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Bickerstaff Brainstem Encephalitis


Presentation

  • 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]
  • Clinical description Patients usually present with onset of diplopia and gait disturbance following upper respiratory or gastrointestinal tract infections.[orpha.net]
  • Inclusion required all of the following criteria: age 20 years, presence of somnolence or alterations in mental status at the time of presentation or developed within 7 days of presentation, ataxia, and ophthalmoplegia.[ncbi.nlm.nih.gov]
  • He presented following a respiratory illness and had laboratory evidence of recent infection with Mycoplasma pneumoniae. M pneumoniae infection has been associated with both Bickerstaff brainstem encephalitis and Miller Fisher syndrome.[ncbi.nlm.nih.gov]
  • 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]
Whipple Disease
  • Whipple's disease. Listeria rhombencephalitis. Vasculitis due to systemic lupus erythematosus (SLE). Acute disseminated encephalomyelitis.[patient.info]
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]
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]
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]
Rhinorrhea
  • Prior to the occurrence of BBE, our patient had a cough and rhinorrhea. These symptoms were quickly followed by wheezing and dyspnea, diagnosed as an episode of bronchial asthma.[bmcresnotes.biomedcentral.com]
Blurred Vision
  • A 48-year-old woman was hospitalized due to blurred vision and unsteady gait lasting for 9 days, and numbness of the limbs lasting for 6 days.[ncbi.nlm.nih.gov]
  • We report the case of an 18-year-old woman presented with cerebellar ataxia, blurred vision, rapidly progressive dementia, tremor and involuntary movements, urinary incontinence, mutism, and eventually myoclonus for 16 weeks.[medworm.com]
  • The symptoms persisted, and on day 3 he developed blurred vision, dizziness, and nausea. On day 12, he became intermittently drowsy, however, he could be aroused by noxious stimulation.[bmcresnotes.biomedcentral.com]
Dysmetria
  • They showed general weakness, severe ataxia, bilateral dysmetria and bilateral lateral gaze limitation. And gaze-evoked nystagmus was observed in one of them.[e-rvs.org]
  • The patient had dysmetria and slowed rapid alternating movements in the upper extremities. Results of the sensory examination were normal. A chest radiograph showed an increase in interstitial markings and small right-sided effusion.[jamanetwork.com]
  • Formal tests of limb coordination showed decomposition and dysmetria. Paraesthesiae and hypoaesthesiae of the glove‐and‐stocking type were present. Vibration and position senses were intact. GBS was diagnosed. By the evening he could not swallow.[doi.org]
Mydriasis
  • Paralytic mydriasis in Bickerstaff brainstem encephalitis. Report of three cases. Autonomic neuroscience: Basic and clinical US 2013;177:53. Koga M, Kusunoki S, Kaida K, Uehara R, et al. Research paper.[new.medigraphic.com]
  • Although ptosis, mydriasis and bulbar palsy are not part of the triad seen in FS, these signs have been reported to occur in both FS and BBE cases. 15 Isolated ptosis, mydriasis or oropharyngeal palsy in association with anti-GQ1b antibodies have been[jnnp.bmj.com]
  • Ptosis, mydriasis, and facial palsy can frequently be seen both MFS and BBE.4 Anti-Gq1b antibodies are seen more frequently in MFS than in BBE.[practicalneurology.com]
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]
Altered Mental Status

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]
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]

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]
  • 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]
  • Bickerstaff brainstem encephalitis is a potentially reversible syndrome that needs early diagnosis (facilitated by magnetic resonance imaging) and prompt aggressive and supportive treatment.[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]

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]
  • Bickerstaff brainstem encephalitis (BBE) is a rare inflammatory, demyelinating disease that generally has a good prognosis. To describe the course of a patient with severe BBE and multiple medical complications. Case report. Academic medical center.[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]
  • 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]
  • 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]
  • 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]
Sex distribution
Age distribution

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]

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