ATAXIAS: General
Classification
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Congenital Hereditary  Autosomal Dominant Autosomal Recessive X-linked Congenital DNA repair defects Episodic Metabolic disorders Mitochondrial Multisystem disorders Comparative pathology External link Immune Acute cerebellar ataxia Miller-Fisher Multiple sclerosis Opsoclonus-Myoclonus Paraneoplastic |
Infections: CNS Acute ataxia: Viral Creutzfeldt-Jakob Meningitis Mass lesion Abscess Neoplasm Sarcoid Paroxysmal Epilepsy Febrile Hereditary Migraine Polyneuropathy Anti-MAG Syndrome GALOP Syndrome Sensory Neuronopathy Large fiber |
Supratentorial Gait disorders Elderly Extrapyramidal Hydrocephalus Systemic Amyloid Autoimmune GAD antibodies Endocrine Hypoparathyroid Thyroid GI disorders Celiac disease; Sprue Vitamin E malabsorption Whipple's Trauma Vascular Vestibular |
Toxins & Drugs
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Ataxia: Differential diagnostic testing 1
- Serum +
- Blood smear
- Cholesterol
- Cholestanol
- Liver enzymes: Alcoholic cerebellar degeneration
- Phytanic acid
- α-fetoprotein
- Amino & Organic acids
- Autoantibodies: Hu; Yo; MAG; GAD; GQ1b ganglioside; Celiac; ZIC4; CV2; Ri; Tr
- Thyroid function
- Thyroperoxidase antibodies: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT)
- Vitamins: B1, B12, E
- Infections: HIV; Syphilis; CJD
- Muscle biopsy
- CSF: Creutzfeld-Jacob
- Duodenal biopsy: Whipple's disease
- MRI: Superficial siderosis
- Genetics
- Also see: Cerebellar anatomy
CEREBELLAR DYSFUNCTION: ANATOMY
| Cerebellar lesion | Signs or Function |
| Posterior (Flocculo-nodular lobe; Archicerebellum) |
Eye movement disorders: Nystagmus; Vestibulo-ocular reflex (VOR) Postural and gait dysfunction |
| Midline (Vermis; paleocerebellum) |
Truncal & gait ataxia |
| Hemisphere (Neocerebellum) |
Limb ataxia: Dysmetria, Dysdiadochokinesis, "Intention" tremor Dysarthria Hypotonia |
| Afferent defect: Mossy fibers Vestibulopontine pathway Spinocerebellar system (Clarke) via: Inferior cerebellar peduncle |
Dysmetria Impaired stance & gait Information about body parts in space |
| Vestibulocerebellar system Vestibular nucleus to Flocculonodular lobe |
Situation & location of head |
| Outflow pathways: Dentate nucleus | Postural instability Limb ataxia |
| Inferior olive | Disturbed teaching error signals Ataxia developing over time: Especially trained limb movements (writing) |
Ataxias: Comparative Pathology 2
| Disease | Dorsal root ganglion |
Clarke's column |
Vestibular nucleus |
Pontine nucleus |
Granule cells |
Purkinje cells |
Dentate nucleus |
Red nucleus |
Inferior olive |
| DRPLA | + | +++ | + | ||||||
| SCA6 | + | +++ | ++ | + | |||||
| SCA31 | +++ | ||||||||
| MSA | + | +++ | + | +++ | |||||
| SCA2 | ++ | +++ | +++ | +++ | ++ | + | +++ | ||
| SCA1 | ++ | + | + | ++ | ++ | ++ | ++ | ||
| SCA3 | +++ | ++ | ++ | + | +++ | ||||
| FRDA | +++ | +++ | ++ | + | +++ | ||||
| AOA1 | +++ | +++ | + | +++ | + | + |
