Bickerstaff's Encephalitis Research Articles

Bickerstaff’s brainstem encephalitis in children – A case series from a tertiary care centre

Background: Bickerstaff’s Brainstem encephalitis(BBE) is rare postinfectious immunemediated disorder.Condition is relatively rare in children and there are very few reports from pediatric population.

Uncommon Presentation of Bickerstaff Encephalitis

Uncommon Presentation of Bickerstaff Encephalitis

Posterior reversible encephalopathy syndrome in a patient with Bickerstaff's brainstem encephalitis

AbstractBackgroundBickerstaff's brainstem encephalitis (BBE) is characterized by acute ophthalmoplegia, ataxia and impaired consciousness. Serum anti‐GQ1b antibody in the acute phase has been detected in many BBE cases. Posterior reversible encephalopathy syndrome (PRES) is characterized by acute neurological symptoms with vasogenic edema in the bilateral parieto‐occipital regions. It has been pointed out that the activation of inflammatory cytokines might be involved in the pathophysiology of PRES and BBE.Case presentationA 37‐year‐old woman had an upper respiratory infection a week before losing consciousness. On admission, she was in a coma and her blood pressure was 134/96 mmHg. Doll's eye phenomenon was negative. Bilateral tendon reflexes were normal, although Babinski signs were bilateral positive. Cerebrospinal fluid examination showed normal protein concentration and cell counts. Brain magnetic resonance imaging findings were normal. We administered intravenous methylprednisolone therapy (1000 mg/day), acyclovir (1500 mg/day) and fosphenytoin sodium hydrate (22.5 mg/kg/day). Brain magnetic resonance imaging on the third admission day showed an increased apparent diffusion coefficient map, and high intensities in diffusion‐weighted imaging in the bilateral occipital cortex and subcortex, which are consistent with PRES. Nerve conduction studies were normal. The patient was treated with intravenous immunoglobulin therapy (0.4 g/kg/day) from the fourth admission day. Her consciousness level rapidly improved, and we were able to detect obvious ophthalmoplegia and limb ataxia. Diffusion‐weighted imaging was improved on the 13th day of admission. Serum anti‐GQ1b immunoglobulin G antibody on admission was positive. We ultimately diagnosed her with BBE.ConclusionsWe reported a rare case of BBE complicated with PRES.

Report on two patients admitted same time presenting as atypical forms of Miller Fisher syndrome: Acute isolated bilateral ophthalmoplegia and bickerstaff brainstem encephalitis

Report on two patients admitted same time presenting as atypical forms of Miller Fisher syndrome: Acute isolated bilateral ophthalmoplegia and bickerstaff brainstem encephalitis

Blink Reflex Usefulness in Acute Phase of Bickerstaff's Brainstem Encephalitis

Bickerstaff’s brainstem encephalitis (BBE) is a Fisher Syndrome (FS) related disorder characterized by the disturbance of consciousness or hyperreflexia in addition to the classical triad of ataxia, areflexia and ophthalmoplegia. The pathophysiology of BBE remains controversial therefore a complete neurophysiological study including the study of cranial nerves may be crucial to add evidence of peripheral or central nervous system involvement in the assessment of patients. We report the case of 29 years old woman with BBE and the blink reflex (BR) follow up studies revealing a correlation between clinical and electrophysiological improvement. This case may support the usefulness of BR studies for electrophisiological diagnosis in BBE in early stages.

Bickerstaff brainstem encephalitis, acute transverse myelitis, and acute motor axonal neuropathy: diagnostic and treatment challenges in patients with concomitant syndromes. Clinical observation

We report a 40-year-old woman presented with consciousness disturbance, ataxia, asymmetrical limb weakness, hyperreflexia. Due to magnetic resonance imaging findings, the patient was diagnosed with Bickerstaff’s brainstem encephalitis overlapped with acute transverse myelitis. Later she developed distal muscles atrophy and the electroneuromyographic study revealed axonal motor neuropathy, therefore acute motor axonal neuropathy was diagnosed. The patient underwent one course of intravenous immunoglobulin therapy with the regression of symptoms and magnetic resonance imaging changes. Nine months after symptoms onset, the patient has completely recovered. This overlapping case of Bickerstaff’s brainstem encephalitis, acute transverse myelitis and acute motor axonal neuropathy provides further support that these conditions are part of the same spectrum.

Neurological Complications during Treatment of Middle East Respiratory Syndrome.

