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cerebellar ataxia (cerebellar syndrome, cerebellar dysmetria)
Etiology:
1) symmetric & progressive signs
a) acute (days to hours)
1] drugs
a] alcohol
b] lithium
c] phenytoin (Dilantin)
d] barbiturates
2] acute viral cerebellitis
3] postinfection syndrome
b) subacute (days to weeks)
1] intoxication
a] mercury
b] solvents
c] gasoline
d] glue
e] cytotoxic chemotherapy
2] alcoholic-related nutritional deficiency
a] thiamine deficiency
b] vit B12 deficiency
3] Lyme disease
c) chronic (months to years)
1] paraneoplastic syndrome
2] hypothyroidism
3] inherited diseases
4] tertiary syphilis (tabes dorsalis)
2) ocal & ipsilateral cerebellar signs
a) acute (hours to days)
1] vascular
a] cerebellar infarction
b] cerebellar hemorrhage
c] subdural hematoma
2] infectious - cerebellar abscess
b) subacute (days to weeks)
1] neoplastic
a] cerebellar glioma
b] metastatic tumor
2] demyelinating
a] multiple sclerosis
b] progressive multifocal leukoencephalopathy (PML), AIDS-related
c) chronic (months to years)
1] gliosis secondary to vascular lesion or demyelinating plaque
2] congenital lesion
a] Dandy-Walker malformation
b] Arnold-Chiari malformation
c] Friedreich ataxia
Epidemiology:
- late-onset cerebellar ataxia prevalence is 1-3 per 100,000 [3]
Genetics:
- intronic FGF14 GAA repeat expansion in late-onset cerebellar ataxia [3]
- autosomal dominant inheritance
Clinical manifestations:
- gait ataxia
- wide-based gait with increased trunk sway
- irregular stepping, staggering, especially on turns
- poor trunk control
- incoordination or other cerebellar signs
- not much change in balance with eyes closed (negative Rhomberg test)
- late-onset cerebellar ataxia manifests after 30 years of age [3]
Specific
cerebellar ataxia, Cayman-type
Friedreich ataxia
spinocerebellar ataxia (SCA)
General
cerebellar degeneration
ataxia; dyssynergia
Database Correlations
OMIM correlations
References
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 2263-67
- Geriatric Review Syllabus, 7th edition
Parada JT et al (eds)
American Geriatrics Society, 2010
- Geriatric Review Syllabus, 8th edition (GRS8)
Durso SC and Sullivan GN (eds)
American Geriatrics Society, 2013
- Pellerin D et al.
Deep intronic FGF14 GAA repeat expansion in late-onset cerebellar ataxia.
N Engl J Med 2023 Jan 12; 388:128
PMID: 36516086
https://www.nejm.org/doi/10.1056/NEJMoa2207406