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Ataxia with Vitamin E Deficiency with Predominant Cervical Dystonia

Ataxia with vitamin E deficiency (AVED) is a rare autosomal recessive neurodegenerative disorder. It is secondary to a mutation in the gene encoding the alpha-tocopherol transfer protein (TTPA), resulting in impaired transportation of vitamin E out of the liver. A prompt diagnosis of AVED is crucial to neurological recovery and halting the progression of this disorder. Without treatment, most patients with disease onset during childhood (age < 18 years) become wheelchair bound within 5 to 20 years of disease onset. Classically, this disorder presents with ataxia or head titubation as it is a primary movement disorder. Cervical dystonia has seldom been described as the initial presentation of AVED. We present a case of 3 siblings with predominant cervical dystonic tremor diagnosed with AVED.