Landau Kleffner Syndrome

September 22, 2015

Landau–Kleffner syndrome (LKS) is a rare childhood neurological syndrome. It has several synonym names including: infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder.  This rare disorder has been reported only 160 times from 1957-1990.  This syndrome was first described in 1957 by Dr. William M. Landau and Dr. Frank R. Kleffner, who identified six children with the disorder. Let’s examine the sign, symptoms, causes and possible treatments of this heartbreaking childhood disorder.

General Characteristics – The major characteristic of LKS is the gradual or sudden loss of the ability to understand and use spoken language. This usually occurs most frequently in normally developing children who are between 3 and 7 years of age. Without warning children with this disorder begin having trouble understanding what is said to them and then progresses many times to the child being unable to use spoken language.

Symptoms –

  • Approximately 80 percent of the children with LKS have one or more epileptic seizures that usually occur at night.
  • Hearing difficulties that can come on suddenly or develop slowly
  • A significant minority of children with LKS also develop serious behavioral dysfunction, including hyperactivity, temper outbursts, or withdrawn behaviors but rarely the severe social impairments seen in autism.
  • Landau-Kleffner syndrome is a rare disorder that affects twice as many males as females.

Causes – The cause of Landau-Kleffner syndrome is unknown although a single patient has been found to have a mutation in the GRIN2A gene and other candidate genes including RELN, BSN, EPHB2 and NID2 have been suggested. The response in some patients to immunosuppression has raised the question of autoimmune and other inflammatory mechanisms as potential contributors. (Source: National Organization for Rare Disorder)

Prognosis and Treatment – 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.(Source: National Institute of Neurological Disorders and Stroke)

There have not been many long-term follow-up studies of children with LKS. This lack of evidence, along with the wide range of differences among affected children, makes it impossible to predict the outcome of this disorder. Complete language recovery has been reported; however, language problems usually continue into adulthood. (Source: Medicine.Net) Medication to control the seizures and abnormal brain wave activity (anticonvulsants) usually has very little effect on language ability. Corticosteroid therapy has improved the language ability of some children. Sign language instruction has benefited others.