Unknown

Dataset Information

0

Neuromyelitis optica spectrum disorders.


ABSTRACT: Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Long segments of spinal cord inflammation (myelitis), severe optic neuritis, and/or bouts of intractable vomiting and hiccoughs (area postrema syndrome) are classic presentations of the disease and may alert the clinician to the diagnosis. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first attack. Unlike multiple sclerosis, a progressive clinical course is very unusual and the accrual of disability is related to relapses. Approximately 75% of patients have antibodies against aquaporin-4, a water channel expressed on astrocytes. Relapses are treated aggressively to prevent residual disability with high-dose steroids and often plasma exchange. Relapse prevention is crucial and achieved with long-term immunosuppression. In this article we review the pathogenesis, clinical features, diagnosis and management of NMOSD.

SUBMITTER: Huda S 

PROVIDER: S-EPMC6454358 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature

altmetric image

Publications

Neuromyelitis optica spectrum disorders.

Huda Saif S   Whittam Dan D   Bhojak Maneesh M   Chamberlain Jayne J   Noonan Carmel C   Jacob Anu A  

Clinical medicine (London, England) 20190301 2


Neuromyelitis optica spectrum disorder (NMOSD) is an uncommon antibody-mediated disease of the central nervous system. Long segments of spinal cord inflammation (myelitis), severe optic neuritis, and/or bouts of intractable vomiting and hiccoughs (area postrema syndrome) are classic presentations of the disease and may alert the clinician to the diagnosis. Untreated, approximately 50% of NMOSD patients will be wheelchair users and blind, and a third will have died within 5 years of their first a  ...[more]

Similar Datasets

| S-EPMC8444436 | biostudies-literature
| S-EPMC4515040 | biostudies-literature
| S-EPMC4720663 | biostudies-literature
| S-EPMC7481470 | biostudies-literature
| S-EPMC3781161 | biostudies-literature
| S-EPMC4841645 | biostudies-literature
| S-EPMC6487334 | biostudies-literature
| S-EPMC6117869 | biostudies-other
| S-EPMC6217894 | biostudies-literature
| S-EPMC5819570 | biostudies-literature