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길랑 바레 증후군으로 오인된 중증의 감각운동 다발성 신경병증 환자에서 급성 간헐 포르피린증 선별의 중요성: 증례 보고

Importance of Screening Acute Intermittent Porphyria in Severe Sensorimotor Polyneuropathy Misdiagnosed as Guillain-Barre Syndrome: A Case Report

대한근전도전기진단의학회지 2020년 22권 2호 p.86 ~ 89
손우철, 김자영, 김대열,
소속 상세정보
손우철 ( Son Woo-Chul ) - University of Ulsan College of Medicine Asan Medical Center Department of Rehabilitation Medicine
김자영 ( Kim Ja-Young ) - University of Ulsan College of Medicine Asan Medical Center Department of Rehabilitation Medicine
김대열 ( Kim Dae-Yul ) - University of Ulsan College of Medicine Asan Medical Center Department of Rehabilitation Medicine

Abstract


Acute intermittent porphyria (AIP) is a rare genetic disorder. Its clinical manifestations include abdominal pain, neurological symptoms, and sensorimotor polyneuropathy. Herein we report the case of a 23-year-old woman who first presented with abdominal pain, followed by severe sensorimotor polyneuropathy, which was first reckoned as Guillain-Barre Syndrome (GBS) or critical illness polyneuropathy (CIP). Her clinical findings and laboratory data corresponded to AIP, which can cause severe peripheral axonal polyneuropathy, that is, porphyric neuropathy. On the basis of a review of the present case, we confirmed that porphyric neuropathy should be considered as a differential diagnosis in patients with an acutely progressing severe symmetric sensorimotor polyneuropathy with particular similarities to GBS or CIP because considerable recovery can be expected in patients with porphyric neuropathy who receive an early diagnosis and treatment.

키워드

Porphyria; Neuropathy; Guillain-Barre Syndrome; Critical illness polyneuropathy

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