Landau-Kleffner syndrome.

Landau-Kleffner syndrome is characterized by 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 has not been established.

What causes Landau-Kleffner syndrome?

The development and causes of Landau-Kleffner syndrome are unknown. Clinical data suggest the possibility of an encephalitic process. In 12% of children with Landau-Kleffner syndrome, cases of epilepsy are found in the family history. Brain biopsy and serologic studies give ambiguous results and do not allow to confirm the presence of a specific encephalopathy.

How does Landau-Kleffner syndrome manifest itself?

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. In about a quarter of cases, speech skills are lost gradually, over the course of months, but more often their loss occurs abruptly, over several days or weeks. Complete loss of speech is often observed. The operational side of thinking is preserved. Half of the children have behavioral disorders, mainly of the hyperkinetic syndrome type. With age, epileptic seizures disappear; by the age of 15-16, all patients note some improvement in speech.

How to recognize Landau-Kleffner syndrome?

Diagnostic algorithm (according to ICD-10)

• Significant loss of expressive and receptive language over a period not exceeding 6 months.
• Previous normal speech development.
• Paroxysmal EEG abnormalities involving one or both temporal lobes, detected two years before and two years after initial speech loss.
• Hearing is within normal limits.
• Maintaining the level of non-verbal intelligence within normal limits.
• Absence of any diagnosable neurological condition other than EEG abnormalities and epileptic seizures.
• No pervasive developmental disorder is detected.

Differential diagnostics

• Differentiation from specific receptive language disorder is based on the identification of a period of normal speech development prior to the manifestation of the disease, the detection of paroxysmal EEG abnormalities related to one or both temporal lobes, appearing two years before the initial loss of speech.
• Since most children are observed by psychiatrists due to hyperdynamic syndrome, after the end of seizures there is a need for differential diagnosis with hyperkinetic disorders based on anamnestic data (features of the onset of the disease, dynamics, outcomes), as well as data from clinical and instrumental examination of patients (severity of receptive speech disorders in children with Landau-Kleffner syndrome, presence of paroxysmal EEG abnormalities).

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Treatment

At the onset of the disease, a positive effect from taking glucocorticoids is possible. During the entire disease, anticonvulsants are recommended. The first-choice drugs are carbamazepines, the second - lamotrigine. Speech therapy and family psychotherapy are recommended throughout the disease.

What is the prognosis for Landau-Kleffner syndrome?

The possibility of speech recovery depends on the age of manifestation, the time of the beginning of antiepileptic therapy and speech therapy. In 2/3 of children, a more or less serious defect of receptive speech remains.