title: An atypical Bickerstaff's brainstem encephalitis with involvement of spinal cord.
abstract: Bickerstaff brainstem encephalitis (BBE) is a rare neurological disease that generally has a good prognosis. We describe an atypical case of a patient with severe BBE; the presentation was uncommon because of the lack of ophthalmoplegia and because of evidence of both peripheral neuropathy and brainstem encephalitis. The article reports clinical and biochemical evaluation and focuses on magnetic resonance imaging (MRI) findings in diagnosis and management of the patient. Notably, we found a previously unreported dramatic spinal cord involvement on MRI. We believe these findings could add to diagnostic tools, and that this case may represent a new variant of BBE with more aggressive behavior.
authors.affiliation: Department of Cardiology-Neurology, Section of Neuroradiology, Neurological Functional Area, San Donato Hospital, Italy leonicuneo@yahoo.it.
authors.forename: Gian Luca
authors.initials: GL
authors.lastname: Cuneo
authors.affiliation: Department of Medical, Surgical and Neuro Sciences, Diagnostic Imaging. University of Siena, Azienda Ospedaliera Universitaria Senese, Italy.
authors.forename: Irene
authors.initials: I
authors.lastname: Grazzini
authors.affiliation: Department of Cardiology-Neurology, Neurological Functional Area, San Donato Hospital, Arezzo, Italy.
authors.forename: Martina
authors.initials: M
authors.lastname: Guadagni
authors.affiliation: Department of Cardiology-Neurology, Neurological Functional Area, San Donato Hospital, Arezzo, Italy.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Venturini
authors.affiliation: Department of Cardiology-Neurology, Neurological Functional Area, San Donato Hospital, Arezzo, Italy.
authors.forename: Amedeo
authors.initials: A
authors.lastname: Bianchi
mesh_terms: D000368:Aged; D018949:Antigens, CD1 / Q000378:metabolism; D001259:Ataxia / Q000000981:diagnostic imaging / Q000209:etiology*; D001323:Autoantibodies / Q000378:metabolism; D001933:Brain Stem / Q000000981:diagnostic imaging / Q000473:pathology*; D004660:Encephalitis / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology*; D005260:Female; D005732:Gangliosides / Q000276:immunology; D006801:Humans; D007091:Image Processing, Computer-Assisted; D008279:Magnetic Resonance Imaging
chemical_list: D018949:Antigens, CD1; D001323:Autoantibodies; D005732:Gangliosides; C039129:GQ1b ganglioside
keywords: Bickerstaff’s brainstem encephalitis; Guillain–Barré syndrome; MRI; Miller Fisher syndrome; anti-GQ1b antibody