Familial Lafora body disease of late onset: Report of four cases in one family and a review of the literature

Lafora body disease is one cause of progressive myoclonus epilepsy, It typically presents in the second decade with generalized seizures, myoclonus and then intellectual decline. Death is usual within 10 years, Diagnosis may be made by biopsy of skin, muscle, liver or brain, We present four siblings whit were normal until their mid-twenties. bur then developed intellectual decline, followed by myoclonus. Although a rare from of Lafora body disease has been de scribed that follows a more benign course, may be uf later onset, and whose pathology is subtly different, this family is so far unique in terms of the late presentation of otherwise typical Lafora body disease.