Severe porphyric neuropathy--importance of screening for porphyria in Guillain-Barré syndrome

South African Medical Journal = Suid-Afrikaanse Tydskrif Vir Geneeskunde
Clara-Maria SchutteNicky M Oosthuizen

Abstract

The hepatic porphyrias are a group of rare metabolic disorders, each of which is associated with a specific enzymatic alteration in the haem biosynthesis pathway. In South Africa (SA), a high incidence of variegate porphyria (VP) is seen as a result of a founder effect, but acute intermittent porphyria (AIP) is also encountered. The development of acute neurovisceral attacks is related to environmental factors, including medications, hormones and diet. A possible manifestation of a severe attack is rapidly progressing quadriparesis, which may mimic Guillain-Barré syndrome. We present four such cases, highlighting that acute porphyria should be considered in the differential diagnosis of Guillain-Barré syndrome. Three patients presented to Steve Biko Academic Hospital, Pretoria, SA, with progressive quadriparesis, and one to a private hospital with acute abdominal pain followed by rapidly progressive quadriparesis. Two patients had started antiretroviral therapy before the development of symptoms, and one had started antituberculosis therapy. All patients had marked weakness with depressed reflexes, and showed varying degrees of confusion. An initial diagnosis of Guillain-Barré syndrome led to administration of intravenous immun...Continue Reading

Citations

Mar 5, 2017·La Revue de médecine interne·L Magy
Oct 3, 2017·Continuum : Lifelong Learning in Neurology·Peter D Donofrio
Aug 14, 2019·The International Journal of Neuroscience·Yiji SuhSardar Ali Khan
Jul 13, 2020·Journal of Inherited Metabolic Disease·Cezar Gavrilovici, Jong M Rho
Jan 13, 2021·The Journal of International Medical Research·Shiqian HuangYin Yuan
Apr 1, 2021·Muscle & Nerve·Rachana K Gandhi MehtaHerbert L Bonkovsky
Jun 18, 2019·Case Reports in Neurology·Mohammed AlqwaiflyDubravka Dodig
Oct 5, 2021·The American Journal of the Medical Sciences·Karl E AndersonHerbert L Bonkovsky

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