Background and PurposeMiddle East respiratory syndrome (MERS) has a high mortality rate and pandemic potential. However, the neurological manifestations of MERS have rarely been reported since it first emerged in 2012.MethodsWe evaluated four patients with laboratory-confirmed MERS coronavirus (CoV) infections who showed neurological complications during MERS treatment. These 4 patients were from a cohort of 23 patients who were treated at a single designated hospital during the 2015 outbreak in the Republic of Korea. The clinical presentations, laboratory findings, and prognoses are described.ResultsFour of the 23 admitted MERS patients reported neurological symptoms during or after MERS-CoV treatment. The potential diagnoses in these four cases included Bickerstaff's encephalitis overlapping with Guillain-Barré syndrome, intensive-care-unit-acquired weakness, or other toxic or infectious neuropathies. Neurological complications did not appear concomitantly with respiratory symptoms, instead being delayed by 2–3 weeks.ConclusionsNeuromuscular complications are not rare during MERS treatment, and they may have previously been underdiagnosed. Understanding the neurological manifestations is important in an infectious disease such as MERS, because these symptoms are rarely evaluated thoroughly during treatment, and they may interfere with the prognosis or require treatment modification.

A severe case of Bickerstaff's Encephalitis: the importance of specialist neuro-respiratory rehabilitation to optimise recovery and outcome

A severe case of Bickerstaff's Encephalitis: the importance of specialist neuro-respiratory rehabilitation to optimise recovery and outcome

Poster 225 Bickerstaff's Encephalitis with an Exemplary Recovery Following Rehabilitation: A Case Report.

Poster 225 Bickerstaff's Encephalitis with an Exemplary Recovery Following Rehabilitation: A Case Report.

Altered gene expression of glycosyltransferases and sialyltransferases and total amount of glycosphingolipids following herpes simplex virus infection

There is a case report of a patient with overlapping Guillain–Barré syndrome and Bickerstaff brainstem encephalitis after infection with herpes simplex virus type 1 (HSV-1), who carried high titers of serum anti-GQ1b IgG antibodies. Several studies have linked viral infection to the modulation of ganglioside expression such as human T-lymphotropic virus to GD2 and simian virus 40 to GM3. Also, enhancement of the expression of GM2 on the cell membrane after cytomegalovirus infection has been reported. The objective of this study was to unveil the relationship between HSV-1 infection and the alteration of cellular ganglioside expression in neuronal and glial cell lines. In addition to these cell lines, several human tumor cell lines including astrocytoma cells, neuroblastoma cells, T-cell leukemia cells and kidney cells derived from normal human and monkey were infected with HSV-1 as well as HSV-2. To measure changes in ganglioside-related gene expressions and gangliosides levels in cells, quantitative PCR and glycosphingolipid-glycomic analysis were performed. Changes in gene expression of glycosyltransferases and sialyltransferases were observed in HSV-1- and HSV-2-infected cells, although with different trends. 39 glycosphingolipid-glycans were quantitatively analyzed. HSV-1 and HSV-2 infections resulted in changes in the total amount of gangliosides depending on the cell lines used and type of virus. Qualitative changes caused by each infection of HSV-1 and HSV-2 were almost negligible.

Intrathecal Anti-GalC Antibodies in Bickerstaff Brain Stem Encephalitis

Intrathecal Anti-GalC Antibodies in Bickerstaff Brain Stem Encephalitis

How often and when Fisher syndrome is overlapped by Guillain-Barré syndrome or Bickerstaff brainstem encephalitis?

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). Our aim was to elucidate the frequency of this overlap and the patterns of clinical progression in patients with FS. Sixty consecutive patients with FS were studied. FS/PCB-GBS was diagnosed when the patients developed pharyngeal, cervical and/or brachial weakness. Patients with flaccid tetraparesis were diagnosed as having FS/conventional GBS. FS/BBE was defined as the development of consciousness disturbances. All 60 patients initially developed the FS clinical triad alone (pure FS). Of these, 30 (50%) patients had pure FS throughout their course, whereas the remaining 50% of patients showed an overlap: PCB-GBS in 14 (23%) patients, conventional GBS in nine (15%) patients and BBE in seven (12%) patients. The median (range) durations from FS onset to progression to FS/PCB-GBS, FS/GBS or FS/BBE were 5 (1-7), 3 (1-4) and 3 (1-5) days, respectively. Patients with overlap syndromes more frequently received immune-modulating treatment, and the outcomes were generally favourable. The frequencies of positivity for anti-GQ1b, GT1a, GD1a, GD1b, GalNAc-GD1a and GM1 antibodies were not significantly different amongst the four groups. Of the patients with pure FS, 50% later developed an overlap with PCB-GBS, conventional GBS or BBE. The overlap occurred within 7 days of FS onset; thus, physicians should pay attention to the possible development of this overlap during the first week after FS onset.

