What is Landau Kleffner Syndrome?

Landau Kleffner Syndrome is not only a rare disease, but one that can be very hard to diagnose, given its tendency to mimic other disorders. This condition affects children between the ages of 3 and 6, and is characterized by the onset of sudden or gradual aphasia, or the inability to express or understand language.  What makes Landau Kleffner Syndrome even worse is that it isn’t like children who have it weren’t born with language skills; as it turns out, for all intents and purposes they have developed normally until they experience the symptoms of this condition, then and only then do they lose their comprehension and speech skills.

Another sign of Landau Kleffner Syndrome is an irregular electro-encephalogram, and patients who are affected by this disease may or may not have seizures as well. Though a seizure may trigger the symptoms, there may be other catalysts such as a simple illness like chicken pox, or trauma, for example falling from a changing table. Landau Kleffner Syndrome is often mistaken for autism, pervasive developmental disorder, hearing impairment, learning disability, auditory/verbal processing disorder, attention deficit disorder, mental retardation, childhood schizophrenia, or emotional/behavioral problems. It may also be misdiagnosed as Asperger’s syndrome.

Landau Kleffner Syndrome can be distinguished from autism, though. Autistic children tend to regress between 18 and 24 months of age; earlier than children with Landau Kleffner Syndrome by at least one full year. More often than not children with Landau Kleffner Syndrome have good eye contact and noticeably try to understand what’s going on around them, as opposed to autistic children who appear unengaged, and display poor eye contact and no language. Approximately two-thirds of children with LKS experience seizures, and two-thirds have behavioral issues; in either case the main symptom is loss of language. This disorder manifests itself in the form of word deafness or verbal auditory agnosia; that is, not being able to understand words that should be familiar.

Additionally, Landau Kleffner Syndrome has been associated with epilepsy; specifically a syndrome known as electrical status epilepticus of sleep. In fact, Landau Kleffner Syndrome usually show spikes on their EEG’s, in particular when they are sleeping. As we can see, this condition is related to several other, better known disorders, which might or might not deprive it from the attention it should be receiving. Fortunately, there are non-profit organizations like Global Genes.org that strive to raise awareness of rare diseases such as this one. You can support the Global Genes Project through Discount Medical Supplies.

Children who are diagnosed with Landau Kleffner Syndrome are usually treated by an entire team of specialists, including a neurologist, a neuropsychologist, and a speech pathologist or audiologist, as well as a child psychologist and a psychopharmacologist. As far as medications go, patients are typically prescribed steroids and anti-epileptic or anti-convulsive drugs. Patients should start speech therapy as soon as they are diagnosed. An intensive language-based speech program yields better results when continued throughout the educational years. There is also a surgical approach in a procedure called multiple subpial transections. Though it may improve EEG abnormalities in a small number of patients, it is still being studied.

Prognosis depends on individual factors, but only 20% of patients still experience seizures after age 10. The course of the disease varies and in some cases it may even recede on its own. On the other hand, language difficulties become permanent in some patients, especially in children in whom medical or surgical treatment does not cancel the epileptic patterns on the EEG.

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