Landau-Kleffner syndrome: A case report

Abstract

Objective

To contribute to the growing body of literature on Landau-Kleffner syndrome (LKS) by detailing its clinical and imaging features, therapeutic approaches, and clinical outcomes.

Case description

5-year-old, female, presented with sleep-related seizures beginning at age 3. At 5, she exhibited psychomotor regression, including loss of drawing skills, language regression, spatial disorientation, and disruptive behaviors such as aggression and attempts to flee. Initial treatment with phenobarbital and oxcarbazepine failed to control seizures. Sleep EEGs revealed a continuous spike-and-wave pattern, and cranial MRI was unremarkable. Based on these findings, LKS was diagnosed. Oxcarbazepine was discontinued, phenobarbital was maintained, and corticosteroid therapy with prednisolone (2 mg/kg/day) was introduced. After one month, she showed significant clinical improvement, including seizure cessation, improved motor function, and partial recovery of cognitive and visuospatial abilities. Subsequent EEGs showed persistent but reduced epileptiform activity. Prednisolone was gradually tapered, and clobazam (20 mg/day) was introduced. She has remained seizure-free for six months, with ongoing speech improvement.

Comments

LKS is a rare pediatric neurological disorder characterized by acquired aphasia and epilepsy, often with electrographic abnormalities during sleep. Its etiology is multifactorial, involving genetic, immunologic, and inflammatory mechanisms. Diagnosis relies on clinical history, neurological exam, and sleep EEG. Management includes a combination of pharmacologic therapies (e.g., corticosteroids, antiepileptics, benzodiazepines) and supportive interventions like speech therapy. This case highlights the favorable clinical response to corticosteroids and clobazam in a child with LKS. Informed consent was obtained from the patient’s guardians.