Bickerstaff's Encephalitis Research Articles

60.: Pharyngeal-cervical-brachial variant of Guillain–Barré syndrome with monospecific anti-GT1a antibodies

Guillain–Barre syndrome (GBS) is well recognised as an acute, immune-mediated polyneuropathy. It encompasses a heterogenous group of disorders with a number of subtypes and variants, some of which are now associated with specific antiganglioside antibodies. The overlap between the clinical features and immunopathology of GBS and its variants, which include acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy(AMAN), pharyngeal-cervical-brachial weakness, Fisher syndrome and Bickerstaff brainstem encephalitis support the theory that these conditions form a spectrum and share a similar pathogenesis. We report a classical case of pharyngeal-cervical-brachial weakness with monospecific anti-GT1a antibodies. A 65-year-old woman presented with a 1 week history of progressive swallowing difficulty and asymmetrical upper limb weakness which was most severe proximally. During the course of the week, she developed lower limb weakness, although this was less severe. There was no preceding gastrointestinal illness, but she reported an exacerbation of respiratory symptoms 1 month prior with increased sputum production. On arrival to our centre, she had weak tongue and palatal movements. Facial power and eye movements were normal. Neck flexion was 3/5. Shoulder abduction was 3/5 on the right and 0/5 on the left. Distal upper limb power was 3/5 on the left and 4/5 on the right. Power in the lower limbs was 4/5 to 4-/5. There was no fatigability or ataxia. Upper limb reflexes were reduced. Lower limb reflexes were normal. The sensory examination was normal. Neurophysiology demonstrated compound muscle action potential dispersion and dispersed F waves with reduced persistence. Sensory responses were normal. Serum immunoglobulin G antibodies to GT1a, GM1, GD1a, GQ1b and GD1b were measured by enzyme-linked immunosorbent assay. Serum was considered positive for antiganglioside antibodies when the absorbance value was ⩾0.5 at a dilution of 1:500 because this high cut-off level gives high specificity. The sample was positive for anti-GT1a antibodies only. This patient presents with clinical features consistent with pharyngeal-cervical-brachial variant of GBS. The presence of anti-GT1a antibodies without antibodies against GQ1b is increasingly being recognised as a subgroup of GBS characterised by oropharyngeal, neck and predominantly upper limb weakness, which is highlighted in the above patient.

Guillain-Barré and Miller Fisher syndromes--new diagnostic classification.

Guillain-Barré syndrome (GBS) and its variant, Miller Fisher syndrome (MFS), exist as several clinical subtypes with different neurological features and presentations. Although the typical clinical features of GBS and MFS are well recognized, current classification systems do not comprehensively describe the full spectrum of either syndrome. In this Perspectives article, GBS and MFS are classified on the basis of current understanding of the common pathophysiological profiles of each disease phenotype. GBS is subclassified into classic and localized forms (for example, pharyngeal-cervical-brachial weakness and bifacial weakness with paraesthesias), and MFS is divided into incomplete (for example, acute ophthalmoparesis, acute ataxic neuropathy) and CNS subtypes (Bickerstaff brainstem encephalitis). Diagnostic criteria based on clinical characteristics are suggested for each condition. We believe this approach to be more inclusive than existing systems, and argue that it could facilitate early clinical diagnosis and initiation of appropriate immunotherapy.

P631: Clinical features of recurrent Fisher syndrome and Bickerstaff brainstem encephalitis

P631: Clinical features of recurrent Fisher syndrome and Bickerstaff brainstem encephalitis

Chronic Bickerstaff's encephalitis with cognitive impairment, a reality?

BackgroundBickerstaff’s encephalitis (BE) is an acute post-infectious demyelinating disease with albuminocytological dissociation. A chronic form has rarely been described previously.Case presentationA 44-year-old man was hospitalized for drowsiness, cognitive complaint limb weakness, ataxia and sensory disturbance after diarrhea. Neuropsychological evaluation showed slowing, memory and executive function impairment, while analysis of the CSF showed albuminocytological dissociation. Immunologic tests showed positive anti-ganglioside antibodies (anti-GM1 IgM, anti-GD1a IgG and anti-GD1b IgM). Brain MRI was normal but SPECT showed bilateral temporal and frontal hypoperfusion. Outcome under immunoglobulin treatment (IVIG) was favorable with an initial improvement but was marked by worsening after a few weeks. Consequently, the patient was treated with IVIG every 2 months due to the recurrence of symptoms after 6 weeks.ConclusionThis case raises the question of the existence of a chronic form of BE with cognitive impairment, in the same way as chronic inflammatory demyelinating polyneuropathy is considered to be a chronic form of Guillain–Barré syndrome.

A pediatric case of Bickerstaff's brainstem encephalitis.

