Leukoencephalopathy with ataxia, hypodontia, and hypomyelination.

Neurology · 2005

case_series · Level IV

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Abstract

The authors describe four unrelated girls with a distinctive neurologic disorder with early-onset progressive ataxia and hypodontia with a characteristic pattern of delayed dentition. Cerebral MRI shows hypomyelinated white matter and cerebellar atrophy; 1H-MRS of white matter reveals a marked elevation of myo-inositol.