Landau-Kleffner syndrome

Landau-Kleffner syndrome

DiseaseDisorder infobox
Name = Landau-Kleffner syndrome


Caption = Landau-Kleffner syndrome is characterized by aphasia and an abnormal EEG
ICD10 = ICD10|F|80|3|f|80
ICD9 =
ICDO =
OMIM = 245570
DiseasesDB = 31407
MedlinePlus =
eMedicineSubj = neuro
eMedicineTopic = 182
MeshID = D018887

Landau-Kleffner syndrome (LKS), also called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder, is a rare, childhood neurological syndrome characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and an abnormal electroencephalogram (EEG). 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).

Differential diagnosis

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

Treatment for LKS usually consists of medications, such as anticonvulsants and corticosteroids, and speech therapy, which should be started early. A controversial treatment option involves a surgical technique called multiple subpial transection in which multiple incisions are made through the cortex of the affected part of the brain, severing the axonal tracts in the subjacent white matter.

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). 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.

References

* Landau, W., & Kleffner, F. (1957). Syndrome of acquired aphasia with convulsive disorder in children. Neurology, 7, 523-530.
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Look at other dictionaries:

  • Landau-Kleffner syndrome — See under syndrome. * * * Lan·dau Kleff·ner syndrome (lahnґdou klefґnər) [William M. Landau, American neurologist, 20th century; Frank R. Kleffner, American speech pathologist, 20th century] see under syndrome …   Medical dictionary

  • Landau-Kleffner syndrome (LKS) — A disorder with seizures starting in childhood in which the patient loses skills, such as speech, and develops behavior characteristic of autism. A major feature of LKS is the gradual or sudden loss of the ability to understand and use spoken… …   Medical dictionary

  • Landau-Kleffner-Syndrom — Klassifikation nach ICD 10 F80.3 Erworbene Aphasie mit Epilepsie (Landau Kleffner Syndrom) …   Deutsch Wikipedia

  • LKS (Landau-Kleffner syndrome) — A disorder with seizures starting in childhood in which the patient loses skills, such as speech, and develops behavior characteristic of autism. A major feature of LKS is the gradual or sudden loss of the ability to understand and use spoken… …   Medical dictionary

  • Syndrome, Landau-Kleffner (LKS) — A disorder with seizures starting in childhood in which the patient loses skills, such as speech, and develops behavior characteristic of autism. A major feature of LKS is the gradual or sudden loss of the ability to understand and use spoken… …   Medical dictionary

  • Síndrome de Landau-Kleffner — El síndrome de Landau Kleffner se caracteriza por la afasia y un EEG anormal Clasificación y recursos externos CIE 10 …   Wikipedia Español

  • Landau (disambiguation) — Landau may refer to: Vehicles* Landau (carriage) * Landau (automobile)Places* Landau, city in Rhineland Palatinate, Germany * Landau an der Isar, town in Bavaria, Germany * Dingolfing Landau, district in Bavaria, Germany * Petit Landau (… …   Wikipedia

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  • Kleffner — Frank, 20th century U.S. neurologist. See Landau K. syndrome. See Landau K. syndrome …   Medical dictionary

  • syndrome — The aggregate of symptoms and signs associated with any morbid process, and constituting together the picture of the disease. SEE ALSO: disease. [G. s., a running together, tumultuous concourse; (in med.) a concurrence of symptoms, fr. syn,… …   Medical dictionary

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