Recurrent Guillain–Barré syndrome, Miller Fisher syndrome and Bickerstaff brainstem encephalitis

ObjectiveGuillain–Barré syndrome (GBS), Miller Fisher syndrome (MFS), and Bickerstaff brainstem encephalitis (BBE) are usually monophasic, but some patients experience recurrences after long asymptomatic intervals. We aimed to investigate clinical features of recurrent GBS, MFS, and BBE at a single hospital. MethodsRecords from 97 consecutive patients with GBS, MFS or BBE who were admitted to a tertiary hospital between 2001 and 2013 were reviewed. Clinical and laboratory features of patients with recurrent GBS, MFS, or BBE were investigated. ResultsPatients included 55 (32 males) with GBS, 34 (22 males) with MFS, and 8 (6 males) with BBE. Recurrent cases occurred in 2 (4%) of the 55 patients with GBS, 4 (12%) of the 34 patients with MFS, and 2 (25%) of the 8 patients with BBE. Patients with recurrent MFS had a tendency to be younger at the first episode than patients with non-recurrent MFS (median, 22 versus 37years old). Symptoms and signs were less severe during relapses than during the initial episode in recurrent patients. ConclusionsRecurrences occurred more frequently in patients with MFS or BBE compared with those with GBS. Patients with recurrent MFS might be younger than those with non-recurrent MFS.

Rehabilitation of a patient with overlap of acute transverse myelitis and Bickerstaff's brainstem encephalitis: a case report.

We report on one patient with Bickerstaff's brainstem encephalitis (BBE) and associated flaccid weakness. Counter to previous studies with BBE which indicate weakness due to Guillain-Barre syndrome, our patient's presentation of paraplegia following BBE is consistent with concomitant acute transverse myelitis. Her findings of BBE largely resolved, although she remained with T6 American Spinal Injury Association (ASIA) A paraplegia. Motor functional impairment measure scores improved from 20 at admission to 66 before discharge home with assistance. This case presents the first potential overlapping case of acute transverse myelitis with BBE and describes how acute inpatient rehabilitation can be effective in facilitating transition back to independence following tetraplegia with BBE.

An Overlapping Case of Miller Fisher Syndrome, Bickerstaff’s Encephalitis, and the ASMAN Variant of Guillain-Barre Syndrome

A 56-year-old man presented with a 3-day history of progressive tingling of the hands, unsteadiness, and diplopia. He was initially diagnosed clinically with Miller Fisher Syndrome (MFS) but later developed limb weakness consistent with Guillain-Barre Syndrome (GBS) and subsequently reduced consciousness consistent with Bickerstaff's brainstem encephalitis (BBE). Neurophysiology revealed an axonal motor and sensory neuropathy, in keeping with the Acute Motor and Sensory Axonal Neuropathy (AMSAN) variant of GBS. We believe that our patient had an MFS-AMSAN-BBE overlap syndrome. This is supported by his glycolipid antibody profile with high titres of anti-GQ1b IgG antibody and anti-GD1a IgG antibody. Anti-GQ1b antibodies are frequently found in both MFS and BBE and the anti-GD1a antibody is associated with axonal forms of GBS. Overlapping cases of MFS and BBE are well described, and because the same antibody is often found in both conditions, it is thought that they share a common autoimmune mechanism. BBE has also been previously reported in association with GBS lending support that it also lies on the same spectrum. This overlapping case of ASMAN variant of GBS, MFS, and BBE provides further support that these conditions are part of the same spectrum.

Recurrent Diplopia in a Pediatric Patient with Bickerstaff Brainstem Encephalitis.

Introduction. 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. Ophthalmoplegia has been reported with acute ataxia in Miller Fisher syndrome (MFS) and Bickerstaff brainstem encephalitis (BBE). Up to 95% of these cases are associated with anti-GQ1b antibodies. Only a small number of cases of anti-GQ1b negative MFS have been documented in pediatric patients. This is the first case reporting a recurrence of ocular symptoms in an anti-GQ1b antibody negative patient with BBE. Case Presentation. An 8-year-old Caucasian boy presented with complete external ophthalmoplegia without ptosis, cerebellar ataxia, and a disturbance of consciousness. He had recently recovered from a confirmed Campylobacter jejuni infection. On subsequent laboratory testing he was anti-GQ1b antibody negative. He had a recurrence of diplopia at four-week follow-up. Conclusions. This patient's recurrence of diplopia was treated with a five-week course of oral corticosteroids which did not worsen his condition, and this may be a therapeutic option for similar patients. We will discuss the symptoms and treatment of reported pediatric cases of anti-GQ1b antibody negative cases of MFS and the variation between cases representing a spectrum of illness.

