What is Acquired Aphasia with Epilepsy (Landau-Kleffner Syndrome)?
It is a regression of speech skills after a period of normal speech development. The main features are sensorimotor aphasia, epileptic EEG changes in the form of multifocal spikes and spike-wave complexes (the temporal parts of the brain are interested, most often bilaterally) and epileptic seizures.
Causes of Acquired Aphasia with Epilepsy (Landau-Kleffner Syndrome)
In most cases, unknown. Presence of an encephalitic process. In 12% of children with Landau-Kleffner syndrome, cases of epilepsy are identified in a family history. Instrumental methods of research (pneumoencephalography, CT, arteriography) do not reveal morphological abnormalities. A brain biopsy and serological studies give mixed results and do not confirm the presence of specific encephalopathy.
Symptoms of Acquired Aphasia with Epilepsy (Landau-Kleffner Syndrome)
Typically, the onset is at the age of 3-7 years, but can occur earlier and later. At the onset of the disease, a relatively slowly progressive violation of speech understanding is observed. From a neuropsychological point of view, auditory verbal agnosia develops. In the future, disorders of expressive speech join a violation of understanding of speech. Spontaneous speech disappears within a few weeks or months. Often there is a complete loss of speech. The operational side of thinking remains intact. In 50% of children with Landau-Kleffner syndrome, behavioral disorders are detected, primarily hyperdynamic syndrome. Clinically, epileptic seizures occur in only 70% of cases. In 1/3 of cases, a single seizure or epileptic status is noted at the onset of the disease. After reaching 10 years of age, seizures are observed only in 20% of patients, and after 15 years they stop. Multiple bilateral high-amplitude spikes and spike-wave complexes, most pronounced in the temporal regions, are recorded on the EEG. With age, epileptic manifestations in the EEG become less noticeable and disappear in all patients by the age of 15-16. In adolescence, there is a slight improvement in speech. However, with sensorimotor aphasia, speech is not fully restored. The prognosis of speech recovery depends on the age of the manifestation and the start time of antiepileptic therapy and reconstructive speech therapy classes.
Most children with this disorder more often fall into the field of vision of clinicians not even about seizures and, especially, not speech disorders, but about behavioral disorders – “disinhibition”, hyperkinesis. Neurophysiologists point out that EEG is the only pathognomonic criterion for identifying the syndrome at those stages when proper treatment can still save speech, although the prognosis is poor.
Treatment of Acquired Aphasia with Epilepsy (Landau-Kleffner Syndrome)
The positive effect of taking corticosteroids at the onset of the disease is expected. Throughout the disease, anticonvulsants are recommended. The first choice drugs are carbamazepines (finlepsin), the second choice – lamictal (lamotrigine). Speech therapy and family therapy are recommended throughout the disease.