Definition: A form of stimulus sensitive myoclonic epilepsy inherited as an autosomal recessive condition. The most common presenting feature is a single seizure in the second decade of life. This is followed by progressive myoclonus, myoclonic seizures, tonic-clonic seizures, focal occipital seizures, intellectual decline, and severe motor and coordination impairments. Most affected individuals do not live past the age of 25 years. Concentric amyloid (Lafora) bodies are found in neurons, liver, skin, bone, and muscle (From Menkes, Textbook of Childhood Neurology, 5th ed, pp111-110)
Other names Lafora Myoclonic Epilepsy; Disorder, Lafora Body; Disease, Lafora Body; Disease, Lafora; Body Disorder, Lafora; Progressive Myoclonic Epilepsy, Lafora; Myoclonic Epilepsy of Lafora; Late Onset Lafora Body Disease; Lafora-Body Disease, Late Onset; Lafora Type Progressive Myoclonic Epilepsy; Lafora Progressive Myoclonic Epilepsy; Lafora Body Disorder; Lafora Body Disease, Late Onset; Lafora Body Disease; Epilepsy Progressive Myoclonic 2; Progressive Myoclonic Epilepsy, Lafora Type; Epilepsy, Progressive Myoclonic, Lafora