A muscle biopsy done to evaluate for ragged red fibers in a patient with progressive myoclonic epilepsy unexpectedly showed prominent inflammation and perifascicular atrophy. Brain biopsy demonstrated Lafora bodies. Lafora body disease is a progressive, fatal myoclonic epilepsy presenting usually in the first or second decade with mental regression, generalized and myoclonic seizures,1,2 ataxia, spasticity, and involuntary movement. It is an autosomal recessive3 disorder localized to chromosome 6q. Phenytoin is herein implicated in the pathogenesis of dermatomyositis.
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