This syndrome is characterized by the progressive loss of speech that develops between the age of 3 and 7 years with or without seizures. It can affect the speaking, understanding of the language or both that can occur suddenly or slowly. This condition can often be misdiagnosed as autism, attention deficit disorder, intellectual disability, behavioral problems or childhood schizophrenia. The prognosis of the affected cases varies with some regaining their language abilities while the others have a permanent language disorder. This rare syndrome is estimated to affect twice as many males as females.
Causes
Landau-Kleffner Syndrome affects the section of the brain responsible for the control of speech and understanding. The exact cause of Landau-Kleffner Syndrome is not known although patients with this condition have presented the mutation in the GRIN2A, EPHB2, RELN, BSN and NID2 genes. In some of the cases, the autoimmune and inflammatory mechanisms have been considered to be the contributing factors of developing this condition.
Symptoms
This syndrome is characterized by the sudden or slow development of aphasia which is the inability of the individual to understand or speak the language. The affected children also present the abnormal electro-encephalogram (EEG). This condition appears to develop among the children between the ages of 2 and 8. Around 70% of the children can also present seizures that are complex partial, generalized clonic and atypical absence which can be controlled and resolved before adolescence. In addition, they can also develop behavioral problems including temper outbursts, attention deficits and hyperactivity. The autism features associated with Landau-Kleffner Syndrome include the poor eye contact, pain insensitivity, sleep problems and aggression.
Diagnosis
As the exact genetic cause has not been identified, the genetic testing for this syndrome is currently not available. The diagnosis is based on the features that are evident of this condition and with the evaluation of an electroencephalogram (EEG) that monitors the electrical activity of the brain. Children with the Landau-Kleffner Syndrome exhibit the abnormal electrical activity of both the sides of the brains. Additional tests can include the magnetoencephalography and an MRI scan of the brain to rule out the possible cause of brain tumors. Other tests can include audiometry and language testing that can evaluate the hearing loss and help with the management of the syndrome.
Treatment
The standard treatment begins with the anti-epileptic medications which are not effective against the language problem. The use of steroids has shown to improve the EEG and the language. The supportive team approach can assist to reestablish the language skills for the affected children. In addition, the speech and language therapy is also very important. Special education may prove to be beneficial for the severely affected children with the language disorders.
References
http://www.epilepsy.com/learn/types-epilepsy-syndromes/landau-kleffner-syndrome
https://rarediseases.info.nih.gov/diseases/6855/landau-kleffner-syndrome
https://rarediseases.org/rare-diseases/landau-kleffner-syndrome/
