Acquired epileptiform aphasia

Other names

Landau-Kleffner syndrome (LKS)

Incidence

It is a rare, childhood neurological disorder

Clinical features

Landau-Kleffner syndrome (LKS) is characterized by the sudden or gradual development of aphasia and an abnormal electro-encephalogram (EEG).

EEG abnormalities in LKS

The EEG typcally shows an increase to nearly continuous abnormal brain activity firing (spikes) during sleep that scientists believe impair memory formation.

Part of the brain affected in LKS

LKS affects the parts of the brain that control comprehension and speech, typically affecting understanding rather than expression.

Ages affected

The disorder usually occurs in children between the ages of 5 and 7 years.

Clinical course

Typically, children with LKS develop normally but then lose their language skills for no apparent reason.

Seizures in LKS

While many of the affected individuals have seizures, some do not.

Diagnosis

The disorder can be difficult to diagnose and may be misdiagnosed and should be recognized as different from the more common causes of autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, childhood schizophrenia, or emotional/behavioral problems.

Prognosis

The prognosis for children with LKS varies.
Some affected children may have a permanent severe language disorder, while others may regain much of their language abilities (although it may take months or years).

Remission and relapse

In some cases, remission and relapse may occur. The prognosis is improved when the onset of the disorder is after age 6 and when speech therapy is started early. Seizures generally disappear by adulthood, and the distinctive epilepsy activity on EEG also tends to improve by that time.

Treatment

Treatment for LKS usually consists of anti-seizure medications and corticosteroids, and speech therapy, which should be started early. A controversial treatment option involves a surgical technique called multiple subpial transection in which the pathways of abnormal electrical brain activity are severed.

Acquired epileptiform aphasia Resources
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