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Acute Intermittent Porphyria: A Rare Cause of Acute Disseminated Encephalomyelitis

机译:急性间歇性卟啉症:急性播散性脑脊髓炎的罕见原因

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Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system (CNS) with no distinct etiology but implications include infections and commonly administered vaccinations. In this case report, we present the case of ADEM in a young female who was subsequently diagnosed with acute intermittent porphyria (AIP) that was the instigator of the initial CNS assault. Our case highlights the peculiar presentation of ADEM which can present as a diagnostic challenge and brings forth AIP as a new and previously unknown affiliate of this rare CNS disease. We also discuss the pathophysiology, diagnostic criteria, and subsequent treatment options for this rare clinical entity.
机译:急性弥漫性脑脊髓炎(ADEM)是中枢神经系统(CNS)的脱髓鞘疾病,病因没有明显区别,但影响范围包括感染和通常接种的疫苗。在本病例报告中,我们介绍了一名年轻女性的ADEM病例,该女性随后被诊断为急性中枢性卟啉症(AIP),是最初的CNS袭击的诱因。我们的案例强调了ADEM的独特表现,它可以作为诊断挑战出现,并提出AIP作为这种罕见的中枢神经系统疾病的新成员,以前未知。我们还讨论了这种罕见的临床实体的病理生理,诊断标准和后续治疗方案。

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