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- W1968973106 abstract "Brain stem auditory (BAERs), visual (VEPs) and somatosensory evoked responses (SEPs) were recorded in 12 patients with Pelizaeus-Merzbacher leukodystrophy (PMD), three with adrenoleukodystrophy (ALD) and three with metachromatic leukodystrophy (MLD). All the 3 evoked responses were abnormal in all patients except normal VEPs and SEPs in a patient with early ALD. In most patients wave I with and without wave II were the only components of the BAERs that remained, subsequent components (waves III–VII) were absent. VEPs were severely altered; either no identifiable response to flash or pattern reversal stimuli could be recorded or the major components were significantly delayed in latency. Short latency SEPs following median nerve stimulation usually showed a normally recorded Erb's potential (N10), but an absence or marked attenuation of cervical (N14) and the early scalp components (N19 and P22) or the occurrence of the scalp components with a significant delay. Multimodality evoked responses provide more information regarding the functional integrity of several afferent systems in patients with white matter disorders. Les réponses évoquées auditives du tronc cérébral (BAER), visuelles (VEP) et somatosensorielles (SEP) ont été enregistrées chez 12 patients atteints de leukodystrophie de Pelizaeus-Merzbacher (PMD), 3 patients avec adréno-leukodystrophie (ALD) et 3 patients avec leukodystrophie métachromatique (MLD). Les 3 types de réponses évoquées sont anormales chez tous les patients, à l'exception de VEP et SEP normaux chez un malade atteint de ALD récente. Chez la plupart des patients, l'onde I avec ou sans l'onde II sont les seules composantes des BAER qui demeurent, les composantes successives (ondes III–VII) étant absentes. Les VEP sont très fortement altérés; ou bien aucune réponse identifiable aux éclair ou aux renversement de damier ne peut être enregistrée, ou bien les composantes principales ont une latence retardée de façon significative. Les SEP de courte latence consécutifs à la stimulation du nerf médian montrent habituellement un potentiel d'Erb normalement enregistré (N10) mais soit l'absence ou l'atténuation marquée des composantes cervicales (N14) et des composantes précoces sur le scalp (N19 et P22), soit la survenue avec un retard significatif des composantes de scalp. Les réponses évoqées à plusieurs modalités fournissent plus d'informations concernant l'intégrité fonctionnelle de plusieurs systèmes afférents chez des malades atteints de troubles de la substance blanche." @default.
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- W1968973106 date "1982-07-01" @default.
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- W1968973106 title "Brain stem auditory, visual and somatosensory evoked potentials in leukodystrophies" @default.
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- W1968973106 doi "https://doi.org/10.1016/0013-4694(82)90229-2" @default.
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