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AHPs are genetically inherited disorders that result from heme biosynthesis enzyme deficiencies and comprise four forms: acute intermittent porphyria (AIP), variegate porphyria (VP), hereditary coproporphyria (HCP), and ALA\u2010dehydratase porphyria (ALADP). Depending on the clinical presentation, the main differential diagnoses are Guillain\u2010Barr\u00e9 syndrome and autoimmune encephalitis. Red flags that could raise the suspicion of acute porphyria are neurological symptoms starting after severe (abdominal) pain, in association with reddish urine, hyponatremia or photodermatitis, and the presence of encephalopathy and\/or axonal neuropathy. We highlight the diagnostic difficulties by presenting three cases from our neurological intensive care unit and give a comprehensive overview about the diagnostic findings in imaging, electrophysiology, and neuropathology.<\/jats:p>","DOI":"10.1002\/brb3.2389","type":"journal-article","created":{"date-parts":[[2021,10,21]],"date-time":"2021-10-21T00:50:24Z","timestamp":1634777424000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":49,"title":["Acute porphyrias \u2013 A neurological perspective"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5746-068X","authenticated-orcid":false,"given":"Lea M.","family":"Gerischer","sequence":"first","affiliation":[{"name":"Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin, Department of Neurology  Berlin Germany"},{"name":"Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin, NeuroCure Clinical Research Center  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Franziska","family":"Scheibe","sequence":"additional","affiliation":[{"name":"Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin, Department of Neurology  Berlin Germany"},{"name":"Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin, NeuroCure Clinical Research Center  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Astrid","family":"N\u00fcmann","sequence":"additional","affiliation":[{"name":"Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin, Department of Neurology  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martin","family":"K\u00f6hnlein","sequence":"additional","affiliation":[{"name":"Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin, Department of Neurology  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ulrich","family":"St\u00f6lzel","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine II, Porphyria Center Saxonia Klinikum Chemnitz gGmbH  Chemnitz Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreas","family":"Meisel","sequence":"additional","affiliation":[{"name":"Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin, Department of Neurology  Berlin Germany"},{"name":"Charit\u00e9 \u2013 Universit\u00e4tsmedizin Berlin, corporate member of Freie Universit\u00e4t Berlin and Humboldt\u2010Universit\u00e4t zu Berlin, NeuroCure Clinical Research Center  Berlin Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,10,17]]},"reference":[{"key":"e_1_2_14_2_1","doi-asserted-by":"publisher","DOI":"10.2214\/ajr.162.5.8166013"},{"key":"e_1_2_14_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.20137"},{"key":"e_1_2_14_4_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003\u20104819\u2010142\u20106\u2010200503150\u201000010"},{"key":"e_1_2_14_5_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2796.2002.01020.x"},{"key":"e_1_2_14_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF00690977"},{"key":"e_1_2_14_7_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1913147"},{"key":"e_1_2_14_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.880170715"},{"key":"e_1_2_14_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/mus.23367"},{"key":"e_1_2_14_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF03004917"},{"key":"e_1_2_14_11_1","doi-asserted-by":"publisher","DOI":"10.1007\/s13312-010-0152-9"},{"key":"e_1_2_14_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1528-1167.2007.01518_2.x"},{"key":"e_1_2_14_13_1","first-page":"432","article-title":"Sudden bilateral reversible vision loss: A rare presentation of acute intermittent porphyria","volume":"62","author":"Bhuyan S.","year":"2014","journal-title":"JAPI"},{"key":"e_1_2_14_14_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0317167100012221"},{"key":"e_1_2_14_15_1","first-page":"1650","article-title":"Angiographic demonstration of reversible cerebral vasospasm in porphyric encephalopathy","volume":"16","author":"Black K. 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