A familial progressive autosomal recessive form of epilepsy beginning in childhood and characterized by convulsions, myoclonic jerking, abnormal EEG, resulting in difficulties in voluntary movement and mental deterioration leading to dementia. Onset usually in childhood between 6 and 19 years of age. Symptoms commonly peak at about the age of 15 years. Lafora’s bodies are present in the nervous system, and sometimes in the retina, heart, muscle, and liver. Average time of survival six years. Inheritance is autosomal recessive.It is a clinico-pathologic form of Unverricht’s disease.
- G. A. Schwartz, M. Yanoff:
Lafora's disease. Distinct clinico-pathologic form of Unverricht’s syndrome.
Archives of Neurology, Chicago, February 1965, 12: 172-188.