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

presentations of cranial neuropathies. [jnnp.bmj.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]

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]

• Weakness

  One patient among Bickerstaff’s original cases had flaccid limb weakness, and BBE cases were divided into ‘BBE without limb weakness’ and ‘BBE with limb weakness’. [doi.org]

  After IV immunoglobulin and corticosteroid therapy, she began to recover consciousness and from the weakness from day 18. [ncbi.nlm.nih.gov]

  Some weeks following an upper tract infection, the children after a short period of recovery, showed acute onset of symmetric weakness of the lower limbs with difficulty in standing by and walking. [europeanreview.org]

• Fever

  The patient initially presented with fever and headache and gradually developed ataxia, disturbance of consciousness, respiratory muscle paralysis, bilateral facial paralysis and quadriplegia accompanied by significant atrophy of limb, temporalis and [ncbi.nlm.nih.gov]

  Our patient was 26-year-old Sudanese lady, had acute falciparum malaria based on positive blood film, clearance of fever in response to artesunate injection. [smj.eg.net]

  Encephalitis can cause flu-like symptoms, such as a fever or severe headache. It can also cause confused thinking, seizures, or problems with senses or movement. Severe cases of encephalitis, while relatively rare, can be life-threatening. [omicsonline.org]

  Fifty‐seven (92%) patients had had antecedent illnesses (66% upper respiratory infectious symptoms only, 9% fever only, 6% fever and headache, 5% diarrhoea only, and 5% both upper respiratory infectious symptoms and diarrhoea). [doi.org]

  Symptoms of severe cases include Severe headache Sudden fever Drowsiness Vomiting Confusion Seizures In babies, additional symptoms may include constant crying, poor feeding, body stiffness, and bulging in the soft spots of the skull. [icdlist.com]

• 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]

• Falling

  Zimbabwe : Bulawayo, Harare, Mutare, Victoria Falls, etc. [maria-online.com]

  Article / Publication Details First-Page Preview Abstract Dies ist der Fall eines 79-jährigen Mannes mit chronischer lymphatischer Leukämie, der sich etwa 6 Monate nach einer Therapie mit Bendamustin und Rituximab mit einem Guillain-Barré-Syndrom vorstellte [karger.com]

• Vomiting

  Drowsiness + Gross flaccid + + – BBE with overlapping GBS 5 Headache, malaise Drowsiness + – Absent Equivocal + – BBE 6 Malaise, fever, vomiting Drowsiness – + – Brainstem encephalitis 7 Headache, malaise, vomiting Drowsiness + Absent + + – BBE 8 Muscular [doi.org]

  On exam, he had right arm and leg ataxia, symmetrically hyperactive reflexes, broad-based ataxic gait, and nausea with vomiting. [neurology.org]

  Symptoms of severe cases include Severe headache Sudden fever Drowsiness Vomiting Confusion Seizures In babies, additional symptoms may include constant crying, poor feeding, body stiffness, and bulging in the soft spots of the skull. [icdlist.com]

• Dysphagia

  Reports of dysphagia in this population are limited and general. The purpose of this article is to heighten awareness of the potential for silent aspiration in patients with BBE. [ncbi.nlm.nih.gov]

  We report a rare case of childhood BBE with elevated anti-GM1 antibodies in the serum, who had specific clinical symptoms such as a cranial polyneuropathy presenting as ophthalmoplegia, dysarthria, dysphagia, and facial weakness; progressive motor weakness [kjp.or.kr]

  Her severe oropharyngeal dysphagia slowly resolved and she progressed to tolerate a general texture diet. [nature.com]

  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 and recovery from dysphagia [malacards.org]

  The patient quickly developed dysphagia and hyperactive reflexes, as well as cardiac arrhythmias and signs of acute kidney injury. [jamanetwork.com]

• Nausea

  On exam, he had right arm and leg ataxia, symmetrically hyperactive reflexes, broad-based ataxic gait, and nausea with vomiting. [neurology.org]

  CASE An obese 15 year-old female presented with a 5-days history of frontal headache, nausea, vomiting and diplopia. Two weeks earlier she had had flu-like symptoms and diarrhea. [scielo.br]

  A 46-year-old previously healthy woman presented to the Emergency Department with 6 days of nausea, diarrhea, fever to 39.4 °C, altered mental status, ataxia and progressive generalized weakness. [nature.com]

  A prodrome is present in half the patients, consisting of fever, headache, nausea and vomiting, which are followed by psychiatric and behavioral abnormalities a few days later in 95% of patients. [news-medical.net]

  There was no evidence of dysarthria, tremor, nausea or vomiting. Investigations Routine blood tests were all within normal ranges including C reactive protein (CRP) of 2.3 mg/L, white cell count (WCC) 6.3×10 9 /L and neutrophil count 3.8×10 9 /L. [ncbi.nlm.nih.gov]

• Diplopia

  A 76-year-old woman was initially presented with diplopia, ophthalmoplegia, and ataxia, but she later developed weakness of limbs, respiratory failure, deterioration of consciousness, and cognitive impairment. [ncbi.nlm.nih.gov]

