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

[ Vol. 19 , Issue. 1 ]

Author(s):

Enrico A. Barletta*, Telmo A. B. Belsuzarri, Augusto R. B. Urena and Eduardo A. Iunes   Pages 3 - 7 ( 5 )

Abstract:


Introduction: 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 non-pathognomonic symptoms, this delays the effective treatment onset. Early diagnosis and treatment highly improve the prognosis and can prevent the development of neuropathic manifestations.

Methods: We assembled a systematic review, following the PRISMA guidelines and using Pubmed as our database. Our aim was to show some peculiarities among patients that present neurological manifestations in acute porphyria attack. We obtained the patients’ age, sex, clinical presentation, eurological manifestations and porphyria type 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.

Results: 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 also presenting a case to illustrate how difficult this diagnosis can be.

Keywords:

Acute, porphyria, neurological, symptoms, attacks, nausea, vomiting.

Affiliation:

Department of Medicine, Pontifical Catholic University of Campinas, São Paulo, Neurosurgery Department, Pontifical Catholic University of Campinas, Campinas, Cardiologist Department, Pontifical Catholic University of Campinas, Campinas, Neurosurgery Department, Federal University of Sao Paulo, Sao Paulo

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