It may also affect the peripheral nervous system, and has features in common with both miller fisher syndrome and guillainbarre syndrome. Know the anticipated clinical course of different types of encephalitis. Bickerstaff s brainstem encephalitis bbe is a rare neurological condition classically characterized by a constellation of signs and symptoms including acute ophthalmoplegia, ataxia, and altered sensorium. A 44yearold man was hospitalized for drowsiness, cognitive complaint limb weakness, ataxia and sensory disturbance after diarrhea. Bickerstaffs encephalitis, hashimotos encephalitis, susacs syndrome, multiple sclerosis, systemic lupus erythematosus, and polychondritis are among autoimmune diseases associated with brain stem encephalitis 3. Chronic bickerstaffs encephalitis with cognitive impairment. Be able to conduct a diagnostic investigation of a patient with encephalitis by using clinical and laboratory criteria. Aug 21, 2014 professional reference articles are designed for health professionals to use. Bickerstaff s brainstem encephalitis bbe is an uncommon nervous system disorder characterized by clinical signs and symptoms.
Bickerstaff s brainstem encephalitis is a clinical syndrome defined by ophthalmoplegia, ataxia, and altered consciousness although other brainstem and long tract signs are often associated bickerstaff and cloake, 1951, odaka et al. Cad includes with or without cabg unless cad is in the graft vessel nosno angina native artery, default i25. An immunologic abnormality common to bickerstaffs brainstem encephalitis and fishers syndrome. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. Pdf a pediatric case of bickerstaffs brainstem encephalitis. Bickerstaff s brainstem encephalitis bbe is a very uncommon central nervous system disease. In the 1950s, bickerstaff and fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia.
Disease bioinformatics bickerstaffs brainstem encephalitis is a rare inflammatory disorder of the central nervous system. Treatment list for bickerstaffs brainstem encephalitis bbe the list of treatments mentioned in various sources for bickerstaffs brainstem encephalitis bbe includes the following list. Ophthalmoplegia has been reported with acute ataxia in miller fisher syndrome mfs and bickerstaff brainstem encephalitis bbe. According to my doc, if it was caught in the first six months, antivirals and blood cleaning could have minimized nerve damage.
Since then, such a condition has been called bickerstaffs brainstem encephalitis bbe. He was initially diagnosed clinically with miller fisher syndrome mfs but later developed limb weakness consistent with guillainbarre syndrome gbs and subsequently reduced consciousness consistent with bickerstaffs brainstem encephalitis bbe. Bickerstaff s brainstem encephalitis bbe is characterised by progressive bilateral ophthalmoparesis and ataxia with impaired consciousness andor pyramidal signs. A diagnosis of viral encephalitis could be a challenge to the clinician, since almost 70% of viral encephalitis cases are left without an etiologic agent identified. Bickerstaffs encephalitis be is an acute postinfectious demyelinating disease with albuminocytological dissociation.
Viral encephalitides are the result of brain parenchymal infection by a number of different viruses, many of which have similar presentations and imaging features. An antigq1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders. Bickerstaff migraine definition of bickerstaff migraine. Encephalitis is a severe inflammatory disorder of the brain with many possible causes and a complex differential diagnosis. Bickerstaff encephalitis is a rare immunemediated condition believed to be one of a number of conditions sharing a similar immunological mechanism, and collectively termed antigq1b igg antibody syndrome. Its been almost six months since jy was admitted to the hospital, 102315. Recurrent diplopia in a pediatric patient with bickerstaff.
