O'Malley Ronan, Rao Ganesh, Stein Penelope, Bandmann Oliver
Department of Neurology, Sheffield Teaching Hospitals (STH), Sheffield, UK.
Department of Neuroscience, University of Sheffield, Sheffield, UK.
Pract Neurol. 2018 Oct;18(5):352-358. doi: 10.1136/practneurol-2017-001878. Epub 2018 Mar 14.
The diagnosis of acute intermittent porphyria (AIP) is often overlooked. We describe a patient with this condition who had all the 'bells and whistles', in whom the diagnosis was only made after considerable delay. Far from an esoteric condition haunting examination candidates, AIP is an important cause of a broad spectrum of neurological symptoms. Its early recognition allows the astute clinician to prevent potentially devastating sequelae. We provide practical guidance on the investigation and management of this complex disorder. With a 'back to basics' approach to the underlying genetics and biochemistry, we hope to dispel some of the confusion that may obstruct a timely diagnosis.
急性间歇性卟啉病(AIP)的诊断常常被忽视。我们描述了一位患有此病且症状典型的患者,其诊断被耽搁了很久。AIP并非困扰应试者的罕见病症,而是导致广泛神经症状的重要病因。早期识别有助于敏锐的临床医生预防潜在的严重后遗症。我们为这种复杂病症的检查和管理提供实用指导。通过回归潜在遗传学和生物化学的基础知识,我们希望消除一些可能阻碍及时诊断的困惑。