Spinocerebellar ataxia
Spinocerebellar ataxias comprise a large (and expanding) group of diseases characterized by degeneration of the spinal cord and cerebellum.
There are well over 25 individual spinocerebellar ataxias referred to sequentially as SCA1, SCA2, ... in order of their discovery and genetic characterization.
Radiographic features
Although individual spinocerebellar ataxias vary in morphological changes, atrophy of the cerebellum is a relatively constant finding. Extracerebellar regions are also affected, depending on the specific SCA 1.
Related Radiopaedia articles
Neurodegenerative diseases
Neurodegenerative diseases are legion and their classification just as protean. A useful approach is to divide them according to underlying pathological process, although even using this schema, there is much overlap and thus resulting confusion.
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neurodegenerative MRI brain (an approach)
- measurements and ratios
- midbrain to pons area ratio (for PSP)
- Magnetic Resonance Parkinsonism Index (MRPI) (for PSP)
- frontal horn width to intercaudate distance ratio (FH/CC) (for Huntington disease)
- intercaudate distance to inner table width ratio (CC/IT) (for Huntington disease)
- signs
- hummingbird sign (of PSP)
- Mickey Mouse sign (of PSP)
- morning glory sign (of PSP)
- hot cross bun sign (of MSA-C)
- hockey stick sign (of Creutzfeldt-Jakob disease)
- pulvinar sign (of Creutzfeldt-Jakob disease)
- scoring systems
- Fazekas scale for white matter lesions
- posterior atrophy score of parietal atrophy (PA/PCA) (Koedam score)
- medial temporal lobe atrophy score (MTA score)
- global cortical atrophy scale (GCA scale)
- measurements and ratios
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neurodegenerative diseases
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synucleinopathies
- diseases with Lewy bodies
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multiple systemic atrophy (MSA)
- Shy-Drager syndrome
- MSA-P (striatonigral degeneration)
- olivopontocerebellar atrophy (MSA-C)
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tauopathies
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Alzheimer disease
- typical/classical Alzheimer disease
- variant (e.g. posterior cortical atrophy)
- chronic traumatic encephalopathy (CTE)
- corticobasal degeneration
- frontotemporal lobar degeneration (FTLD) (not all are tau)
- Pick disease
- progressive supranuclear palsy (PSP)
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Alzheimer disease
- amyloidoses
- TDP-43 proteinopathies
- spinocerebellar ataxias
- Huntington disease
- hereditary spastic paraplegia
- clinically unclassifiable parkinsonism (CUP)
- Unverricht-Lundborg disease
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prion diseases (not always included as neurodegenerative)
- Creutzfeldt-Jakob disease (sporadic, variant, familial, and iatrogenic)
- fatal familial insomnia
- Gerstmann-Straussler-Scheinker disease
- kuru
- variably protease sensitive prionopathy
-
synucleinopathies