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Neurophysiological Studies in a Case of Sjögren-Larsson Syndrome

Sjögren-Larsson Syndrome之神經電氣生理學研究:一病例報告

摘要


Sjögren-Larsson Syndrome是一種罕見神經皮膚症候群,肇因於第十七對染色體的異常。此一個案乃一四歲大的男童,表現出典型的皮膚症狀並合併嚴重的發展遲緩及痙攀性四肢麻痺。尤其,關於此症候群的神經電氣生理學方面的研究很少被報告。所以,我們將最近經驗的個案,施行腦電圖,聽覺腦幹誘發電位,視覺誘發電位及體感覺誘發電位等檢查,結果均顯示聽覺,視覺和體感覺之異常。因此推測此症候群可能也合併多種感覺傳導路逕的異常。

並列摘要


We describe a 4-year-old boy with Sjogren-Larsson syndrome (SLS). Clinical neurophysiological studies, including electroencephalography (EEG) and brainstem auditory evoked potential (BAEP), visual evoked potentials (VEP), and short-latency somatosensory evoked potential (SSEP) tests, were performed. The results of multimodality evoked potential tests suggested that SLS may involve dysfunction of various sensory pathways.

並列關鍵字

Sjögren-Larsson Syndrome EEG BAEP VEP SSEP

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