EEG changes and epilepsy in developmental dysphasia.
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Biomedical subjects
Publications and source records attributed to O Dlouhá.
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Four children, 3 boys and 1 girl, with the syndrome of acquired aphasia Landau-Kleffner were followed up during the last 10 years. In 3 children an episodic form of the disease with a good response to corticosteroid treatment and with a favourable prognosis was observed. In the other 1 no improvement of speech function was observed and also epileptic fits were reduced only with difficulty. A positive autoimmune reaction to central (MOZAN) and peripheral (LISAN) myelin was observed repeatedly during the attacks of clinical worsening. On the contrary, during the periods of improvement of language disturbances during the treatment with corticosteroids this hypersensitivity to central as well as peripheral myelin disappeared. Possible changes in myelinization and the role of autoimmune reactions in the etiopathogenesis of the disease are discussed.
The authors demonstrate a child with Landau-Kleffner's syndrome, i.e. the syndrome of so-called acquired aphasia of child age associated with paroxysmal EEG activity, which was described in 1957 by the above mentioned authors. It is characterized by loss of speech (receptive and expressive function), EEG changes, behavioral disorders, psychomotor disorders and epileptic seizures. In the discussion the authors advocate the term "so-called epileptic aphasia", as at the onset of the disease acoustic agnosia is not involved but rather sensory aphasia with subsequent impairment of the expressive speech component and epileptic EEG activity.
Brain-stem auditory evoked potentials (BAEPs) were tested in 34 patients with hereditary motor and sensory neuropathy (HMSN), most of which with HMSN type I with the autosomal dominant type of inheritance. In only a few cases a recessive or sporadic form of the disease was diagnosed, and one patient was rated as HMSN type III. In 44% complicated forms with signs of CNS involvement (cerebellar manifestation, nystagmus or cranial nerve lesions) were ascertained. BAEPs tests revealed slightly, albeit non-significantly prolonged wave III latency while wave V latency was significantly prolonged and so was the interpeak interval of waves III-V. One third of the records showed a prominent decrease in wave V amplitude.
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