Fisher Syndrome and Bickerstaff Brainstem Encephalitis

Fisher syndrome has been regarded as a peculiar inflammatory neuropathy with ophthalmoplegia, ataxia, and areflexia, whereas Bickerstaff brainstem encephalitis has been considered a pure central nervous system disease characterized by ophthalmoplegia, ataxia, and consciousness disturbance. Both disorders share common features including preceding infection, albumin-cytological dissociation, and association with Guillain-Barré syndrome. The discovery of anti-GQ1b IgG antibodies further supports the view that the two disorders represent a single disease spectrum. The lesions in Fisher syndrome and Bickerstaff brainstem encephalitis are presumably determined by the expression of ganglioside GQ1b in the human peripheral and central nervous systems. Bickerstaff brainstem encephalitis is likely to represent a variant of Fisher syndrome with central nervous system involvement.

Poster 84 Bickerstaff Encephalitis Associated with Cortical Features: A Case Report

Poster 84 Bickerstaff Encephalitis Associated with Cortical Features: A Case Report

Therapeutic plasma exchange in children with acute autoimmune central nervous system disorders.

There is a growing evidence for autoimmunity in acute central nervous system (CNS) disorders and treatment with therapeutic plasma exchange (TPE) may be considered. The aim was to share our experience on the clinical application of TPE in these disorders and to present a reproducible protocol which can be used even in small children. We present a series of 8 children aged 2-12 years with transverse myelitis, Bickerstaff's brainstem encephalitis, neuromyelitis optica, and acute paraneoplastic or unspecified encephalitis in whom TPE was used as a second-line or rescue treatment. A total of 104 TPE sessions were performed where 80-110 ml/kg of plasma was exchanged using 4% albumin solution and fresh frozen plasma. Six episodes of TPE-related adverse events were documented. Fibrinogen concentrations decreased after the first TPE, whereas platelets decreased gradually. One patient died in the course of the acute illness. Three children achieved a complete resolution of symptoms, 2 children have mild sequelae; whereas 2 children remain paraplegic after a follow-up of 3 to 17 months. We report 8 children with presumably autoimmune-mediated, acute CNS disorders treated with TPE as a rescue therapy. Although the effect of TPE can only be inferred, 5 children had a good clinical outcome. TPE is feasible even in small children with acute autoimmune CNS disorders.

Subtypes and Prognosis of Guillain-Barré Syndrome in Southwest China.

The proportion of different subtypes of Guillain-Barré syndrome (GBS) and their prognosis varied significantly among different regions. This study attempts to investigate the clinical subtypes and outcome of GBS in southwest China. Patients with GBS admitted to The First Affiliated Hospital of Chongqing Medical University from January 2006 to March 2013 were included in our study. Patients were classified into acute inflammatory demyelinating polyneuropathy (AIDP) group, acute motor axonal neuropathy (AMAN) group, Miller-Fisher syndrome (MFS) group, cranial nerve variants(CNV), Bickerstaff's brainstem encephalitis overlaps with GBS (BBE-GBS) group and unclassifiable group based on clinical features and electrophysiological findings. Hughes function grade score (HFGS) was used to assess the prognosis at 3 and 6 months. The prognosis of different subtypes and outcome predictors were analyzed. The most common subtype of GBS was AIDP (57%), followed by AMAN (22%) and MFS (7%). The prognosis of AMAN and BBE-GBS is similar at 3 month(P = 0.0704)and 6 month (P = 0.1614) follow-up. The prognosis of AMAN group was poorer than that of AIDP group at 3 month and 6 month follow-up (P<0.001). Outcome of MFS group and that of CNV group at 6 months were both good(Hughes≤1). Hughes≥3(P<0.0001,OR = 6.650,95%CI = 2.865 to 15.023))and dysautonomia (P = 0.043,OR = 2.820,95%CI = 1.031 to 7.715)) were associated with poor outcome at 6 month follow-up. AIDP is the most common subtype of GBS. Prognosis of AMAN group and BBE-GBS group is poorer than that of AIDP group at 3 month and 6 month follow-up. Hughes≥3 at nadir and dysautonomia are predictors of poor prognosis at 6 month follow-up.