Landau-Kleffner Syndrome

Landau-Kleffner syndrome is characterized by a regression of speech skills after a period of normal speech development against the background of epileptic changes in the EEG and epileptic seizures.

ICD-10 code

P80.3. Acquired aphasia with epilepsy (Landau-Kleffner).

Epidemiology

The frequency of the disorder is not established.

What causes Landau-Kleffner syndrome?

How the causes of the Landau-Kleffner syndrome develop also are unknown. Clinical data suggest the possibility of an encephalitic process. 12% of children with Landau-Kleffner syndrome have a family history of epilepsy. Brain biopsy and serological tests give mixed results and do not confirm the presence of specific encephalopathy.

How is Landau-Kleffner syndrome manifested?

The onset of the disease is always accompanied by paroxysmal pathology on the EEG and in most cases (up to 70%) epileptic seizures. The first signs appear at the age of 3-7 years. Approximately in a quarter of cases, speech skills are lost gradually over the course of months, but more often their loss occurs abruptly, within a few days or weeks. Often observed complete loss of speech. The operational side of thinking is preserved. In half of the children, behavioral disorders are found, mainly by the type of hyperkinetic syndrome. With age, epileptic seizures disappear, by the age of 15-16 all patients have noted a slight improvement in speech.

How to recognize the Landau-Kleffner syndrome?

Diagnostic algorithm (ICD-10)

• A significant loss of expressive and receptive speech during the periode, which does not exceed 6 months.
• Previous normal development of speech.
• Paroxysmal anomalies of the EEG, related to one or both temporal lobes, are detected two years before and two years after the initial loss of speech.
• The hearing is within normal limits.
• Preservation of the level of non-verbal intellectuality is within the norm.
• The absence of any diagnosed neurologic condition, if not to take into account the anomalies of the EEG and epileptic seizures.
• Do not show a general developmental disorder.

Differential diagnostics

• Differentiation from a specific disorder of receptive speech is based on the observation of the period of normal speech development before the manifestation of the disease, the detection of paroxysmal anomalies of the EEG related to one or both temporal lobes appearing two years before the initial loss of speech.
• Since most children fall under the supervision of psychiatrists in connection with the hyperdynamic syndrome, after the seizures, there is a need for differential diagnostics with hyperkinetic disorders based on anamnestic data (features of onset of the disease, dynamics, outcomes), as well as clinical and instrumental studies of patients (severity of disorders receptive speech in children with Landau-Kleffner syndrome, the presence of paroxysmal anomalies of the EEG).

[1], [2]

Treatment

At the beginning of the disease, a positive effect of taking glucocorticoids is possible. During the whole of the disease, anticonvulsants are recommended. The drugs of the first choice are carbamazepines, the second - lamotrigine. Throughout the disease, it is recommended to conduct speech therapy and family psychotherapy.

What forecast does Landau-Kleffner syndrome have?

The ability to restore speech depends on the age of the manifestation, the time of onset of antiepileptic therapy and speech therapy. In 2/3 of the children, a more or less serious defect in receptive speech persists.