gq1b-ganglioside and Encephalitis

To characterize the phenotype of pediatric Bickerstaff's brainstem encephalitis (BBE) and evaluate prognostic features in the clinical course, diagnostic studies, and treatment exposures.. We systematically reviewed PubMed, Web of Science, and SCOPUS databases as well as medical records at the Lucile Packard Children's Hospital to identify cases of pediatric BBE. 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.. We reviewed 682 manuscripts, identifying a total of 47 pediatric BBE cases. We also describe five previously unreported cases. The phenotype of these pediatric patients was similar to previously published literature. Sixty-eight percent of patients demonstrated positive anti-GQ1b antibody titers, yet the presence of these antibodies was not associated with longer times to recovery. Patients with neuroimaging abnormalities featured a longer median time to recovery, but this was not statistically significant (p = 0.124). Overall, patients treated with any form of immunotherapy (intravenous immunoglobulin, steroids, or plasmapheresis) demonstrated shorter median time to resolution of symptoms compared to supportive therapy, although this trend was not statistically significant (p = 0.277). Post-hoc t tests revealed a trend towards use of immunotherapy against supportive care alone (p = 0.174).. Our study identified clinical, radiologic, and treatment features that may hold prognostic value for children with BBE. The role of immunotherapy remains under investigation but may prove of utility with further, randomized controlled studies in this rare disease.

Topics: Adolescent; Autoantibodies; Brain Stem; Child; Child, Preschool; Databases, Bibliographic; Encephalitis; Female; Gangliosides; Humans; Male

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.

Topics: Animals; Autoantibodies; Brain Stem; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome

Fisher syndrome has been regarded peculiar inflammatory neuropathy with ophthalmoplegia, ataxia, and areflexia, whereas Bickerstaff brainstem encephalitis has been considered pure central nervous system disease characterized with ophthalmoplegia, ataxia, and consciousness disturbance. Both disorder share common features including preceding infection, albumin-cytological dissociation, and association with Guillain-Barre syndrome. The discovery of anti-GQ1b IgG antibodies further supports the view that the two disorder represent a single disease spectrum. Currently Bickerstaff brainstem encephalitis can be regarded as a variant of Fisher syndrome with central nervous system involvement.

Topics: Autoantibodies; Biomarkers; Brain Stem; Encephalitis; Gangliosides; Humans; Miller Fisher Syndrome; Peripheral Nervous System

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.

Topics: Age Factors; Autoantibodies; Autoimmunity; Biomarkers; Brain Stem; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Japan; Male; Miller Fisher Syndrome; Sex Factors

In the 1950s, Bickerstaff and Fisher independently described cases with a unique presentation of ophthalmoplegia and ataxia. The neurological features were typically preceded by an antecedent infection and the majority of patients made a spontaneous recovery. 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. However, both authors recognised certain similarities to Guillain-Barré syndrome such as the presence of peripheral neuropathy and cerebrospinal fluid albuminocytological dissociation. The discovery of immunoglobulin G anti-GQ1b antibodies in patients with Fisher syndrome and later in Bickerstaff brainstem encephalitis was crucial in providing the necessary evidence to conclude that both conditions were in fact part of the same spectrum of disease by virtue of their common clinical and immunological profiles. Following this, other neurological presentations that share anti-GQ1b antibodies emerged in the literature. These include acute ophthalmoparesis and acute ataxic neuropathy, which represent the less extensive spectrum of the disease whereas pharyngeal-cervical-brachial weakness and Fisher syndrome overlap with Guillain-Barré syndrome represent the more extensive end of the spectrum. The conditions can be referred to as the 'anti-GQ1b antibody syndrome'. In this review, we look back at the historical descriptions and describe how our understanding of Fisher syndrome and Bickerstaff brainstem encephalitis has evolved from their initial descriptions more than half a century ago.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Antibodies, Anti-Idiotypic; Axons; Child; Child, Preschool; Demyelinating Diseases; Encephalitis; Female; Gangliosides; History, 20th Century; Humans; Infant; Infant, Newborn; Male; Middle Aged; Miller Fisher Syndrome; Molecular Mimicry; Young Adult

