Generic placeholder image

Cardiovascular & Hematological Agents in Medicinal Chemistry


ISSN (Print): 1871-5257
ISSN (Online): 1875-6182

Systematic Review Article

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

Author(s): Enrico A. Barletta*, Telmo A. B. Belsuzarri, Augusto R. B. Urena and Eduardo A. Iunes

Volume 19 , Issue 1 , 2021

Published on: 10 September, 2020

Page: [3 - 7] Pages: 5

DOI: 10.2174/1871525718666200910162000

Price: $65


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.

Graphical Abstract
Besur, S.; Schmeltzer, P.; Bonkovsky, H.L. Acute porphyrias. J. Emerg. Med., 2015, 49(3), 305-312.
[] [PMID: 26159905]
Lin, C.S.; Park, S.B.; Krishnan, A.V. Porphyric neuropathy. Handb. Clin. Neurol., 2013, 115, 613-627.
[] [PMID: 23931806]
Yandel, M.L.; Watters, M.R. Treatment of complex partial status epilepticus unmasking acute intermittent porphyria in a patient with resected anaplastic glioma. Clin. Neurol. Neurosurg., 1995, 97(3), 261-263.
[] [PMID: 7586862]
Cerbino, G.N.; Gerez, E.N.; Varela, L.S.; Melito, V.A.; Parera, V.E.; Batlle, A.; Rossetti, M.V. Acute intermittent porphyria in Argentina: An update. BioMed Res. Int., 2015, 2015, 946387.
[] [PMID: 26075277]
Schmitt, C.; Lenglet, H.; Yu, A.; Delaby, C.; Benecke, A.; Lefebvre, T.; Letteron, P.; Paradis, V.; Wahlin, S.; Sandberg, S.; Harper, P.; Sardh, E.; Sandvik, A.K.; Hov, J.R.; Aarsand, A.K.; Chiche, L.; Bazille, C.; Scoazec, J.Y.; To-Figueras, J.; Carrascal, M.; Abian, J.; Mirmiran, A.; Karim, Z.; Deybach, J.C.; Puy, H.; Peoc’h, K.; Manceau, H.; Gouya, L. Recurrent attacks of acute hepatic porphyria: Major role of the chronic inflammatory response in the liver. J. Intern. Med., 2018, 284(1), 78-91.
[] [PMID: 29498764]
Edel, Y.; Mamet, R. Porphyria: What is it and who should be evaluated? Rambam Maimonides Med. J., 2018, 9(2), e0013.
[] [PMID: 29553924]
Pischik, E.; Kauppinen, R. Neurological manifestations of acute intermittent porphyria. Cell. Mol. Biol., 2009, 55(1), 72-83.
[PMID: 19268005]
Yang, J.; Zhu, T.; Zhao, Y.; Yu, X.; Zhu, H.; Jiang, Y.; Li, X. Acute intermittent porphyria in the North of China: The acute attack effect on quality of life and psychological condition. BioMed Res. Int., 2018, 2018, 3216802.
[] [PMID: 29862261]
Kuo, H.C.; Huang, C.C.; Chu, C.C.; Lee, M.J.; Chuang, W.L.; Wu, C.L.; Wu, T.; Ning, H.C.; Liu, C.Y. Neurological complications of acute intermittent porphyria. Eur. Neurol., 2011, 66(5), 247-252.
[] [PMID: 21986212]
Altintoprak, A.E.; Ersel, M.; Bayrakci, A. An unusual suicide attempt: A case with psychosis during an acute porphyric attack. Eur. J. Emerg. Med., 2009, 16(2), 106-108.
[] [PMID: 19262206]
Tran, T.P.; Leduc, K.; Savard, M.; Dupré, N.; Rivest, D.; Nguyen, D.K. Acute porphyria presenting as epilepsia partialis continua. Case Rep. Neurol., 2013, 5(2), 116-124.
[] [PMID: 23898283]
Kochar, D.K.; Pal, M.; Kochar, S.K.; Vyas, A.; Kochar, A.; Bindal, D.; Agrawal, R.P. Acute intermittent porphyria presenting with neurological emergency: Review of six cases. Neurol. India, 2007, 55(4), 413-415.
[] [PMID: 18040123]
Patell, R.; Dosi, R.; Joshi, H.; Shah, P.; Tripathi, R. Challenges in the successful management of a case of acute intermittent porphyria in India. Trop. Doct., 2016, 46(3), 150-153.
[] [PMID: 26472549]
Dagens, A.; Gilhooley, M.J. Acute intermittent porphyria leading to Posterior Reversible Encephalopathy Syndrome (PRES): A rare cause of abdominal pain and seizures. BMJ Case Rep., 2016, 2016, bcr2016215350.
[] [PMID: 27277587]
O'Malley, R; Rao, G; Stein, P; Bandmann, O. Porphyria: Often discussed but too often missed. Pract Neurol., 2018.
Zhang, J.; Hu, Y.; Zheng, J.; Gao, J.; Hou, H.; Liu, N.; Wang, Y. Treatment of acute intermittent porphyria during pregnancy and posterior reversible encephalopathy syndrome after delivery: A case report. Exp. Ther. Med., 2017, 14(6), 5554-5556.
[] [PMID: 29285091]
Periasamy, V.; Al Shubaili, A.; Girsh, Y. Diagnostic dilemmas in acute intermittent porphyria. A case report. Med. Princ. Pract., 2002, 11(2), 108-111.
[] [PMID: 12123102]
Zheng, X.; Liu, X.; Wang, Y.; Zhao, R.; Qu, L.; Pei, H.; Tuo, M.; Zhang, Y.; Song, Y.; Ji, X.; Li, H.; Tang, L.; Yin, X. Acute intermittent porphyria presenting with seizures and posterior reversible encephalopathy syndrome: Two case reports and a literature review. Medicine (Baltimore), 2018, 97(36), e11665.
[] [PMID: 30200061]
Olivier, P.; Van Melkebeke, D.; Honoré, P.J.; Defreyne, L.; Hemelsoet, D. Cerebral vasospasm in acute porphyria. Eur. J. Neurol., 2017, 24(9), 1183-1187.
[] [PMID: 28695690]

Rights & Permissions Print Export Cite as
© 2022 Bentham Science Publishers | Privacy Policy