegenerative disorders

Disorder Anatomy Major clinical feature
Amyloidoses
 Aβ Alzheimer disease Corticolimbic Dementia
 ABri Familial British dementia Corticolimbic & cerebellar Dementia & ataxia
 PrP Creutzfeldt-Jakob disease Cortical & basal ganglia Dementia & movement disorder
Gerstmann-Straussler-Scheinker Cortical & Cerebellar Ataxia & dementia
Tauopathies
 4R Progressive supranuclear palsy Basal ganglia & brainstem Parkinsonism
Corticobasal degeneration Cortical & basal ganglia Focal cortical syndrome & parkinsonism
Argyrophilic grain disease Limbic Amnestic cognitive impairment
 3R Pick's disease Corticolimbic Dementia & focal cortical syndromes
 3R+4R Tangle predominant dementia Limbic Dementia
Guam Parkinson dementia complex Cortex & brainstem Dementia & parkinsonism
Synucleinopathies
Parkinson disease Brainstem Parkinsonism
Dementia with Lewy bodies Corticolimbic & brainstem Dementia & parkinsonism
Multiple system atrophy Basal ganglia, brainstem & cerebellum Parkinsonism & ataxia
TDP-43 proteinopathies
Amyotrophic lateral sclerosis Motor neurons Spasticity & weakness
Frontotemporal lobar degeneration with ubiquitinated inclusions Cortex & basal ganglia Dementia & focal cortical syndromes