Miller Fisher syndrome is a localized variant of Guillain-Barré syndrome, characterized by ophthalmoplegia, areflexia and ataxia. Bickerstaff's brainstem encephalitis is a related syndrome in which upper motor neurone features accompany the classic triad. Anti-GQ1b antibodies are uniquely found in both conditions and are believed to be pathogenic.. Infectious illnesses usually precede Miller Fisher syndrome. The clearest associations have been described with Haemophilus influenzae and Campylobacter jejuni infection. Raised cerebrospinal fluid protein is seen in 60% of patients, but clinical features and anti-GQ1b antibody testing are diagnostically more informative. Experimental studies demonstrating complement-dependent neuromuscular block may be relevant to the clinical pathophysiology of Miller Fisher syndrome. Recent neurophysiological studies suggest abnormal neuromuscular transmission occurs in some cases of Miller Fisher syndrome and Guillain-Barré syndrome. Recent mouse models have demonstrated that presynaptic neuronal membranes and perisynaptic Schwann cells are targets for anti-GQ1b antibody attack. The elimination of antiganglioside antibodies from the circulation through specific immunoadsorption therapy has the potential to ameliorate the course of Miller Fisher syndrome. This condition is typically a benign, self-limiting illness. Both plasmapheresis and intravenous immunoglobulin may be employed as treatment, especially in cases of Bickerstaff's brainstem encephalitis or those with overlapping Guillain-Barré syndrome.. Anti-GQ1b antibody testing has allowed clinicians to develop a greater understanding of the spectrum of Miller Fisher syndromes and to refine clinical diagnoses in patients with unusual presentations. Experimental studies strongly suggest anti-GQ1b antibodies are pathogenic, which in principle should direct treatments towards antibody neutralization or elimination.

Topics: Animals; Bacterial Infections; Brain Stem; Disease Progression; Encephalitis; Gangliosides; Humans; Miller Fisher Syndrome

Bickerstaff reported eight patients who, in addition to acute ophthalmoplegia and ataxia, showed drowsiness, extensor plantar responses or hemisensory loss. This condition has been named Bickerstaff's brainstem encephalitis (BBE). One patient had gross flaccid weakness in the four limbs. Presumably because of the rarity of this disorder, there has been no reported study on a large number of patients with BBE. To clarify its clinical features, we reviewed detailed clinical profiles and laboratory findings for 62 cases of BBE diagnosed by the strict criteria of progressive, relatively symmetrical external ophthalmoplegia and ataxia by 4 weeks, and disturbance of consciousness or hyperreflexia. Ninety-two per cent of the patients involved had had an antecedent illness. Besides ophthalmoplegia and ataxia, disturbance of consciousness was frequent (74%), and facial diplegia (45%), Babinski's sign (40%) and pupillary abnormality and bulbar palsy (34%) were present. Almost all the patients had a monophasic remitting course and generally a good outcome. Serum anti-GQ1b IgG antibody was positive in 66%, and MRI showed brain abnormality in 30% of the patients. Another striking feature was the association with flaccid symmetrical tetraparesis, seen in 60% of the patients. An autopsy study of a BBE patient clearly showed the presence of definite inflammatory changes in the brainstem: there was perivascular lymphocytic infiltration with oedema and glial nodules. Electrodiagnostic study results suggested peripheral motor axonal degeneration. Limb weakness in the BBE cases studied was considered the result of overlap with the axonal subtype of Guillain-Barré syndrome. These findings confirm that BBE constitutes a clinical entity and provide additional clinical and laboratory features of BBE. A considerable number of BBE patients have associated axonal Guillain-Barré syndrome, indicative that the two disorders are closely related and form a continuous spectrum.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Ataxia; Autoantibodies; Brain Stem; Chi-Square Distribution; Child; Child, Preschool; Electroencephalography; Electromyography; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Magnetic Resonance Imaging; Male; Middle Aged; Motor Neurons; Ophthalmoplegia; Quadriplegia