  The patient was witnessed to have multiple episodes of paroxysmal worsening of slurred speech and ataxia with diplopia. [jamanetwork.com]

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

  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, a 71 year-old woman developed progressive diplopia [thieme-connect.com]

  Abstract A 39-year-old man acutely developed diplopia, vertigo, unsteady gait, and disturbance of consciousness following an upper respiratory infection. [jstage.jst.go.jp]

• Ataxia

  We describe the case of an 11-year-old boy with encephalopathy, external ophthalmoplegia, brainstem signs, and ataxia with raised titers of anti-GQ1b antibodies. [ncbi.nlm.nih.gov]

  His symptoms progressed to bilateral lower extremity ataxia precluding ambulation and right-sided internuclear ophthalmoplegia(INO). The patient was treated with a five-day course of IVIG with immediate improvement of ataxia and the INO. [neurology.org]

  Instead, these patients have either ophthalmoplegia or ataxia. [jnnp.bmj.com]

  Deep sensation was frequently unimpaired despite profound ataxia. [doi.org]

• Guillain-Barré Syndrome

  That finding is further evidence of continuity between Bickerstaff brainstem encephalitis and Fisher syndrome, a variant of Guillain-Barré syndrome. [ncbi.nlm.nih.gov]

  Keywords: Bickerstaff's brain-stem encephalitis Guillain-Barré syndrome Miller Fisher syndrome Issue Date: 2006 Citation: Sharma, V., Chan, Y.C., Ong, Teoh, H.L., Wilder-Smith, E.P. (2006). Bickerstaff's brainstem encephalitis: Can it recur?. [scholarbank.nus.edu.sg]

  It is a variant of Guillain-Barré syndrome. Unlike Guillain-Barré syndrome, it does not cause limb or respiratory muscle weakness. [cochrane.org]

  Anti-GM1b and anti-GalNAc-GD1a antibodies have been detected in some patients who developed Guillain-Barré syndrome after C. jejuni enteritis, whereas the anti-GQ1b IgG antibody is associated with BBE. [unboundmedicine.com]

• Areflexia

  He also suggested that MFS and BBE may belong to the same group of disorders as syndrome of ophthalmoplegia, ataxia and areflexia (SOAA). [ncbi.nlm.nih.gov]

  O'Connor P.S., Rothner A.D. : Acute ophthalmoplegia, ataxia and areflexia (Fisher syndrome) in childhood. Cleveland Clin. Quart, 45:247–252, 1978. [link.springer.com]

  An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 1956;255:57-65. [ Links ] 10. Bickerstaff ER. [scielo.br]

• Limb Weakness

  One patient among Bickerstaff’s original cases had flaccid limb weakness, and BBE cases were divided into ‘BBE without limb weakness’ and ‘BBE with limb weakness’. [doi.org]

  Discussion Our patient was initially diagnosed with MFS given the classic triad of ophthalmoplegia, areflexia, and ataxia but he later developed limb weakness consistent with GBS. [hindawi.com]

  Overlapping forms between BBE and GBS (BBE/GBS) are described in patients with lower limbs weakness and typical signs of BBE, suggesting a combined involvement of Central and Peripheral Nervous System (PNS), but only few reported cases are focused on [ncbi.nlm.nih.gov]

• Hyperreflexia

  The presence of drowsiness, Babinski's sign or hyperreflexia favors Bickerstaff's brainstem encephalitis over Miller Fisher Syndrome. References: Bickerstaff ER, Cloake PCP. Mesencephalitis and rhomboencephalitis. Br Med J. 1951; 2: 77-91. [meducator3.net]

  Subsequent to Campylobacter jejuni enteritis, the patient rapidly developed consciousness disturbance and hyperreflexia in addition to external ophthalmoplegia and cerebellar-like ataxia. [unboundmedicine.com]

  Seizure Young Rok Do Department of Neurology, Catholic University of Daegu School of Medicine, Daegu, Korea Abstract Bickerstaff's brainstem encephalitis (BBE), characterized by acute ophthalmoplegia and ataxia, often causes impaired consciousness and hyperreflexia [jkna.org]

  Some patients with BBE show hyperreflexia, pyramidal signs and sensory disturbance. The pathophysiology of BBE is not proven; it is presumed that an autoimmune response has a key role. [jksem.org]

  In the cases with Bickerstaff brainstem encephalitis, there was associated altered consciousness and in some, hyperreflexia, in support of a central pathology whereas in Fisher syndrome, patients were areflexic in keeping with a peripheral aetiology. [ncbi.nlm.nih.gov]

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]

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]

CONCLUSION: Our study identified clinical, radiologic, and treatment features that may hold prognostic value for children with BBE. [ncbi.nlm.nih.gov]

Randomised controlled trials are needed to establish the value of immunotherapies or other treatments. [cochrane.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]

Treatment and prognosis Although symptoms are severe, the condition is usually self-limiting with a good prognosis 2. [radiopaedia.org]

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]

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]

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]

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]

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]