PT  - JOURNAL ARTICLE
AU  - Jonathan Cleaver
AU  - Richard James
AU  - Claire M Rice
TI  - Rhomboencephalitis
AID  - 10.1136/practneurol-2020-002680
DP  - 2020 Dec 08
TA  - Practical Neurology
PG  - practneurol-2020-002680
4099  - http://pn.bmj.com/content/early/2020/12/08/practneurol-2020-002680.short
4100  - http://pn.bmj.com/content/early/2020/12/08/practneurol-2020-002680.full
AB  - 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.