To determine the relation between developmental dysphasia and EEG anomalies during sleep, we compared 52 subjects with dysphasia with a control group of 20 children by using the ambulatory EEG method. Whereas 50% of the children with dysphasia experienced paroxysmal activity (PA), only two of the control group did. It is likely that paroxysmal abnormalities and language impairment are related to architectural dysplasia and neuron‐migration disturbances. PA is frequent in subjects with receptive developmental dysphasia and may be the cause of language deterioration. When the occurrence of paroxysmal abnormalities during sleep is higher than 8% of total sleep time, we suggest the use of antiepileptic drugs.
BackgroundCongenital central hypoventilation syndrome (CCHS) is a rare disease characterized by severe central hypoventilation due to abnormal autonomic control of breathing. The PHOX2B gene, mutations of which define the disease, is expressed in a group of nuclei located in brainstem areas. Pathways controlling breathing and auditory pathways traverse very similar anatomic structures. In the present study, we measure brainstem auditory evoked potentials (BAEP) to assess auditory pathways in CCHS and investigate to which extent brainstem auditory pathways are also affected.MethodsBAEPs were measured in 15 patients with PHOX2B mutations positive CCHS (8 boys and 7 girls. mean age 6.3 yrs ± 5) as part of their regular follow-up in the Centre of reference for central hypoventilation (Robert Debré University Hospital. Paris. France).ResultsBAEP responses were found normal in nine patients (60% of the study group) and abnormal in the other six (40%). Abnormal BAEPs which resulted from brainstem dysfunction were found in three patients (20%).ConclusionDysfunction of brainstem auditory pathways can be observed in CCHS. However, auditory evoked responses can be normal in the disease, therefore suggesting much more complex yet-to-be determined interactions between pathways and functions of central control of breathing and of control of hearing.
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