We report an 11-year-old boy with Bickerstaff's brainstem encephalitis (BBE). He had gait disturbance, disturbed consciousness, and diplopia after upper respiratory tract infection. On admission, he showed multiple cranial nerve palsy, muscle weakness of arms, cerebeller ataxia and generalized areflexia. The cerebrospinal fluid on day 7 revealed albuminocytologic dissociation. IgG antibodies against GQ1b and GT1a were detected in the serum. Immunoglobulin was administered intravenously from day 11, and then his symptoms gradually diminished. When he was discharged on day 27, he had neither conscious disturbance nor limb weakness. There still were mild ophthalmoparesis and diminished deep tendon reflexes, but they disappeared by 10 months after the onset. Effective therapy for BBE has yet to be established. Our case had features of Guillain-Barré syndrome (GBS) and Miller Fisher syndrome, such as an acute monophasic course, limb weakness with areflexia, albuminocytologic dissociation in the cerebrospinal fluid, detection of serum anti-ganglioside antibodies and efficacy of intravenous immunoglobulin, indicating that BBE and GBS are closely related. Our case suggested that intravenous immunoglobulin therapy, an established treatment for GBS, should be considered in some patients with BBE.

Topics: Autoantibodies; Brain Stem; Child; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Male; Miller Fisher Syndrome

Bickerstaff's brainstem encephalitis (BBE) and Fisher syndrome (FS) are immune-mediated diseases associated with anti-ganglioside antibodies, specifically the anti-GQ1b IgG antibody. These two diseases potentially lie on a continuous spectrum with Guillain-Barré Syndrome (GBS). There are some reports of family cases of GBS and fewer of FS. However, there are no reports of family cases of BBE and FS.. We report a familial case of an 18-year-old son who had BBE and his 52-year-old mother diagnosed with FS within 10 days. The son showed impaired consciousness 1 week after presenting with upper respiratory symptoms and was brought to our hospital by his mother. He showed decreased tendon reflexes, limb ataxia, albuminocytologic dissociation in his spinal fluid, and positive serum anti-GQ1b antibodies. Haemophilus influenzae was cultured from his sputum. He was diagnosed with BBE and treated with intravenous immunoglobulin (IVIg) therapy, which led to an improvement in symptoms. The mother presented with upper respiratory symptoms 3 days after her son was hospitalized. Seven days later, she was admitted to the hospital with diplopia due to limited abduction of the left eye. She showed mild ataxia and decreased tendon reflexes. Her blood was positive for anti-GQ1b antibodies. She was diagnosed with FS and treated with IVIg, which also led to symptomatic improvement.. There are no previous reports of familial cases of BBE and FS; therefore, this valuable case may contribute to the elucidation of the relationship between genetic predisposition and the pathogenesis of BBE and FS.

Topics: Adolescent; Brain Stem; Encephalitis; Female; Gangliosides; Genetic Predisposition to Disease; Humans; Immunoglobulins, Intravenous; Male; Middle Aged; Miller Fisher Syndrome; Mothers; Nuclear Family

Miller-Fisher syndrome (MFS) together with Guillan-Barré syndrome (GBS) and Bickerstaff brainstem encephalitis (BBE) are considered to form a continuous clinical spectrum of the same disease, possibly affecting the peripheral and/or central nervous systems, with monophasic symptoms. The frequency of overlapping clinical signs and the risk of recurrence are independent and very low, but no cases of GQ1b-seropositive recurrent MFS overlapping with GBS and BBE have been described so far. Here, we describe for the first time an atypical case of recurrent GQ1b-seropositive MFS overlapping GBS and BBE, 12 years after a previous GQ1b-seronegative typical MFS episode. Our case expands the clinical spectrum of recurrent MFS, and it should prompt clinicians to investigate the presence of anti-ganglioside antibodies in recurrent MFS even when these were negative in the previous episode, especially in those presenting with overlapping spectrum symptoms and a critically ill picture during the second episode.

