Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. This condition has been named Bickerstaff's brainstem encephalitis BBE. One patient had gross flaccid weakness in the four limbs. Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE.
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Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. This condition has been named Bickerstaff's brainstem encephalitis BBE.
One patient had gross flaccid weakness in the four limbs. Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE. To clarify its clinical features, we reviewed detailed clinical profiles and laboratory findings for 62 cases of BBE diagnosed by the strict criteria of progressive, relatively symmetrical external ophthalmoplegia and ataxia by 4 weeks, and disturbance of consciousness or hyperreflexia.
Ninety-two per cent of the patients involved had had an antecedent illness. Almost all the patients had a monophasic remitting course and generally a good outcome. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: there was perivascular lymphocytic infiltration with oedema and glial nodules. Electrodiagnostic study results suggested peripheral motor axonal degeneration.
These findings confirm that BBE constitutes a clinical entity and provide additional clinical and laboratory features of BBE.
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Chaudhry F, et al. Semin Ophthalmol. Bickerstaff's brainstem encephalitis: can it recur? Sharma V, et al. J Clin Neurosci. Epub Jan Kitazawa K, Yuki N. Kitazawa K, et al. Show more similar articles See all similar articles. Cited by 70 articles Clinical spectrum and diagnostic pitfalls of neurologic syndromes with Ri antibodies. Simard C, et al. Neurol Neuroimmunol Neuroinflamm. Print May. Anti-GQ1b antibody syndrome presenting with visual deterioration as the initial symptom: A case report.
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Adult Actions. Aged Actions. Aged, 80 and over Actions. Chi-Square Distribution Actions. Child Actions. Child, Preschool Actions. Electroencephalography Actions. Electromyography Actions. Female Actions. Humans Actions. Magnetic Resonance Imaging Actions. Male Actions. Middle Aged Actions. Substances Autoantibodies Actions. Gangliosides Actions. Immunoglobulin G Actions. GQ1b ganglioside Actions.
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Bickerstaff's brainstem encephalitis associated with ulcerative colitis
Metrics details. A year-old man acutely developed ophtalmoparesis, ataxia and areflexia suggesting MFS. Preceding infection with Mycoplasma Pneumoniae and anti-GQ1b antibodies were detected. On day 4, nerve conduction study showed reduced or non-recordable compound muscle action potentials CMAPs and sensory nerve action potentials SNAPs without demyelinating features, indicating the electrodiagnosis of acute motor and sensory axonal neuropathy and suggesting a poor prognosis. Intravenous immunoglobulins IVIg were given but clinical status worsened to ophthalmoplegia, tetraplegia and coma needing mechanical ventilation.
Bickerstaff brainstem encephalitis
At about the same time, C. Miller Fisher described cases of ophthalmoplegia and ataxia in a edition of. New England Journal of Medicine. But in contradistinction to BBE, these patients had no change in sensorium and almost universally these patients were areflexic. The majority of patients achieved spontaneous recovery without the need for treatment despite the alarming initial presentation of BBE. The discovery that a large number of affected patients tested positive for the Anti-Gq1b antibody led to a greater understanding of BBE.
Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system ,  first described by Edwin Bickerstaff in In order to diagnose Bickerstaff brainstem encephalitis, ataxia and ophthalmoplegia must be present. These are also diagnostic features of Miller Fisher syndrome , and so Bickerstaff's is only diagnosed if other features are present which exclude Miller Fisher syndrome. These may include drowsiness, coma or hyperreflexia. Like some other autoimmune diseases , the condition usually follows a minor infection, such as a respiratory tract infection or gastroenteritis. The clinical features and course of the condition, the associated auto-antibodies against relevant antigens, and the response to treatment, all suggest that Bickerstaff brainstem encephalitis is an autoimmune disease. However, each of these criteria fails to fit a substantial proportion of patients, and there is no single test or feature which is diagnostic of Bickerstaff brainstem encephalitis.
He later developed hypoesthesia ascending from the lower extremities and consulted the emergency outpatient department with the chief complaint of generalised dysesthesia. Because of a history of ulcerative colitis, his condition was initially treated as acute aggravation of the disease; however, after admission, his consciousness level gradually deteriorated. Physical findings showed weakened tendon reflexes, and anti-GQ1b antibodies were strongly positive in the cerebrospinal fluid. Therefore, the patient was diagnosed with Bickerstaff's brainstem encephalitis BBE. Campylobacter infections are the main cause of BBE, and its incidence is high among patients with ulcerative colitis.