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Neuromyelitis optica spectrum disorder presenting as rhomboencephalitis.
BMJ Case Rep 2018; 2018BC

Abstract

Rhomboencephalitis, at least in its acute phase, is often a severely disabling syndrome, and can be life threatening. A range of underlying conditions can lead to this clinical syndrome. Rapid diagnosis to initiate treatment early is key to a beneficial outcome. We report the case of a 22 year old Afro-Caribbean woman, who presented with a two -week history of walking difficulties, upper limb incoordination and slurred speech. Her brainstem function deteriorated at pace, and she developed hypersomnia. A broad diagnostic approach led to prophylactic treatment for the most common infectious causes. This did not improve her symptoms. Non-infectious inflammatory causes were therefore considered and plasma exchange treatment was initiated leading to marked improvement within days. Screening for autoimmune conditions confirmed aquaporin-4 positive neuromyelitis optica spectrum disorder (NMOSD) as the underlying cause. Immunotherapy with rituximab was started. So far, no relapse has been observed. While the definition of NMOSD continues to be refined, aquaporin-4 testing should be considered early in patients presenting with rhomboencephalitis who do not respond to antibiotic and antiviral treatment. Vigilance and early intervention are key to limit morbidity and mortality from NMOSD.

Authors+Show Affiliations

Centre of Clinical Brain Sciences, University of Edinburgh, Edinburgh. Clinical Board: Medicine (Neuroscience), The Royal London Hospital, Barts Health NHS Trust, London, United Kingdom. The Blizard Institute (Neuroscience), Barts and the London School of Medicine & Dentistry, Queen Mary University of London, London, United Kingdom.Clinical Board: Medicine (Neuroscience), The Royal London Hospital, Barts Health NHS Trust, London, United Kingdom. Critical Care, Hammersmith Hospital, Imperial College Healthcare NHS Trust, London, United Kingdom.Haematology Department, University College London, United Kingdom, London.Clinical Board: Medicine (Neuroscience), The Royal London Hospital, Barts Health NHS Trust, London, United Kingdom. The Blizard Institute (Neuroscience), Barts and the London School of Medicine & Dentistry, Queen Mary University of London, London, United Kingdom.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

29794010

Citation

Stavrou, Maria, et al. "Neuromyelitis Optica Spectrum Disorder Presenting as Rhomboencephalitis." BMJ Case Reports, vol. 2018, 2018.
Stavrou M, Francis L, Tshuma N, et al. Neuromyelitis optica spectrum disorder presenting as rhomboencephalitis. BMJ Case Rep. 2018;2018.
Stavrou, M., Francis, L., Tshuma, N., & Schmierer, K. (2018). Neuromyelitis optica spectrum disorder presenting as rhomboencephalitis. BMJ Case Reports, 2018, doi:10.1136/bcr-2017-222255.
Stavrou M, et al. Neuromyelitis Optica Spectrum Disorder Presenting as Rhomboencephalitis. BMJ Case Rep. 2018 May 23;2018 PubMed PMID: 29794010.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Neuromyelitis optica spectrum disorder presenting as rhomboencephalitis. AU - Stavrou,Maria, AU - Francis,Lucy, AU - Tshuma,Nomathamsanqa, AU - Schmierer,Klaus, Y1 - 2018/05/23/ PY - 2018/5/26/entrez PY - 2018/5/26/pubmed PY - 2018/11/1/medline KW - brain stem / cerebellum KW - multiple sclerosis KW - neuroimaging KW - neurological injury KW - neurology JF - BMJ case reports JO - BMJ Case Rep VL - 2018 N2 - Rhomboencephalitis, at least in its acute phase, is often a severely disabling syndrome, and can be life threatening. A range of underlying conditions can lead to this clinical syndrome. Rapid diagnosis to initiate treatment early is key to a beneficial outcome. We report the case of a 22 year old Afro-Caribbean woman, who presented with a two -week history of walking difficulties, upper limb incoordination and slurred speech. Her brainstem function deteriorated at pace, and she developed hypersomnia. A broad diagnostic approach led to prophylactic treatment for the most common infectious causes. This did not improve her symptoms. Non-infectious inflammatory causes were therefore considered and plasma exchange treatment was initiated leading to marked improvement within days. Screening for autoimmune conditions confirmed aquaporin-4 positive neuromyelitis optica spectrum disorder (NMOSD) as the underlying cause. Immunotherapy with rituximab was started. So far, no relapse has been observed. While the definition of NMOSD continues to be refined, aquaporin-4 testing should be considered early in patients presenting with rhomboencephalitis who do not respond to antibiotic and antiviral treatment. Vigilance and early intervention are key to limit morbidity and mortality from NMOSD. SN - 1757-790X UR - https://www.unboundmedicine.com/medline/citation/29794010/Neuromyelitis_optica_spectrum_disorder_presenting_as_rhomboencephalitis_ L2 - http://casereports.bmj.com/cgi/pmidlookup?view=long&pmid=29794010 DB - PRIME DP - Unbound Medicine ER -