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2020 Sep 10

Cardiovasc Hematol Agents Med Chem

Acute Neurological Manifestations of Porphyrias and its Types: A Systematic-Review.


Barletta E A, Belsuzarri T A B, Urena A R B, Iunes E A
Cardiovasc Hematol Agents Med Chem. 2020 Sep 10.
PMID: 32914723.


Acute porphyrias cause life-threatening attacks of neurovisceral non-specific symptoms, so this condition mimics many acute medical and psychiatric diseases. The disease is very misdiagnosed, probably due to its low incidence and nonpathognomonic symptoms, this delays the effective treatment onset. Early diagnosis and treatment improve highly the prognosis and can prevent the development of neuropathic manifestations. We assembled a systematic review, following the PRISMA guidelines and using Pubmed as our database. Our aim is to show some peculiarities among patients that present neurological manifestations in acute porphyria attack. We have obtained the patients age, sex, clinical presentation, neurological manifestations and porphyria type out of 16 patients. We also evaluated the time between symptoms onset and neurological manifestations. The average age was 28,4 ± 11,1; 50% of patients were male. AIP was the most prevalent porphyria type. The average time between symptoms onset and neurological manifestations was of 9,53 ± 11,6 days. Abdominal pain; nausea and vomiting and psychiatric manifestations were the most common symptoms preceding neurological attacks. Seizures and consciousness disturbance were the most prevalent findings within an attack. We are also presenting a case to illustrate how difficult this diagnosis can be.