Landau–Kleffner syndrome

Landau–Kleffner syndrome
Classification and external resources
Landau–Kleffner syndrome is characterized by aphasia and an abnormal EEG
ICD-10	F80.3
ICD-9	345.8
OMIM	245570
DiseasesDB	31407
eMedicine	neuro/182
MeSH	D018887

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]

Presentation

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 (Broca's area and Wernicke's area)). 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^[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 beneath the pia mater, 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

External links

• "Landau–Kleffner syndrome information page". National Institute of Neurological Disorders and Stroke. 2007-02-13. http://www.ninds.nih.gov/disorders/landaukleffnersyndrome/landaukleffnersyndrome.htm. Retrieved 2007-08-23. 
• "Landau–Kleffner syndrome". National Institute on Deafness and other Communication Disorders. 2002. http://www.nidcd.nih.gov/health/voice/landklfs.htm. Retrieved 2007-08-23. 
• Rotenberg J, Pearl PL (2003). "Landau–Kleffner syndrome". Arch Neurol 60 (7): 1019–21. doi:10.1001/archneur.60.7.1019. PMID 12873863. http://archneur.ama-assn.org/cgi/content/full/60/7/1019.