Cerebellar ataxia

Characterized by incoordination of movement and unsteadiness due to cerebellar dysfunction.

Manifested clinically by a gait disorder with imbalance, difficulties with tandem walking, staggering, upper and lower limb dysmetria, dysdiadochokinesia, dysarthria, hypotonia, and saccadic ocular pursuit.

Acquired causes include: stroke, multiple sclerosis, sarcoidosis, primary brain tumors of the posterior fossa, metastatic tumors to the posterior fossa, paraneoplastic syndrome, lead, anticonvulsants, salicylates, 5FU, cytosine arabinoside, sedative treatment, aminoglycosides, HIV infection, viral cerebellitis, Creutzfeldt-Jakob disease, Whipple’s disease, autoimmune thyroiditis, alcoholic cerebeller degeneration, thiamine deficiency, SLE, Sjögren syndrome, Hashimoto’s disease, cerebeller ataxia with antiglutamic acid decarboxylase antibodies and with antigliadin antibodies.

May be an autosomal recessive process.

Acute onset is not suggest a neurodegenerative disease, but does suggest a diagnostic and therapeutic emergency because of related conditions such as a cerebellar abscess, cerebellar stroke, meningitis, vitamin B1 deficiency, and drug toxicity.

Neurodegenerative cerebellar ataxia suggested when the process is progressive and unremitting.

Patients must be evaluated for fever, intracranial hypertension, autonomic dysfunction, MRI analysis of the brain, CSF examination, and blood tests detect specific autoantibodies.

Autosomal dominant spinocerebellar ataxias are Inherited neural degenerative diseases that are usually evident by 35 years of age, and usually with a multi generational pattern.

Autosomal recessive cerebellar ataxia is considered in patients younger than 30 years of age with persistent disorder of gait and balance, or with development of hypotonia or excessive clumsiness.

Autosomal recessive cerebellar ataxias or heterogeneous, complex, neurodegenerative diseases manifested primarily in children and young adults.

Cerebellar ataxia may be associated with peripheral neuropathy, movement disorders, ocular motor abnormalities, pyramidal track dysfunction, mental retardation, cognitive impairment and epilepsy.

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