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A rare neurological disorder, Landau-Kleffner Syndrome (LKS), goes by many names: acquired epileptic aphasia, infantile acquired aphasia and aphasia with convulsive disorder. The disorder is “characterized by the sudden or gradual development of aphasia (the inability to understand or express language) and an abnormal electroencephalogram (EEG),” according to the National Institute of Neurological Disorders and Stroke (NINDS).
With LKS, the child has normal language development up until the onset of the disorder, which is between the ages of three and seven. The Iris Cantor Women's Health Center states that signs of auditory agnosia include problems with language comprehension and hearing, and the patient has problems expressing herself through language. The patient may even appear to be developmentally delayed. As a result, the NINDS states that LKS can be difficult to identify, and the patient “may be misdiagnosed [with] autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, mental retardation, childhood schizophrenia, or emotional/behavioral problems."
The New York University Langone Medical Center adds that seizures are another possible symptom of LKS, though they are rare and occur while the patient is asleep. Since most of the seizures occur at night, getting an EEG reading while the patient is sleeping is important in proper diagnosis. The Center notes that the connection between the language problems and seizures is not fully known, though epilepsy may contribute to the loss of language skills. The main type of seizure experienced with LKS is a simple partial motor seizure, which the Merck Manual states does not cause a loss of consciousness, and only affects one cerebral cortex. Tonic-clonic seizures can also occur in some patients, which causes a loss of consciousness. The Iris Cantor Women's Health Center adds that social development, self-sufficiency, academic performance, employment opportunities and personal relationships can also be affected by LKS.
The NINDS notes that prognosis varies: one patient may have severe language problems, while another patient may regain her language abilities later on; prognosis improves if the onset is after age six and if speech therapy is started early. Other treatment options include anticonvulsants and corticosteroids. Multiple subpial transection, a type of surgery that cuts the electrical pathways affected by the disorder, is considered a controversial option.
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Elizabeth Stannard Gromisch received her bachelor’s of science degree in neuroscience from Trinity College in Hartford, CT in May 2009. She is the Hartford Women's Health Examiner and she writes about abuse on Suite 101.
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Landau Kleffner syndrome has been thought to be very rare but, with improved diagnostic techniques, the known prevalence may increase significantly.
March 18, 2011 - 12:07amThis Comment