Landau-Kleffner syndrome

Disease Overview

Landau-Kleffner syndrome (LKS) is a rare neurological syndrome characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and recurrent seizures (epilepsy). Children with LKS typically develop normally until signs and symptoms of the syndrome begin to develop between age 2 and 8 years. Males are more often affected by LKS than females. In about 20% of people with LKS, mutations (changes) in the GRIN2A gene have been identified. The syndrome is inherited in an autosomal dominant manner. In other cases, the syndrome may be caused by changes to other unidentified genes. LKS is diagnosed when a doctor sees clinical features that are consistent with the syndrome such as a loss of speech and an electroencephalogram (EEG) that shows specific kinds of seizure activity. Genetic testing can be used to confirm if there is a mutation in GRIN2A, but this testing is not done routinely. Treatment for LKS usually consists of medications such as anticonvulsants and corticosteroids to help prevent seizures. Speech therapy should also be started promptly in order to ensure the best long-term outlook for children with LKS.

Synonyms

• FESD
• LKS
• Landau-Kleffner syndrome
• Rolandic epilepsy, intellectual disability, and speech dyspraxia, autosomal dominant
• Rolandic epilepsy, mental retardation, and speech dyspraxia, autosomal dominant
• acquired aphasia with convulsive disorder
• acquired epileptic aphasia
• acquired epileptiform aphasia
• aphasia, acquired, with epilepsy
• benign epilepsy of childhood with centrotemporal spikes
• continuous Spike and waves during slow-Wave sleep syndrome
• epilepsy, focal, with speech disorder and with or without impaired intellectual development
• epilepsy, focal, with speech disorder and with or without intellectual disability
• epilepsy, focal, with speech disorder and with or without mental retardation