Bickerstaff's brainstem encephalitis is characterized by ophthalmoplegia, ataxia, and disturbance of consciousness. It is similar to Miller Fisher syndrome, a variant of Guillain-Barre syndrome, in that they share features such as ophthalmoplegia and ataxia. The difference is that patients with Bickerstaff's brainstem encephalitis have impaired consciousness, whereas patients with Miller Fisher syndrome have alert consciousness and areflexia. Here, we report the case of a 3-year-old child who was diagnosed with Bickerstaff's brainstem encephalitis presenting typical clinical features and interesting radiological findings. The patient showed ophthalmoplegia, ataxia, and subsequent stuporous mentality. Brain magnetic resonance imaging revealed high signal intensity in the pons and cerebellum around the 4th ventricle on a T2-weighted image. He was successfully treated with intravenous immunoglobulin. Differentiation of Bickerstaff's brainstem encephalitis and Miller Fisher syndrome is often difficult because they possess many overlapping features. Brain magnetic resonance imaging may be helpful in diagnosing Bickerstaff's brainstem encephalitis, especially when lesions are definitely found.

Overlapping Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus.

A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barré syndrome (GBS). In addition, BBE and Fisher syndrome, which are clinically similar and are both associated with the presence of the immunoglobulin G anti-GQ1b antibody, represent a specific autoimmune disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia. A 2-year-old boy presented with rapidly progressive ophthalmoplegia, ataxia, hyporeflexia, weakness of the lower extremities, and, subsequently, disturbance of consciousness. He experienced bronchitis with watery diarrhea and had laboratory evidence of recent infection with Epstein-Barr virus (EBV). He was diagnosed as having overlapping GBS and BBE associated with EBV and received treatment with a combination of immunoglobulin and methylprednisolone, as well as acyclovir, and had recovered completely after 3 months. In addition, he has not experienced any relapse over the past year. We suggest that combinations of symptoms and signs of central lesions (disturbance of consciousness) and peripheral lesions (ophthalmoplegia, facial weakness, limb weakness, and areflexia) are supportive of a diagnosis of overlapping GBS and BBE and can be helpful in achieving an early diagnosis, as well as for the administration of appropriate treatments.

Fisher-Bickerstaff syndrome with negative anti-ganglioside antibody test results associated with mycoplasma pneumoniae infection

Miller-Fisher syndrome and Bickerstaff brainstem encephalitis are two conditions that have probably a common autoimmune etiology. Anti-ganglioside antibodies are present in most of the patients with these clinical conditions. The symptoms of these disorders variably involve peripheral nervous system or central nervous system. There is an unclassified group of patients who have symptoms of both Miller-Fisher syndrome and Bickerstaff brainstem encephalitis and a new eponymic terminology “Fisher-Bickerstaff syndrome” is suggested for these patients. Mycoplasma Pneumonia as a cause of Fisher-Bickerstaff syndrome has not been reported before. Clinical and radiologic features of a nine-year-old boy with “Fisher-Bickerstaff syndrome” associated with Mycoplasma Pneumonia infection are presented. A nine-year-old boy presented with ptosis, diplopia, drowsiness, areflexia. He had a history of recent upper respiratory tract infection and laboratory evidence of Mycoplasma Pneumonia infection. Brain magnetic resonance imaging revealed hyperintense lesions in the brainstem and electromyography revealed absent H reflex and reduced F wave responses in median nerves. Anti-ganglioside antibodies were negative. The patient dramatically responded to intravenous immunoglobuline treatment. Mycoplasma Pneumonia may cause Fisher-Bickerstaff syndrome without a rise in anti-ganglioside antibodies. Intravenous immunoglubuline treatment seems a good therapeutic option for these cases.

Atypical Bickerstaff's brainstem encephalitis: Clinical vigilance ensures favourable prognosis

Atypical Bickerstaff's brainstem encephalitis: Clinical vigilance ensures favourable prognosis

Isolated acute bilateral ophthalmoplegia as a form of anti-GQ1b syndrome - a case report and differential diagnostic considerations