Topics: Adult; Autoantibodies; Autoantigens; Brain Stem; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Recurrence

Miller-Fisher syndrome and Bickerstaff brainstem encephalitis, among others, constitute the anti-GQ1b syndrome, with a common immune pathophysiologic pathway characterized by the presence of anti-GQ1b antibodies, which react against the different nervous system GQ1b sites according to their different accessibility. The Miller-Fisher syndrome has a prevalence of 0.09 cases per 100 000 people-year but there are not epidemiological studies about Bickerstaff brainstem encephalitis, that it seems to be less frequent. In spite of having a good natural outcome, the immunoglobulin administration has been established as efficacious at improving it. A twelveyear- old boy suffering from Miller-Fisher-Bickerstaff syndrome after an acute Campylobacter jejuni diarrhea with positive titers of anti-GQ1b and anti-QGT1a antibodies is presented. We communicate a very uncommon pediatric disease with the aim of warning about the importance of its early suspicion and the need of specific laboratory determinations.. El síndrome anti-GQ1b reúne el síndrome de Miller-Fisher y la encefalitis del tronco cerebral de Bickerstaff, entre otras entidades. Tienen etiopatogenia común, constituida por la presencia de anticuerpos anti-GQ1b que reaccionan contra los sitios GQ1b del sistema nervioso según sea su accesibilidad. La prevalencia anual del síndrome de Miller-Fisher es de 0,09 casos por 100 000 habitantes por año y no existen estudios epidemiológicos sobre la encefalitis del tronco cerebral de Bickerstaff, que sería menos frecuente. De evolución natural hacia la mejoría, se beneficia del tratamiento con gammaglobulina endovenosa. Se presenta a un paciente de 12 años con síndrome de Miller- Fisher–Bickerstaff tras un episodio de diarrea aguda por Campylobacter jejuni en el que los anticuerpos anti-GQ1b resultaron positivos. Es nuestro objetivo comunicar sobre un síndrome de presentación poco habitual en pediatría a fin de advertir acerca de la necesidad de su sospecha precoz y solicitud de estudios de laboratorio específicos.

Topics: Autoantibodies; Autoantigens; Biomarkers; Child; Encephalitis; Gangliosides; Humans; Male; Miller Fisher Syndrome; Syndrome

To clarify the differences in clinical characteristics between anti-GQ1b antibody-positive and antibody-negative Bickerstaff brainstem encephalitis (BBE).. We compared 73 anti-GQ1b antibody-positive BBE cases with 10 antibody-negative cases. Their clinical information and sera were collected from various hospitals throughout Japan between 2014 and 2017. The anti-GQ1b antibody was examined in each serum sample by ELISA.. We identified the distinctive findings of anti-GQ1b antibody-positive BBE compared with the antibody-negative cases: (1) upper respiratory infection and sensory disturbance were more common, (2) the cell count or protein concentration was lower in the CSF, (3) the abnormal findings on brain MRI were less, and (4) the consciousness disturbance disappeared earlier. Furthermore, IV immunoglobulin (IVIG) was more frequently administered to the anti-GQ1b antibody-positive cases of BBE compared with the antibody-negative cases.. BBE with anti-GQ1b antibody has homogeneous features. IVIG is the treatment used prevalently for BBE with anti-GQ1b antibody in Japan.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Autoantibodies; Autoimmune Diseases of the Nervous System; Brain Stem; Encephalitis; Female; Gangliosides; Humans; Immunoglobulins, Intravenous; Immunologic Factors; Japan; Male; Middle Aged; Young Adult

