title: Rhombencephalitis: a series of 97 patients.
abstract: The term rhombencephalitis refers to inflammatory diseases affecting the hindbrain (brainstem and cerebellum). Rhombencephalitis has a wide variety of etiologies, some of them potentially severe and life threatening without proper early treatment. In this retrospective observational study, we reviewed the records of consecutively hospitalized patients at University Hospital of Bellvitge (Barcelona, Spain) from January 1990 to December 2008. Rhombencephalitis was defined as a brainstem and/or cerebellar condition demonstrated clinically or by neuroimaging, with pleocytosis (>4 cells/mm) in cerebrospinal fluid. Ninety-seven patients (48 female; mean age, 37 yr; range, 14-79 yr) fulfilled these criteria. We reviewed their clinical, cerebrospinal fluid, and radiologic characteristics. The mean follow-up was 5 years (range, 0-20 yr). The etiologies of rhombencephalitis were as follows: unknown cause (n = 31), multiple sclerosis (n = 28), Behçet disease (n = 10), Listeria monocytogenes infection (n = 9), paraneoplastic syndrome (n = 6) (3 cases associated with anti-Yo antibodies and 3 with anti-Tr antibodies), Epstein-Barr virus (n = 4), tuberculosis (n = 2), pneumococcal infection (n = 2), systemic lupus erythematosus (n = 1), lymphoma (n = 1), Brucella species infection (n = 1), JC virus (n = 1), and relapsing polychondritis (n = 1). Certain clinical, cerebrospinal fluid, and radiologic characteristics that are commonly seen in some of these etiologies can guide us in the first approach to the etiologic diagnosis of rhombencephalitis.
authors.affiliation: From the Department of Neurology (MM, SMY, FR, TxA), Institut de Diagnòstic per la Imatge (CM), and Department of Infectious Diseases (PFV), University Hospital of Bellvitge, IDIBELL, Barcelona, Spain.
authors.forename: Mireia
authors.initials: M
authors.lastname: Moragas
authors.forename: Sergio
authors.initials: S
authors.lastname: Martínez-Yélamos
authors.forename: Carles
authors.initials: C
authors.lastname: Majós
authors.forename: Pedro
authors.initials: P
authors.lastname: Fernández-Viladrich
authors.forename: Francisco
authors.initials: F
authors.lastname: Rubio
authors.forename: Txomin
authors.initials: T
authors.lastname: Arbizu
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D015331:Cohort Studies; D003131:Combined Modality Therapy; D004569:Electroencephalography / Q000379:methods; D004660:Encephalitis / Q000134:cerebrospinal fluid / Q000453:epidemiology* / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D015994:Incidence; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D012249:Rhombencephalon / Q000473:pathology*; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D013030:Spain / Q000453:epidemiology; D015996:Survival Rate; D055815:Young Adult