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Porphyric neuropathy.
- Source :
-
Muscle & nerve [Muscle Nerve] 2021 Aug; Vol. 64 (2), pp. 140-152. Date of Electronic Publication: 2021 Mar 31. - Publication Year :
- 2021
-
Abstract
- Acute hepatic porphyrias are inherited metabolic disorders that may present with polyneuropathy, which if not diagnosed early can lead to quadriparesis, respiratory weakness, and death. Porphyric neuropathy is an acute to subacute motor predominant axonal neuropathy with a predilection for the upper extremities and usually preceded by a predominantly parasympathetic autonomic neuropathy. The rapid progression and associated dysautonomia mimic Guillain-Barré syndrome but are distinguished by the absence of cerebrospinal fluid albuminocytologic dissociation, progression beyond 4 wk, and associated abdominal pain. Spot urine test to assess the porphyrin precursors delta-aminolevulinic acid and porphobilinogen can provide a timely diagnosis during an acute attack. Timely treatment with intravenous heme, carbohydrate loading, and avoidance of porphyrinogenic medications can prevent further neurological morbidity and mortality.<br /> (© 2021 Wiley Periodicals LLC.)
- Subjects :
- Aminolevulinic Acid metabolism
Guillain-Barre Syndrome mortality
Guillain-Barre Syndrome pathology
Humans
Peripheral Nervous System Diseases diagnosis
Radial Nerve pathology
Peripheral Nervous System Diseases mortality
Peripheral Nervous System Diseases pathology
Polyneuropathies mortality
Polyneuropathies pathology
Porphobilinogen Synthase deficiency
Porphyrias, Hepatic mortality
Porphyrias, Hepatic pathology
Subjects
Details
- Language :
- English
- ISSN :
- 1097-4598
- Volume :
- 64
- Issue :
- 2
- Database :
- MEDLINE
- Journal :
- Muscle & nerve
- Publication Type :
- Academic Journal
- Accession number :
- 33786855
- Full Text :
- https://doi.org/10.1002/mus.27232