Bickerstaff brainstem encephalitis is a rare, immune-mediated disorder of the brainstem and peripheral nervous system. Published knowledge of pediatric Bickerstaff brainstem encephalitis focuses on the acute phase of the disease process. This study evaluated long-term neurologic and immune sequelae of Bickerstaff brainstem encephalitis in children. A single-center retrospective chart review was performed. Clinical data, neuroimaging, polysomnograms, and serum data were reviewed. Five patients were included in this study. Four patients had no neurologic residua, and 1 patient continued to have mild bulbar dysfunction. There was neither recurrence of symptoms nor development of other neurologic or immunologic disorders at a median of 3 years after diagnosis. Review of systems was largely negative, although 2 patients endorsed symptoms consistent with mild orthostatic hypotension for 1 year after diagnosis, but these findings were not sustained. Four of 5 patients endorsed sleep dysregulation. Three patients met criteria for pediatric obstructive sleep apnea. Prognosis following pediatric Bickerstaff brainstem encephalitis is excellent although posttreatment autonomic and sleep dysregulation may reflect residua from acute phase inflammation in the peripheral nervous system and connections of the reticular activating formation of the brainstem, although this was time limited. Further prospective, multicenter, analysis is warranted.

Topics: Adolescent; Antibodies; Autonomic Nervous System Diseases; Child; Child, Preschool; Encephalitis; Female; Gangliosides; Humans; Male; Polysomnography; Retrospective Studies; Sleep Wake Disorders; Time Factors

Periodic alternating ping-pong gaze (PPG) is a rare disease with few reports. To our knowledge, there was no report on anti GQ1b antibody syndrome accompanied by PPG. This paper reported a case of anti GQ1b antibody syndrome with Bickerstaff's Encephalitis (BBE) overlapping classic Guillain-Barre Syndrome (GBS) after aortic valve replacement, accompanied by an excessive PPG in the course of diagnosis and treatment, this was indeed rarely.. A 55-year-old male patient was admitted to our hospital with intermittent chest tightness for 3 months, and his condition has worsened in the past 10 days. Aortic valve replacement was performed because of the existence of the moderate and severe stenosis of aortic valve. Horizontal movement of the eyeball was involuntarily slow. The eyeball hovered and returned from one side to the other horizontally for 3-4 s per cycle. In combination with the patient's typical clinical and laboratory tests, the final diagnosis was anti GQ1b antibody syndrome BBE combined with GBS, accompanied by saccadic ping pong gaze. Intravenous immunoglobulin (0.4 g/kg) was given for immunomodulation, methylprednisolone (1000 mg) therapy and symptomatic treatment were performed in the patient.. The patients were discharged from hospital on the thirtieth day because of economic reasons. After 6 months of follow up, the patients left behind a lack of fluency in speech and limb mobility, but the basic life can be taken care of by himself.

Topics: Aortic Valve Stenosis; Autoantibodies; Cardiovascular Surgical Procedures; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Heart Valve Prosthesis; Humans; Immunoglobulins, Intravenous; Male; Middle Aged; Ocular Motility Disorders

A 16-year-old healthy male experienced gastrointestinal symptoms and 9 days later developed fever, headache, numbness of the left hand, and disturbance of consciousness with rapid deterioration to a comatose state. These clinical symptoms resolved after treatment with steroid pulse, plasma exchange, and intravenous immunoglobulin. Along with the recovery, ophthalmoplegia and ataxia were observed. These symptoms and the detection of a high titer of serum anti-GQ1b immunoglobulin G autoantibodies led to the diagnosis of Bickerstaff's brainstem encephalitis (BBE). Brain

Topics: Adolescent; Autoantibodies; Biomarkers; Brain Stem; Cerebellum; Encephalitis; Gangliosides; Humans; Immunoglobulin G; Male; Tomography, Emission-Computed, Single-Photon

Topics: Adult; Ataxia; Autoantibodies; Autoantigens; Autoimmune Diseases; Brain Stem; Cognitive Dysfunction; Diplopia; Encephalitis; Female; Gangliosides; Humans

A 33-year-old woman was admitted due to disturbance of consciousness, dysarthria, dysphagia, sensory disturbances and weakness of the left upper limb after mycoplasma infection. She was treated with intravenous immunoglobulin and intravenous high-dose methylprednisolone as Bickerstaff brainstem encephalitis (BBE). On the 15th hospital day, reflex myoclonus appeared on her face, neck, body and limbs induced by techniques of jaw jerk reflex and patellar tendon reflex. The myoclonus was disappeared after two weeks in accordance with improvement of BBE. The transient reflex myoclonus may be originated from brainstem lesion which was affected by BBE. Reflex myoclonus is thought to be rare symptom in patient with BBE.

