Abstracts

Rationale: The goal of antiepileptic therapy is to provide the most effective seizure control with minimal side-effects. There is a special caution for patients with juvenile myoclonic epilepsy (JME) and liver disease, because of hepatotoxic potential of valproate most often used as antiepileptic drug (AED) of choice.Methods: A group of 12 children and adolescents aged 4.5 to 18.5 years (mean 14.2) with JME and hepatic disorder was studied for antiepileptic management. Nine patients were successfully treated with valproate (25-45 mg/kg) for 3.2 to 5.5 years before the onset of liver disease. In addition, JME was newly diagnosed in 3 adolescents with ongoing hepatic disease. Results: Primary sclerosing cholangiitis and autoimmune hepatitis in one and chronic hepatitis B (HBV) in two patients (recipients of blood products), were diagnosed before the JME onset. Stable long-term myoclonic and generalized tonic-clonic seizure (GTCS) control was achieved with topiramate (4 and 7 mg/k) or levetiracetam (28 mg/kg) in them, with no hepatic side effects for at least 12 months. Recurrences followed by marked elevation of serum ALT, AST, gama-GT levels, mildly increased protrombine time and decreased albumine levels developed in 9 patients with acute viral hepatitis A (3), HBV(2) and infectious mononucleosis (4). Valproate was withdrawn in all patients soon after the diagnosis of liver disease was made and switched to topiramate (4), levetiracetam (3) or lamotrigine (2). Topiramate or lamotrigine were efficacious for absences in 4 patients but failed for myoclonic jerks in 2/9 patients. GTC seizures which reappeared in 6/9 patients concurrently with liver dysfunction, were completely controlled with new AEDs. After the clinical and biochemical recovery (4 to 8 months) valproate was reintroduced in 4 patients, with no hepatic side-effects during the clinical follow-up. Conclusions: Children and adolescents with JME and liver diseases are a challenge for therapeutic decision providing acceptable long-term hepatic safety profile and efficacy in control of various seizures.