Clinical description Patients usually present with onset of diplopia and gait disturbance following upper respiratory or gastrointestinal tract infections. [orpha.net]

In this case, we illustrate the complexity in diagnosing BBE, a rare variant of GBS due to atypical presentation. Download File Additional Metadata Actions (login required) View Item [psasir.upm.edu.my]

Presentation and Clinical Investigation Our present day resources bear no clinical guidelines that describe specific diagnostic criteria for BBE. [practicalneurology.com]

Thirty-six days after initial presentation, the patient presented to acute inpatient rehabilitation with flaccid tetraplegia, urinary retention, dysphagia with percutaneous endoscopic gastrostomy tube in place and on a ventilator with tracheostomy. [nature.com]

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]

• Malaise

  malaise, vomiting Drowsiness + Absent + + – BBE 8 Muscular pain, malaise Drowsiness + Decreased + + BBE Al‐Din et al. (1982) 1 Headache Drowsiness + Brisk + + – BBE 2 Headache Drowsiness + Brisk + – BBE 3 + Absent + – FS 4 Drowsiness + Normal + + – [doi.org]

• Whipple Disease

  Behçet's disease. Lyme disease. Progressive multifocal leukoencephalopathy. Sarcoidosis. Whipple's disease. Listeria rhombencephalitis. Vasculitis due to systemic lupus erythematosus (SLE). Acute disseminated encephalomyelitis. [patient.info]

• 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]

• Sputum

  Patient 3 A 54‐year‐old man suffered coughing with sputum and diarrhoea, which all abated after a few days. Several days after their resolution, he experienced diplopia in the morning (day 1). [doi.org]

• 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]

• 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]

• Positive Romberg Sign

  She had negative tendon reflex, bilateral Babinski signs, hypalgesia, and numbness in all limbs. She had positive Romberg sign and failed the right heel-knee-tibia tests. [ncbi.nlm.nih.gov]

• Absent Deep Tendon Reflexes

  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]

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]

• Brain MRI Abnormal

  The BBE and FS groups had similar features; positive serum anti-GQ1b IgG antibody (68 % versus 83 %), antecedent Campylobacter jejuni infection (23 % versus 21 %), CSF albuminocytological dissociation (46 % versus 76 %), brain MRI abnormality (11 % versus [f1000.com]

• Candida

  In addition to Patient 2 described in the case reports, two patients died, one of sudden cardiopulmonary arrest and the other of haemorrhagic infarction, a complication in the right cerebrum caused by candida meningitis. [doi.org]

• Absent A-Waves

  […] studies (low amplitudes of compound muscle action potentials), F-wave and H-reflex studies (absent F-waves and soleus H-reflexes), and brainstem auditory evoked potentials (prolongation of wave I latency) suggested peripheral abnormalities. [ncbi.nlm.nih.gov]

Yuki N ; Bickerstaff's brainstem encephalitis and Fisher syndrome: their relationship and treatment. Rinsho Shinkeigaku. 2004 Nov44(11):802-4. [patient.info]

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 Effective management and treatment of BBE and other variants of Anti-Gq1b syndrome requires prompt recognition and diagnosis. [practicalneurology.com]

Falsaperla Bickerstaff’s brainstem encephalitis (BBE) in childhood: rapid resolution after intravenous immunoglobulins treatment Eur Rev Med Pharmacol Sci Year: 2014 Vol. 18 - N. 17 Pages: 2496-2499 [europeanreview.org]

Management and treatment Management is based on immunotherapy with intravenous immunoglobulin (IVIg) or plasma exchange. [orpha.net]

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]

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]

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]

Treatment and prognosis Although symptoms are severe, the condition is usually self-limiting with a good prognosis 2. Related Radiopaedia articles Infections of the central nervous system Promoted articles (advertising) [radiopaedia.org]

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]

BACKGROUND: Information regarding the epidemiological background of Bickerstaff brainstem encephalitis (BBE) is limited. [ncbi.nlm.nih.gov]

Epidemiology The incidence of BBE is higher in Japan compared to Western nations. [practicalneurology.com]

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]

Email: [email protected] Abstract In the present review article epidemiology of Japanese encephalitis, disease incidences and its control measures have been described in detail. [sciepub.com]

型脳幹脳炎の疫学調査 Epidemiological studies on pediatric Guillain-Barre syndrome, Fisher syndrome, and Bickerstaff brainstem encephalitis 科学的試験名/Scientific Title 小児ギラン・バレー症候群，フィッシャー症候群，ビッカースタッフ 型脳幹脳炎の疫学調査 Epidemiological studies on pediatric Guillain-Barre syndrome [upload.umin.ac.jp]

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]

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]