ABSTRACT
This autosomal recessive condition is manifested by stimulus-sensitive myoclonus, generalized tonicclonic seizures and partial seizures with visual auras. Onset is during late childhood/adolescence. The clinical course involves progressive dementia, dysarthria and ataxia until death in the teens or early twenties. Pathologically, the condition is characterized by neuronal inclusions (Lafora bodies; Figure 4.34) in the cerebellar cortex, brain stem nucleus and spinal cord.
