Article Text
Abstract
Rhomboencephalitis—inflammation of the brainstem and cerebellum—has myriad clinical presentations including encephalopathy, cranial neuropathies, long tract signs and cerebellar dysfunction and is associated with significant morbidity and mortality. There are a variety of potential underlying causes that respond variably to treatment, including infections, parainfective syndromes, inflammatory disorders including autoimmune encephalitis and paraneoplastic syndromes. Here, we review its clinical presentation and outline a practical approach to its investigation, aiming to facilitate prompt diagnosis and confirmation of the underlying cause, to start appropriate management early and optimise the clinical outcome.
- infectious diseases
- immunology
- neuroimmunology
- neuropathology
- virology
- neurovirology
Statistics from Altmetric.com
Footnotes
Contributors The manuscript was designed by JC and CMR. All authors contributed to drafting and revision of the manuscript for intellectual content.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Commissioned. Externally peer reviewed by Emma Tallantyre, Cardiff, UK.
Linked Articles
- Editors’ commentary
Read the full text or download the PDF:
Other content recommended for you
- Infectious encephalitis: mimics and chameleons
- Bickerstaff’s brainstem encephalitis associated with anti-GM1 and anti-GD1a antibodies
- Bickerstaff’s brainstem encephalitis mimicking herpetic encephalomyelitis in a liver transplant patient with anti-GQ1b antibodies
- Nationwide survey of patients in Japan with Bickerstaff brainstem encephalitis: epidemiological and clinical characteristics
- Cortical abnormalities on MRI: what a neurologist should know
- Bickerstaff’s brainstem encephalitis related to Campylobacter jejuni gastroenteritis
- Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in limited cutaneous sclerosis: a rare disease combination
- An MRI review of acquired corpus callosum lesions
- Neuromyelitis optica presenting as acute bilateral ptosis
- Multicentre comparison of a diagnostic assay: aquaporin-4 antibodies in neuromyelitis optica