Presentation
Clinical description Patients usually present with onset of diplopia and gait disturbance following upper respiratory or gastrointestinal tract infections. [orpha.net]
Through this case report, we present the clinical course, treatment, recovery, and sequelae of an overlapping MFS, GBS, and BBE syndrome and provide a review of the literature. [ncbi.nlm.nih.gov]
In addition, we present pathological findings for a patient with BBE. [doi.org]
Entire Body System
Respiratoric
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Pharyngitis
BACKGROUND AND PURPOSE: Fisher syndrome (FS) may overlap with Guillain-Barré syndrome (GBS), in particular the pharyngeal-cervical-brachial variant form (PCB-GBS), or Bickerstaff brainstem encephalitis (BBE). [ncbi.nlm.nih.gov]
These include acute ophthalmoparesis and acute ataxic neuropathy, which represent the less extensive spectrum of the disease whereas pharyngeal-cervical-brachial weakness and Fisher syndrome overlap with Guillain-Barré syndrome represent the more extensive [scienceopen.com]
Continuous Spectrum of Pharyngeal-Cervical-Brachial Variant of Guillain-Barre Syndrome. JAMA 2007. 64(10): 1519-23. Sharma V. Bickerstaff’s brainstem encephalitis: can it recur? Journal of Clinical Neuroscience 2006. 12:277-299. [practicalneurology.com]
Isolated, unilateral cranial neuropathies are also reported, and in one study of 100 patients with isolated abducens palsy, 25% were positive for anti-GQ1b antibodies. 1 Pharyngeal-cervical-brachial weakness, affecting oropharyngeal, neck, and shoulder [ahcmedia.com]
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Nasal Discharge
Results Case reports Illustrative case of typical BBE Patient 1 A 29‐year‐old man experienced coughing and nasal discharge, which resolved in 7 days. [doi.org]
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Aspiration
The purpose of this article is to heighten awareness of the potential for silent aspiration in patients with BBE. [ncbi.nlm.nih.gov]
Pavone P...Falsaperla R 2014 27 A pediatric case of Bickerstaff's brainstem encephalitis. ( 25653689 ) Park JY...Kim HJ 2014 28 Atypical Bickerstaff brainstem encephalitis: pitfalls of diagnosis. ( 23953997 ) Wakerley B.R....Yuki N. 2013 29 Silent aspiration [malacards.org]
Neurologic
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Upbeat Nystagmus
There was upbeating nystagmus on upward gaze, and bilateral horizontal gaze‐evoked nystagmus. His pupils were dilated and light reflexes were absent. Bell’s phenomenon and oculocephalic reflexes were preserved. [doi.org]
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Altered Mental Status
mental status; and ataxia. [kjp.or.kr]
In addition to opthalmoplegia, and ataxia, altered mental status (74%), 2 bulbar palsy (34%) 1 and facial diplegia(45%) 2 have all also been noted in the literature. [nature.com]
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Positive Romberg Sign
She had positive Romberg sign and failed the right heel-knee-tibia tests. Her brain diffusion-weighted magnetic resonance imaging (DWI) showed an abnormally high circular signal in the brainstem surrounding the fourth ventricle. [ncbi.nlm.nih.gov]
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Truncal Ataxia
He also developed ballism-like involuntary movements in all extremities, loss of proprioception predominantly on the left, and severe truncal ataxia. [jstage.jst.go.jp]
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Absent Deep Tendon Reflex
Lower extremities remained largely flaccid with absent deep tendon reflexes and Babinski reflexes bilaterally. Discharge ASIA exam demonstrated T6 ASIA impairment scale A paraplegia. [nature.com]
Workup
1439; 1869-1447; 1615-6706; 0939-7116 Deuschl, Cornelius; Göricke, Sophia Luise; Gramsch, Carolin; Özkan, Neriman; Lehnerdt, Götz; Kastrup, Oliver; Ringelstein, Adrian; Wanke, Isabel; Forsting, Michael; Schlamann, Marc Value of DSA in the diagnostic workup [uni-due.de]
The routine diagnostic workup of M. pneumoniae encephalitis should therefore aim for the detection of M. pneumoniae antibodies in both CSF and serum, in addition to M. pneumoniae PCR in CSF. [journals.plos.org]
Microbiology
Jugular Venous Pressure
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Absent A-Waves
F-waves and soleus H-reflexes), and brainstem auditory evoked potentials (prolongation of wave I latency) suggested peripheral abnormalities. [ncbi.nlm.nih.gov]
Treatment
CONCLUSION: Our study identified clinical, radiologic, and treatment features that may hold prognostic value for children with BBE. [ncbi.nlm.nih.gov]
Management and treatment Management is based on immunotherapy with intravenous immunoglobulin (IVIg) or plasma exchange. [orpha.net]
Yuki N ; Bickerstaff's brainstem encephalitis and Fisher syndrome: their relationship and treatment. Rinsho Shinkeigaku. 2004 Nov44(11):802-4. [patient.info]
