| |
Landau Kleffner syndrome - Landau Kleffner syndrome Symptom, Cause, Treatment
Landau Kleffner Syndromes is a rare form of childhood epilepsy, which results in a severe language disorder. This syndrome languished as a relatively rare neurologcal entity until quite recently. Landau-Kleffner syndrome (LKS), also called acquired epileptiform aphasia, is a rare, childhood neurological disorder characterized by the sudden or gradual development of aphasia (loss of language) and an abnormal electro-encephalogram (EEG). A syndrome characterized by the onset of isolated language dysfunction in otherwise normal children (age of onset 4-7 years) and epileptiform discharges on ELECTROENCEPHALOGRAPHY. Seizures, including atypical absence (EPILEPSY, ABSENCE), complex partial (EPILEPSY, COMPLEX PARTIAL), and other types may occur.
Symptoms of Landau Kleffner syndrome
Some children lose their speech completely. Children with this condition show not only difficulty in understanding what is said to them (a receptive dysphasia) but may also have difficulty in putting their thoughts into words (an expressive dysphasia). In some rare cases, a child may be able to understand language, but have difficulty speaking. Some of the symptoms of Landau-Kleffner Syndrome incude:
- Early signs may be referred to as auditory agnosia, which includes the child:
- suddenly having problems understanding what is said.
- appearing to have problems with hearing - deafness may be suspected.
- appearing to be autistic or developmentally delayed.
- Spoken language is eventually affected, which may lead to complete loss of the ability to speak.
- Some children develop their own method of communicating, such as with gestures or signs.
- Aphasia
- Abnormal electro-encephalogram (EEG)
- Seizures
Causes of Landau Kleffner syndrome
The cause of LKS is unknown. Some experts think there is more than one cause for this disorder. All of the children with LKS appear to be perfectly normal until their first seizure or the start of language problems. There have been no reports of children who have a family history of LKS. Therefore, LKS is not likely to be an inherited disorder. Othe causes are
- Most cases of AEA do not have a well-defined cause.
- A few cases of secondary AEA have been described.
- Low-grade brain tumors, closed-head injury, neurocysticercosis, and demyelinating disease have been associated with the clinical picture of AEA.
- CNS vasculitis may also be associated with AEA.
- Hyperactivity and decreased attention span are common in AEA. Patients in whom attention deficit disorder is suspected should be carefully examined for aphasia. The resemblance of AEA and attention deficit disorder is superficial, and differentiation of the 2 should not be difficult.
- Speech in children with the syndrome slowly declines over time. There are epilepsy-related abnormalities on the EEG, even though some of the children do not have seizures.
- Landau-Kleffner syndrome, a rare disorder, causes children to develop the inability to speak.
- Children with LKS have abnormal electrical brain waves that can be documented by an electroencephalogram (EEG). LKS may also be called infantile acquired aphasia, acquired epileptic aphasia or aphasia with convulsive disorder.
- Oromotor apraxia and speech problems may be congenital, or they may develop or worsen with episodes of sustained spike and wave discharges during sleep.
- Developmental dysphasia is a syndrome in which language acquisition does not occur despite normal intelligence and the lack of brain or hearing pathology.
Treatment of Landau Kleffner syndrome
Treatment may include medication for seizures, convulsions, and language ability. Sign-language instruction may also be suggested.
- your age, overall health, and medical history
- extent of the disease
- your tolerance for specific medications, procedures, or therapies
- expectations for the course of the disease
- your opinion or preference
- Most children in the early stages will need treatment with anti-epileptic drugs for the partial seizures
- Appropriate speech and language therapy and individual educational assessment are essential for children with LKS.
- Multiple subpial transection surgery.
|
|
|