A 56yearold man presented with a 3day history of progressive tingling of the hands, unsteadiness, and diplopia. A considerable number of patients with bickerstaff brainstem encephalitis have associated guillainbarre syndrome, indicative that these two disorders are closely related on a continuous spectrum. Bickerstaffs brainstem encephalitis an analysis of. Bickerstaffs brainstem encephalitis bbe, together with miller fisher syndrome mfs and guillainbarre syndrome gbs were considered to form a continuous clinical spectrum. Encephalitis and postinfectiousencephalitis john e. Bickerstaffs brainstem encephalitis is a very rare localized central nervous system inflammatory demyelinating disorder. Bickerstaffs brainstem encephalitis is a clinical syndrome defined by ophthalmoplegia, ataxia, and altered consciousness although other brainstem and long tract signs are often associated bickerstaff and cloake, 1951, odaka et al. A 33yearold woman three weeks after a febrile illness presented with a syndrome of ophthalmoplegia, ataxia and areflexia soaa that characterizes clinically both bickerstaff and miller fisher syndromes. Background bickerstaff brainstem encephalitis bbe is a rare inflammatory, demyelinating disease that generally has a good prognosis objective to describe the course of a patient with severe bbe and multiple medical complications design case report setting academic medical center patient an 81yearold woman with bbe who fully recovered. Refman endnote bibtex refworks excel csv pdf send via email scopus tm citations 17 checked on apr 12. Fisher and bickerstaff, in the 1950s, reported some cases with distinctive features of ophthalmoplegia and ataxia. Bickerstaff brainstem encephalitis pediatric oncall journal. Treatments for bickerstaffs brainstem encephalitis bbe.
It is considered to be a variant of miller fisher syndrome and guillainbarre syndrome but is differentiated from the two by the presence of central nervous system involvement, commonly in the form of impaired consciousness. A case of bickerstaff brainstem encephalitis and acute. Bickerstaff brainstem encephalitis and fisher syndrome. It is estimated that viral encephalitis occurs at a rate of 1. You may find one of our health articles more useful. That finding is further evidence of continuity between bickerstaff brainstem encephalitis and fisher syndrome, a variant of guillainbarre. Bilateral babinski signs were positive, but tests on the rest of the nervous system could not be performed.
They are written by uk doctors and based on research evidence, uk and european guidelines. We sought to characterize clinical presentations, etiologies, response to treatment, and predictors of outcome. Professional reference articles are designed for health professionals to use. However, existing criteria for autoimmune encephalitis are too reliant on.
Severe bickerstaffs encephalitis treated with rituximab. A pediatric case of bickerstaffs brainstem encephalitis. In order to diagnose bickerstaffs brainstem encephalitis, ataxia and ophthalmoplegia must be present. An overlapping case of miller fisher syndrome, bickerstaff. In the cases with bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support. Neuropsychological evaluation showed slowing, memory and executive function.
A case of bickerstaff brainstem encephalitis and acute lymphoblastic leukaemia hasriza hashim1, narisa sulaiman sahari2, sazlyna mohd sazlly lim2, shariful hasan2, fan kee hoo2 summary bickerstaff brainstem encephalitis bbe is a postinfectious immune disorder. A chronic form has rarely been described previously. Rehabilitation of a patient with overlap of acute transverse. Bickerstaff encephalitis is a rare and severe inflammatory disease with a serious presentation, which belongs to the spectrum of.
A clinical approach to diagnosis of autoimmune encephalitis. Guillainbarre syndrome gbs, fisher syndrome fs and bickerstaff brainstem encephalitis represent a spectrum of acute postinfectious immunemediated diseases. Acute complete external ophthalmoplegia is a rare finding in clinical practice that is associated with diseases affecting the neuromuscular junction, the oculomotor nerves, or the brainstem. Bickerstaffs brainstem encephalitis bbe is a very uncommon central nervous system disease. Find out information about bickerstaff brainstem encephalitis. Background bickerstaff brainstem encephalitis bbe is a rare inflammatory, demyelinating disease that generally has a good prognosis. Bickerstaffs brainstem encephalitis, miller fisher. Counter to previous studies with bbe which indicate weakness due to guillainbarre.
This disease is notifiable in the uk see noids article for more details. We here describe a 12 year old girl with bickerstaffs brainstem encephalitis who had multiple cranial nerve palsies. Mri abnormalities suggestive of autoimmune encephalitis. Bickerstaff brainstem encephalitis and guillainbarre syndrome had low muscle tone and weak tendon reflexes in his left arm.
