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Key Features

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  • Most common cause of acute childhood ataxia

  • Accounts for about 40% of all cases

  • Occurs most commonly in children aged 2–6 years

  • Symptoms may include refusal to walk due to ataxia, in addition to sudden development of a wide-based, drunken gait

  • Families may not report unsteadiness of arm movements, ataxia of the trunk, or dysarthria, but these symptoms are essential to localization

  • Serious causes include cns infections and intracranial mass lesions

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Clinical Findings

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  • Onset is abrupt

  • Evolution of symptoms is rapid

  • In about 70% of patients, a prodromal illness occurs with fever, respiratory or gastrointestinal symptoms, or an exanthem within 3 weeks of onset.

  • Associated viral infections include varicella, rubeola, mumps, echovirus infections, poliomyelitis, infectious mononucleosis, and influenza.

  • Bacterial infections such as scarlet fever and salmonellosis have also been incriminated.

  • Mental status is normal in these patients, as is sensory and reflex testing.

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Diagnosis

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  • Cerebrospinal fluid opening pressure, protein, and glucose levels are typically normal, though a mild pleocytosis with lymphocytic predominance can be seen

  • Any significant elevation in white blood count and protein level should prompt an evaluation for meningitis or encephalitis

  • CT and MRI scans are typically normal

  • Occasionally focal cerebellar or cerebellopontine demyelinating lesions or enhancement of the meninges can be seen

  • Decreased regional blood flow in the cerebellum on SPECT without abnormal foci on MRI of the brain has also been reported

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Treatment

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  • Supportive

  • Intravenous immunoglobulin (IVIg) has been used

  • Corticosteroid use does not result in any improvement

  • About 80–90% of patients recover without sequelae within 6–8 weeks, though some may demonstrate residual behavioral changes, learning problems, eye movement abnormalities, and speech problems

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