Lamotrigine (LTG) long-term anticonvulsant therapy was evaluated in 29 patients, aged 6-28 years (mean, 14 years), with juvenile neuronal ceroid lipofuscinosis (JNCL), followed for 1-6 years (mean, 3 years) at the Hospital for Children and Adolescents, University of Helsinki, Finland. An initial dose of 0.1-0.5 mg/kg/day was increased every 2 weeks up to a maintenance dose of 1.25-15 mg/kg/day. After 1 year, seizures were decreased by more than 50% in 10, and seizures became less severe in 9 of 22. General patient well-being was improved in 18 of 28. LTG monotherapy was continued in 13 of 19 patients. [1]
COMMENT. The classical form of JNCL has an onset between 5 and 8 years with visual loss followed by progressive psychomotor retardation, epilepsy beginning at 8 to 13 years, extrapyramidal signs, and behavior disorders. Seizures are usually generalized tonic-clonic and partial, occasionally absence or atonic, and in the final stages of the disease, sometimes myoclonic. Lamotrigine may be an effective anticonvulsant in patients with JNCL.