Landau-Kleffner syndrome

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Landau-Kleffner syndrome (LKS), also called infantile acquired aphasia, acquired epileptic aphasia[1] or aphasia with convulsive disorder, is a rare, childhood neurological syndrome.

It is named for William Landau and Frank Kleffner, who characterized it in 1957.[2]

It is characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and an abnormal electroencephalogram (EEG).[3] LKS affects the parts of the brain that control comprehension and speech. The disorder usually occurs in children between the ages of 5 and 7 years. Typically, children with LKS develop normally but then lose their language skills. While many of the affected individuals have clinical seizures, some only have electrographic seizures, including electrographic status epilepticus of sleep (ESES).

The syndrome can be difficult to diagnose and may be misdiagnosed as autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, mental retardation, childhood schizophrenia, or emotional/behavioral problems.

Treatment for LKS usually consists of medications, such as anticonvulsants[4] and corticosteroids[5] (such as prednisone),[6] and speech therapy, which should be started early.

A controversial treatment option involves a surgical technique called multiple subpial transection[7] in which multiple incisions are made through the cortex of the affected part of the brain (pia mater), severing the axonal tracts in the subjacent white matter.

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