Topics: Adult; Autoantibodies; Brain Stem; Encephalitis; Female; Gangliosides; Humans; Immunoglobulins, Intravenous; Methylprednisolone; Mycoplasma Infections; Myoclonus; Myography; Pulse Therapy, Drug; Time Factors

Topics: Adult; Animals; Autoantibodies; Autonomic Nervous System Diseases; Brain Stem; Encephalitis; Gangliosides; Humans; Magnetic Resonance Imaging; Male; Tilt-Table Test; Valsalva Maneuver

We report the first case of Listeria monocytogenes meningoencephalitis associated with anti-GQ1b antibody syndrome in an immunocompetent adult. A prompt diagnosis, made thanks to the multidisciplinary contribution, allowed a combined therapeutic approach leading to final favourable outcome, despite several intercurrent complications.

Topics: Autoantibodies; Autoimmune Diseases; Encephalitis; Gangliosides; Humans; Male; Meningitis, Listeria; Middle Aged; Miller Fisher Syndrome

Central nervous system (CNS) autoantibodies have been reported in a range of neuroimmune diseases, but there has not been a systematic evaluation of autoantibodies in paediatric patients with brainstem encephalitis.. Serum samples from 57 children (40 male, 17 female, median age 12y, range 0.6-18y) with a diagnosis of brainstem encephalitis were tested retrospectively for antibodies to GQ1b, aquaporin-4 (AQP4), myelin oligodendrocyte glycoprotein (MOG), N-methyl-D-aspartate receptor, LGI1, CASPR2, glycine receptor (GlyR), DPPX, and the voltage gated potassium channel (VGKC)-complex.. Disease localized to the brainstem was seen in 19 patients: Bickerstaff's brainstem encephalitis (n=14) and clinically isolated syndrome (n=5). Polyfocal presentation was seen in 38 children, with predominantly white matter disease in 18 patients and grey matter in 20 patients. CNS surface antibodies were found in 22/57 patients (two patients with double positivity): GQIb (n=6), NMDAR (n=7), GlyR (n=5), MOG (n=5), and one AQP4. Three patients were positive for VGKC-complex antibodies. All patients were negative for antibodies to DPPX and the VGKC-complex antigens LGI1, CASPR2, and contactin-2. Although there were some partial differences in the presentations, the clinical features and outcomes did not relate clearly to the presence or absence of specific antibodies.. As determined retrospectively, 39% of patients had cell surface antibodies. The results did not suggest any relationship with treatment or outcomes obtained but it is possible that specific antibody detection could be a helpful guide to more intensive immunotherapies in some cases.

Topics: Adolescent; Aquaporin 4; Autoantibodies; Brain Stem; Child; Child, Preschool; Encephalitis; Female; Gangliosides; Humans; Infant; Male; Myelin-Oligodendrocyte Glycoprotein; Neuroglia; Potassium Channels, Voltage-Gated; Receptors, Glycine; Receptors, N-Methyl-D-Aspartate; Retrospective Studies

Bickerstaff brainstem encephalitis (BBE) is a rare neurological disease that generally has a good prognosis. We describe an atypical case of a patient with severe BBE; the presentation was uncommon because of the lack of ophthalmoplegia and because of evidence of both peripheral neuropathy and brainstem encephalitis. The article reports clinical and biochemical evaluation and focuses on magnetic resonance imaging (MRI) findings in diagnosis and management of the patient. Notably, we found a previously unreported dramatic spinal cord involvement on MRI. We believe these findings could add to diagnostic tools, and that this case may represent a new variant of BBE with more aggressive behavior.

