Bickerstaff brain stem encephalitis (BBE) is a rare brainstem disorder characterized by acute onset of ophthalmoplegia, ataxia, and altered consciousness. Guillain Barre syndrome (GBS), Miller Fischer syndrome and BBE share certain similarities such as the presence of anti-ganglioside antibodies. The use of Therapeutic Plasma Exchange (TPE) has been reserved for severe to fulminant cases of BBE mostly as an 'off label' use. The role of TPE in the overlapping syndrome of BBE and GBS has not been explored much, especially in the paediatric population. Herein, we describe a case of 2-year-old male who presented with features of BBE and later evolved to an overlapping syndrome with BBE and GBS. A multi-disciplinary team managed the patient and TPE was initiated as a part of the treatment plan. Five cycles of TPE were done from day 24 after which the patient improved. In our case, TPE was used as rescue therapy in patients with BBE overlapping with GBS. The effectiveness of TPE can be further explored as a modality in such disorders.
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http://dx.doi.org/10.1016/j.transci.2021.103260 | DOI Listing |
Cureus
September 2024
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo, JPN.
A 68-year-old man developed diplopia, unsteady walking, and bladder and bowel dysfunction followed by consciousness disturbance within four weeks. On physical examination, consciousness disturbance, bilateral ptosis, ophthalmoplegia, disappearing of doll's eye phenomenon, dysarthria, and diminished deep tendon reflexes were observed. Cerebrospinal fluid (CSF) examination showed oligoclonal bands.
View Article and Find Full Text PDFNeurol Sci
December 2024
Neurology Unit, University Hospital "San Giovanni di Dio e Ruggi d'Aragona", University of Salerno, Salerno, Italy.
Introduction: Since the initial identification of Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE),significant milestones have been achieved in understanding these diseases.Discoveries of common serum antibodies (IgG anti-GQ1b), antecedent infections, neurophysiological data, andneuroimaging suggested a shared autoimmune pathogenetic mechanism rather than distinct pathogenesis, leadingto the hypothesis that both diseases are part of a unified syndrome, termed "Fisher-Bickerstaff syndrome". The subsequent identification of atypical anti-GQ1b-positive forms expanded the classification to a broader condition known as "Anti-GQ1b-Antibody syndrome".
View Article and Find Full Text PDFCureus
June 2024
Intensive Care, Blackpool Victoria Hospital, Blackpool, GBR.
A woman in her 60s initially presented with rapid-onset left-sided hemiparesis with later development of slurred speech and left-sided facial droop. Despite ruling out common causes, her condition rapidly progressed with the development of bilateral proximal weakness, ophthalmoplegia, ataxia, and pyramidal signs eventually leading to a cardiorespiratory arrest. Extensive investigations, including computerised tomography (CT), magnetic resonance imaging (MRI), and lumbar puncture (LP), were negative for infectious or vascular aetiologies.
View Article and Find Full Text PDFRadiology
May 2024
From the Department of Radiology, Division of Neuroradiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Ave, Boston, MA 02115 (M.D.S.); and Department of Diagnostic Imaging, Division of Neuroradiology, The Hospital for Sick Children, Toronto, Ontario, Canada (B.B.E.W.).
Cureus
April 2024
Neurology, Institute for Neurosciences, St. Luke's Medical Center, Quezon City, PHL.
Bickerstaff brainstem encephalitis (BBE) is a rare disorder that is characterized by ophthalmoplegia, ataxia, and disturbance in consciousness. Definite diagnosis is made primarily through clinical presentation and serology testing with anti-GQ1b antibody. However, in a country where access to serologic testing is scarce, electrophysiologic tests such as brainstem auditory evoked response (BAER) may contribute to the diagnosis.
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