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Acute Intermittent Porphyria: A Rare Cause of Acute Disseminated Encephalomyelitis

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 dia...

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Detalles Bibliográficos
Autores principales: Sheikh, Abdul Ahad E, Sheikh, Abu Baker, Sagheer, Shazib, Tariq, Usman, Bukhari, Marvi M, Fatima, Zainab, Afzal, Rao M
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6141216/
https://www.ncbi.nlm.nih.gov/pubmed/30237948
http://dx.doi.org/10.7759/cureus.2989
Descripción
Sumario: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.