Management and Treatment Effective management and treatment of BBE and other variants of Anti-Gq1b syndrome requires prompt recognition and diagnosis. [practicalneurology.com]
Prognosis
Serial electrophysiological studies allowed not only to understand the underlying pathophysiology and formulate a more correct prognosis but also to guide the treatment. [ncbi.nlm.nih.gov]
Prognosis Although the clinical picture is severe, the disease course is generally monophasic with complete remission of symptoms within 6 months in over half of the patients. Other patients may have mild to severe residual findings. [orpha.net]
Further observation on a grave syndrome with benign prognosis. Br. Med. J., 1:1384–1387, 1957. Google Scholar [6] Fisher M. : An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N. Engl. J. [link.springer.com]
Brain-stem encephalitis: further observations on a grave syndrome with benign prognosis. Br Med J. 1957; 1: 1384-1387. Odaka M, Yuki N, et al. [meducator3.net]
Etiology
Etiology BBE has been reported to occur following infection with several agents including cytomegalovirus, Campylobacter jejuni, and Mycoplasma pneumonie. [orpha.net]
However, absence of anti-GQ1b antibodies and normal MRI do not exclude the diagnosis, which remains based on clinical criteria and exclusion of other etiologies. [scholarbank.nus.edu.sg]
Nguyen-Huu 1, F Birklein 1, S Haegele-Link 1, P Stoeter 2, M Dieterich 1, P Urban 1 1 Klinik und Poliklinik für Neurologie, Universitätsklinik Mainz 2 Institut für Neuroradiologie, Universitätsklinik Mainz Four weeks after a severe pneumonia of unknown etiology [thieme-connect.com]
The etiology of this condition was speculated to be similar to that of Guillain-Barré syndrome because of evidence of prodromal upper respiratory infection, areflexia, and CSF albuminocytological dissociation ( Bickerstaff and Cloake 1951 ). [medlink.com]
Recent observations suggest that intrathecally detectable antibodies against the bacterium, which can serve to establish the etiology of encephalitis, may indeed mediate the disease. [journals.plos.org]
Epidemiology
BACKGROUND: Information regarding the epidemiological background of Bickerstaff brainstem encephalitis (BBE) is limited. [ncbi.nlm.nih.gov]
Summary Epidemiology Prevalence is unknown. Clinical description Patients usually present with onset of diplopia and gait disturbance following upper respiratory or gastrointestinal tract infections. [orpha.net]
Epidemiology The incidence of BBE is higher in Japan compared to Western nations. [practicalneurology.com]
There is a clinical continuum between BBE and Fisher's syndrome. [ 1 ] Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia (diplopia) and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. [ 2 ] Epidemiology [patient.info]
( 23606734 ) Kuwabara S....Mori M. 2013 36 Nationwide survey of patients in Japan with Bickerstaff brainstem encephalitis: epidemiological and clinical characteristics. ( 22851608 ) Koga M....Kanda T. 2012 37 How do we identify infectious agents that [malacards.org]
Pathophysiology
Serial electrophysiological studies allowed not only to understand the underlying pathophysiology and formulate a more correct prognosis but also to guide the treatment. [ncbi.nlm.nih.gov]
The pathophysiology of BBE is not proven; it is presumed that an autoimmune response has a key role. Encephalopathy-associated with autoimmune thyroid disease (EAATD) is another encephalopathy presumed to be related to autoimmune responses. [jksem.org]
Common seropositivity between these two conditions highlights their pathophysiological and clinical similarities. [practicalneurology.com]
However, it has some different pathophysiological mechanisms that are responsible of the clinical and laboratory and imaging studies differences. [new.medigraphic.com]
An anti-GQ1b antibody syndrome has been proposed to underlie the common pathophysiology for the three disorders; however, other studies have found a positive anti-GM1 instead of anti-GQ1b antibody. [digitalcommons.wayne.edu]
Prevention
Preventing encephalitis It's not always possible to prevent encephalitis, but some of the infections that cause it can be prevented with vaccinations. [nhs.uk]
Neurological Disorders and Stroke Cerebral spinal fluid (CSF) collection (Medical Encyclopedia) CSF analysis (Medical Encyclopedia) Encephalitis (Medical Encyclopedia) Japanese Encephalitis Vaccine: What You Need to Know (Centers for Disease Control and Prevention [icdlist.com]
Epidemiology, Disease Transmission and Pathogenesis Caused by JE Virus: Its Prevention and Control. American Journal of Infectious Diseases and Microbiology. 2015; 3(1):38-64. doi: 10.12691/ajidm-3-1-6. [sciepub.com]
• Role of chronic immunosuppression in preventing or reducing the risk of relapses – not clear 36. [de.slideshare.net]