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Abstract
The presentation of a patient with brainstem symptoms and signs invokes a number of common and less common differential diagnoses, and accurate diagnosis can be challenging. We review the major immune-mediated and inflammatory syndromes that can affect the brainstem including multiple sclerosis, neuromyelitis optica spectrum disorder, neuro-Behçet disease, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, neurosarcoidosis, Susac syndrome, and the histiocytic disorders. We focus on clinical features and MRI clues that help to distinguish among the different brainstem conditions. Accurate diagnosis is important to guide appropriate treatment and limit neurologic disability.
Glossary
- ACE=
- angiotensin-converting enzyme;
- ADC=
- apparent diffusion coefficient;
- ADEM=
- acute disseminated encephalomyelitis;
- AQP4=
- aquaporin-4;
- BD=
- Behçet disease;
- BBE=
- Bickerstaff brainstem encephalitis;
- CLIPPERS=
- chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids;
- DWI=
- diffusion-weighted imaging;
- ECD=
- Erdheim Chester disease;
- FLAIR=
- fluid-attenuated inversion recovery;
- IgG=
- immunoglobulin G;
- INO=
- internuclear ophthalmoplegia;
- LCH=
- Langerhans cell histiocytosis;
- LETM=
- longitudinally extensive transverse myelitis;
- MOG=
- myelin oligodendrocyte glycoprotein;
- MS=
- multiple sclerosis;
- NBD=
- neuro-Behçet disease;
- NMOSD=
- neuromyelitis optica spectrum disorder;
- NS=
- neurosarcoidosis;
- SLE=
- systemic lupus erythematosus;
- SS=
- Susac syndrome;
- SWI=
- susceptibility-weighted imaging
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
- Received December 17, 2018.
- Accepted in final form May 29, 2019.
- © 2019 American Academy of Neurology
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You May Also be Interested in
- Article
- Abstract
- Glossary
- Introduction
- Multiple sclerosis
- NMOSD and myelin oligodendrocyte glycoprotein (MOG) antibody-induced demyelination
- Neuro-Behçet disease
- CLIPPERS
- Neurosarcoidosis
- Susac syndrome
- Histiocytic disorders
- Other inflammatory and immune-mediated disorders in which the brainstem can be involved
- Discussion
- Study funding
- Disclosure
- Appendix Authors
- Footnotes
- References
- Figures & Data
- Info & Disclosures
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