WCN 2013 No: 1101 Topic: 7 — Neuromuscular disorders Isolated acute bilateral ophthalmoplegia as a form of anti-GQ1b syndrome a case report and differential diagnostic considerations A. Toth, G. Aradi, I. Vastagh, K. Pozsegovits, L. Rencz, D. Bereczki, Z. Aranyi. Department of Neurology, Semmelweis University, Faculty of Medicine, Budapest, Hungary; Department of Neurology, Dr. Kenessey Albert Hospital, Balassagyarmat, Hungary Background: Isolated acute bilateral ophthalmoplegia is an uncommon occurrence, but it may cause differential diagnostic problems. It may be a manifestation of the Miller Fisher syndrome spectrum, which has been recently referred to as anti-GQ1b syndrome. Objective: To present a case with acute bilateral ophthalmoplegia associated with anti-GQ1b antibody positivity, but without other typical signs of Miller Fisher syndrome (MFS). Differential diagnostic aspects are highlighted. Patient: A 57-year-old female patient presented with an acute onset of diplopia, progressing to bilateral ophthalmoplegia within days. These symptoms were preceded by flu-like symptoms. Deep tendon reflexes were diminished, otherwise her neurological status was unremarkable, the typical triad of MFSwas not observed. MRI of the brain was normal. Cerebrospinal fluid examination revealed increased protein content with normal cell count. Electrophysiological assessment showed normal sensory nerve action potentials, whereas low amplitude or absent sensory nerve action potentials are characteristic findings in MFS. Serological testing confirmed anti-GQ1b class IgG antibody positivity, supporting the diagnosis of MFS spectrum. The patient fully recovered within 3 months, without any specific treatment, showing the benign nature of the condition. Discussion: Acute ophthalmoparesis may be a restricted form antiGQ1b syndrome. In addition to classical MFS, further variants include Bickerstaff's brainstem encephalitis, pharyngeal-cervical-brachial paresis, and acute ataxic sensory neuropathy. Although acute ophthalmoparesis as a manifestation of anti-GQ1b syndrome is a benign condition, it may cause differential diagnostic problems with potentially more serious conditions needing prompt treatment, such as Wernicke's encephalopathy, ocularmyasthenia, botulism, paraneoplastic brainstemencephalitis, or brainstem stroke. doi:10.1016/j.jns.2013.07.1569 Abstract — WCN 2013 No: 1413 Topic: 7 — Neuromuscular disorders “Reporting biomarker” development: Update in als patients treated with G-CSF -mobilized hematopoietic stem cells WCN 2013 No: 1413 Topic: 7 — Neuromuscular disorders “Reporting biomarker” development: Update in als patients treated with G-CSF -mobilized hematopoietic stem cells A.V. Khomenko, D. Baldaranov, J. Grassinger, S.W. Johannesen, I. Kobor, J. Roesl, K. Kollewe, S. Petri, R. Dengler, M. Deppe, A. Ludolph, J. Kassubek, G. Schuierer, T. Bruun, W. Schulte-Mattler, U. Bogdahn. Department of Neurology, University of Regensburg, Regensburg, Germany; Department of Haematology and Internal Oncology, University Hospital Regensburg, Regensburg, Germany; Clinic for Neurology, Medizinische Hochschule Hannover, Hannover, Germany; Department of Neurology, University of Muenster, Muenster, Germany; Neurological University Clinic, University Ulm, Ulm, Germany; Department of Neuroradiology, University of Regensburg,

Bickerstaff brainstem encephalitis — /INS;A case report

Background: Bickerstaff brainstem encephalitis (BBE) is a rare, postinfectious, immuno-mediated disorder clinically presenting with acute ophthalmoplegia, gait ataxia and occasionally decreased level of consciousness. Clinical signs result from the inflammatory lesion in the region adjacent to the IVth ventricle and the mesencephalic reticular formation.

Atypical Bickerstaff Brainstem Encephalitis: Pitfalls of Diagnosis

We read with interest the article recently published by Gologorsky et al.,1 which reported two unusual cases of brainstem encephalitis and suggested they may represent atypical variants of Guillain-Barré syndrome (GBS). Although they are correct in recognizing that “incomplete” forms of Fisher syndrome (FS) and Bickerstaff brainstem encephalitis (BBE) exist and may overlap with other forms of GBS, the clinical features and laboratory investigations presented here do not support a diagnosis of GBS variants.

Paralytic mydriasis in Bickerstaff brainstem encephalitis. Report of three cases

Bickerstaff brainstem encephalitis(BBE) is an immune-mediated syndrome of consciousness disturbance, cranial nerve symptoms/signs, cerebellar ataxia and altered deep reflexes, preceded by a febrile illness. Simultaneous involvement of both brainstem and peripheral nerves has been reported. Here we report three BBE patients with paralytic mydriasis whose autonomic dysfunctions were analyzed. Case1: A 42-year-old woman acutely appeared dysarthria, quadriplegia, left-dominant cerebellar ataxia, complete extraocular muscle palsy and hyperreflexia in four limbs, following to fever, headache and diarrhea for several days.

Mycoplasma pneumoniae Intrathecal Antibody Responses in Bickerstaff Brain Stem Encephalitis

The pathogenesis of Mycoplasma pneumoniae encephalitis is not established. We report, for the first time, the case of a patient with severe Bickerstaff brain stem encephalitis in whom we detected intrathecal production of M. pneumoniae-specific antibodies, contrasting the findings in another patient with less severe encephalitis in whom we detected intrathecal M. pneumoniae DNA but no specific antibodies. Our observations suggest that intrathecal M. pneumoniae-specific antibody responses may contribute to a more severe course of M. pneumoniae encephalitis.

