Nonepileptic visual hallucinations in Lafora disease

Lafora disease (LD) is a fatal form of progressive myoclonus epilepsy, transmitted as an autosomal recessive trait. LD presents with seizures in late childhood or adolescence, after a period of normal development. Myoclonus and seizures become intractable within 2 years of onset, along with progressive cognitive decline. Most patients are in a vegetative state with continuous myoclonus prior to death, which usually occurs within 10 years of first symptoms.1 The epileptic disorder consists of increasing frequency and intractability of different types of focal and generalized seizures. Status epilepticus is common. Occipital-onset seizures have been described to be a prominent seizure type, manifesting as transient blindness, photoconvulsive reactions, or visual hallucinations (VHs).1–6 Because of this, visual manifestations (both simple and complex) in patients with LD are usually considered to be due to occipital seizures and are treated with antiepileptic medication. Here we report the case of a patient with LD due to mutations in the recently isolated EPM2B gene, who developed complex VHs that were not a manifestation of occipital seizures and who responded well to antipsychotic medication. The patient was well …