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Landau-Kleffner syndrome (acquired epileptic aphasia) (LKS)

Description

Landau Kleffner syndrome (LKS), which is extremely rare, consists of a sudden or gradual development of language loss or aphasia, affecting both expressive and receptive language. It occurs in children who have been developing normally between the ages of 3 and 7, and is sometimes part of the differential diagnosis of a child who is being considered for a diagnosis of autism or an autistic spectrum disorder. Intelligence does not seem to be affected. Children with LKS have a bilaterally abnormal EEG, especially during sleep, and there is some overlap with the syndrome of continuous spike and wave during slow-wave sleep. Many (80%) children with LKS, but not all, have seizures, usually at night. Many will exhibit behavioral problems, particularly hyperactivity, and aggressiveness and depression. At present, there is not thought to be a genetic component to LKS; the etiology is not known. [NINDS: 2007]

Prognosis

Some children with LKS develop a permanent language disorder, while others do relatively well and regain language skills over time. There is a better prognosis if the affected child at the onset of the disorder is older, and if speech therapy is started promptly after problems begin. Many children with LKS have associated seizures; these usually stop in adolescence.

Treatment

Anticonvulsant medications, steroids, and speech therapy have all been used as treatment for LKS and may be helpful; speech therapy often involves teaching sign language and is the mainstay of treatment. Better outcomes seem to be associated with prompt initiation of speech therapy. Occasionally, a surgery, multiple subpial resection, is used for children who have not responded to other treatments, although it is quite controversial. No controlled studies of outcome with or without various forms of treatment have been performed.

Resources

Helpful Articles

PubMed search for articles on Landau Kleffner Syndrome in children for the last 5 years.

Nieuwenhuis L, Nicolai J.
The pathophysiological mechanisms of cognitive and behavioral disturbances in children with Landau-Kleffner syndrome or epilepsy with continuous spike-and-waves during slow-wave sleep.
Seizure. 2006;15(4):249-58. PubMed abstract

Mikati MA, Shamseddine AN.
Management of Landau-Kleffner syndrome.
Paediatr Drugs. 2005;7(6):377-89. PubMed abstract

Authors

Author: Lynne M Kerr, MD, PhD - 2/2009
Content Last Updated: 6/2011

Page Bibliography

NINDS.
NINDS Landau-Kleffner Syndrome Information Page.
NINDS at the NIH; (2007) http://www.ninds.nig.gov/disorders/landaukleffnersyndrome/landaukleffn....