Encefalitis de Bickerstaff: informe de caso y revisión de la literatura

We describe the case of a 34-year-old male patient, who was referred to the Instituto Neurológico de Colombia with probable Guillain-Barré syndrome, requiring intensive care management. The presence of cognitive alterations during his evolution, lead the team to reconsider the initial diagnosis for the Bickerstaff's brainstem encephalitis diagnosis. We aim to describe the patient's treatment and evolution, as well as a brief review and discussion.

Complement activation by anti-GQ1b antibodies in Fisher syndrome and its variants

IgG anti-GQ1b antibodies are associated with Fisher syndrome (FS) as well as with its more and less extensive variants, Bickerstaff brainstem encephalitis and acute ophthalmoparesis. Passive transfer experiments provide evidence that anti-ganglioside antibodies and complement are pathogenic in the development of FS. In the current in vitro study, we could not demonstrate the association of complement activation with disease extension.

Atypical Bickerstaff brainstem encephalitis: ataxic hypersomnolence without ophthalmoplegia

ObjectiveClinical and immunological evaluation of ‘incomplete’ Bickerstaff brainstem encephalitis (BBE).MethodsWe studied two patients with postinfectious brainstem syndromes who presented at National University Hospital Singapore. Laboratory work-up included measurement of antiganglioside...

Bickerstaff型脳幹脳炎の全国疫学調査からわかったこと：病態機序の多様性

Bickerstaff brainstem encephalitis (BBE) is characterized by acutely progressive bilateral ophthalmoparesis and ataxia with impaired consciousness or pyramidal signs, or both; all of which are followed by a monophasic course with good recovery. Alike Guillain-Barré syndrome (GBS), BBE is proposed to have an autoimmune mechanism triggered by antecedent infection. The nationwide epidemiologic survey for BBE, which the author had performed in Japan, suggests that BBE consists of typical and atypical cases. Typical BBE has similar neurological and serological features to Fisher syndrome and shows good recovery, whereas atypical BBE is characterized by delayed recovery, negative anti-GQ1b antibodies, and abnormal cerebrospinal fluid and brain MRI findings with other possible pathogeneses.

Cranial neuropathies, confusion, and ataxia— Challenges for diagnosis and therapy

Introduction: The differential diagnosis of patients presenting with multiple cranial neuropathies, ataxia, and altered mentation is broad and includes immunologic, infectious, vasculitic and metabolic conditions. Primary considerations are Bickerstaff’s brainstem encephalitis (BBE), the Miller Fisher syndrome (MFS), Wernicke’s encephalopathy and botulism. The initial workup may be unrevealing. Timely treatment is imperative and unnecessary treatment can be associated with serious adverse reactions. Sensitivity to the decisions needed in such patients is therefore important. Case report: A 58-year-old male presented with symptoms of altered mental status, blurred vision, dysphagia and dysarthria, impaired pupillary responses to light, facial diplegia, ataxia, and decreased tendon reflexes after an episode of a self resolving diarrheal disease. Primary initial diagnostic concerns were Bickerstaff’s brainstem encephalitis (BBE), Miller Fisher syndrome (MFS), Wernicke’s encephalopathy and botulism. Initial work-up including cerebrospinal fluid analyses, imaging studies, and an electrodiagnostic examination did not provide information helpful for narrowing this differential. The patient was treated with botulinum antitoxin, thiamine and intravenous immune globulin (IVIG) before the results of specialized tests were available. The patient’s clinical condition improved. Retrospectively, the patient was diagnosed as BBE. Conclusion: This case emphasizes the difficulties in distinguishing between BBE, MFS, and botulism as well as demonstrating the complexities of treating such patients.

Silent Aspiration and Recovery From Dysphagia in a Case of Bickerstaff Brainstem Encephalitis

Bickerstaff brainstem encephalitis (BBE) is a rare inflammatory demyelinating disease with rapid progression typically followed by complete recovery. 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. This article details the nature of dysphagia during the recovery phase of BBE and includes findings from serial videofluoroscopic swallowing evaluations. In addition, it shows the impact of cognitive impairment on the ability to eat safely. Early instrumental swallowing evaluation, close monitoring, and conservative management are essential to ensure safety in patients recovering from BBE.

Prolonged Comatose State Followed by Rapid Recovery in a Patient with Bickerstaff's Brainstem Encephalitis

Prolonged Comatose State Followed by Rapid Recovery in a Patient with Bickerstaff's Brainstem Encephalitis