The rest of the body had normal muscle tone and tendon reflexes. We here describe a 12 year old girl with bickerstaff s brainstem encephalitis who had multiple cranial nerve palsies. Bickerstaffs brainstem encephalitis is a rare syndrome defined by the triad of ophthalmoplegia, ataxia and decreased consciousness. Brainstem encephalitis abstract in 1950 decade, bickerstaff described a clinical setting preceded most cases by a febrile disease, that since its beginning shows evidence of participation of the brain stem, followed by a stationary period of 1to3. Abstract clinical features of brainstem encephalitis bbe in 62 cases and a subgroup with guillainbarre syndrome gbs are reported from centers in japan. Bickerstaffs encephalitis be is an acute postinfectious demyelinating disease with albuminocytological. Case report open access chronic bickerstaffs encephalitis with cognitive impairment, a reality. In the case of bickerstaffs encephalitis, serum antigq1b antibodies are generally positive. This has not been reported in children in india before. To characterize the phenotype of pediatric bickerstaffs brainstem encephalitis bbe and evaluate prognostic features in the clinical course, diagnostic studies, and treatment exposures. Bickerstaff migraine basilar migraine a type of classical migraine often associated with the menstrual cycle that first appears in.
Bickerstaff s brainstem encephalitis is a rare syndrome defined by the triad of ophthalmoplegia, ataxia and decreased consciousness. Its etiology remains unknown but it is postulated that bbe has an autoimmunologic origin. Bickerstaff encephalitis radiology reference article. In 1951, bickerstaff and cloake described an encephalitis consisting in an association of ophthalmoplegia, ataxia and consciousness disturbance following an acute infection 14. For viral infection of the meninges, please refer to the general article on viral meningitis, and, for a broad view on the curriculum of infections of the central nervous system, refer to cns. Be familiar with the modalities used to treat encephalitis.
An inservice on bickerstaffs brainstem encephalitis. A case of brainstem encephalitis treated with plasmapheresis. Bickerstaff migraine definition of bickerstaff migraine by. A case of bickerstaffs brainstem encephalitis in childhood khalid hama salih submitted 3rd december 2014. This entity known bickerstaffs encephalitis be is close to guillainbarre syndrome gbs and miller fischer syndrome, with the presence of an. Objective to describe the course of a patient with severe bbe and multiple medical complications. Encephalitis and postinfectiousencephalitis neuroscience. Case report open access chronic bickerstaffs encephalitis.
An elderly patient with bickerstaff brainstem encephalitis. I wanted to give an update on his recovery because when i was searching the web for children recovering from bickerstaff, encephalitis in general, and guillainbarre syndrome, there wasnt a lot of information available. Advances in autoimmune encephalitis research in the past 10 years have led to the identification of new syndromes and biomarkers that have transformed the diagnostic approach to these disorders. Brainstem encephalitis be is an uncommon condition. Encephalitis and postinfectious encephalitis represent two important conditions for the neurologist, both in terms of their presentations as neurologic emergencies and their potential to cause death or serious neurologic impairment. Bickerstaff brainstem encephalitis is a rare inflammatory disorder of the central nervous system, first described by edwin bickerstaff in 1951. The normality of the electrophysiological tests performed, the csf findings and the magnetic resonance images proved that the syndrome stemmed from brainstem pathology. We report on one patient with bickerstaffs brainstem encephalitis bbe and associated flaccid weakness.
Anti gq1b antibody syndrome basal ganglia and brainstem encephalitis, optic neuritis, and radiculomyelitis in epsteinbarr virus infection. Bickerstaff s brainstem encephalitis is a very rare localized central nervous system inflammatory demyelinating disorder. Fisher syndrome and bickerstaff brainstem encephalitis. Encephalitis a syndrome characterized by altered mental status and various combinations of acute fever, seizures, neurologic deficits, cerebrospinal fluid csf pleocytosis, and neuroimaging and electroencephalographic eeg abnormalities, commonly associated with neurotrophic viruses 20 arboviruses causing encephalitis in the united states. Symptoms typically develop following a respiratory or diarrheal illness and serum antigq1b antibodies. An overlapping case of miller fisher syndrome, bickerstaffs. Guillainbarre syndrome, fisher syndrome and bickerstaff. Always seek professional medical advice about any treatment or change in treatment plans.
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