Topics: Aged; Antigens, CD1; Ataxia; Autoantibodies; Brain Stem; Encephalitis; Female; Gangliosides; Humans; Image Processing, Computer-Assisted; Magnetic Resonance Imaging

Clinical and immunological evaluation of 'incomplete' Bickerstaff brainstem encephalitis (BBE).. We studied two patients with postinfectious brainstem syndromes who presented at National University Hospital Singapore. Laboratory work-up included measurement of antiganglioside antibodies.. Both patients displayed hypersomnolence and cerebellar-like ataxia in the absence of external ophthalmoplegia and carried high serum titres of IgG anti-GQ1b antibodies, strongly indicative of BBE.. Ophthalmoplegia can be absent or incomplete in BBE, and the absence of this clinical feature should not exclude BBE from the clinicians' differential. Such cases of incomplete BBE could be defined as 'ataxic hypersomnolence without ophthalmoplegia'.

Topics: Adult; Autoantibodies; Brain Stem; Cerebellar Ataxia; Disorders of Excessive Somnolence; Encephalitis; Female; Follow-Up Studies; G(M1) Ganglioside; Gangliosides; Humans; Immunization, Passive; Middle Aged; Neurologic Examination; Ophthalmoplegia

Edwin Bickerstaff and Philip Cloake reported in the 1950's three cases of reversible encephalitis. The concept of antibody associated encephalitis had not been proposed at the time they astutely recognized the importance of disease pattern recognition and postulated a potential immune based mechanism. The syndrome defined by Bickerstaff of progressive, external ophthalmoplegia and ataxia, with disturbance of consciousness or hyperreflexia, has subsequently been associated with anti-GQ1b antibodies. Interestingly one of the three original cases, a young woman who developed seizures, an eye movement disorder and acute psychosis while awaiting ovarian cystectomy, has features that may be more consistent with anti-NMDA receptor encephalitis.

Topics: Anti-N-Methyl-D-Aspartate Receptor Encephalitis; Antibodies, Anti-Idiotypic; Encephalitis; Female; Gangliosides; History, 20th Century; Humans; Young Adult

We report a case of a 23-year-old man diagnosed with overlapping Bickerstaff brainstem encephalitis (BBE) and Guillain-Barre syndrome (GBS). 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 masseter muscles. Brain MRI revealed abnormality in the left basal ganglia, thalamus, and rightside posterior limb of the internal capsule. Electromyogram indicated neurogenic damage (mainly axonal damage) in the upper and lower limbs and bilateral facial nerve damage. Cerebrospinal fluid (CSF) collected via lumbar puncture was colorless and transparent with a pressure of 330 mm H2O. The white blood cell count in CSF was 200×106/L, the protein concentration was 1.25 g/L, and Pandy's reaction was positive. Both the blood and CSF were negative for GQ1b antibody. The patient was clinically diagnosed with overlapping BBE and GBS. After treatment with ventilator assisted breathing, hormone therapy, neurotrophic and anti-infection therapies, and symptomatic and supportive care for more than three months, spontaneous breathing was restored. By the 5-month follow-up examination, the patient had completely recovered and returned to work. Like GBS and Fisher syndrome, BBE might be an anti-GQlb IgG antibody syndrome. Although the serum GQlb IgG antibody-positive rate for BBE is only 66%, a normal brainstem MRI or GQlb lgG antibody-negative finding cannot completely rule out BBE. Therefore, identifying critical illness polyneuropathy for patients with respiratory muscle paralysis and tracheal extubation difficulties at early stages is clinically important.

Topics: Autoantibodies; Brain Stem; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Humans; Magnetic Resonance Imaging; Male; Tomography, X-Ray Computed; Young Adult

Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bicker staff`s brain stem encephalitis (BBE) and Acute Ophtamoplegia (AO). We report four patients aged 16 to 76 years, with anti-GQ1b syndrome. All presented with MFS, one of them evolved to GBS pharyngeal-cervical-brachial variant and other to GBS with BBE. All had a previous history of diarrhea or upper respiratory tract infection. All had positive anti-GQ1b serum antibodies. Both brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Electrophysiology studies were compatible with a demyelinating disease. Two patients needed airway protection with an orotracheal tube and developed dysautonomia. All four patients were treated with immunomodulation. On the sixth month follow-up, patients had only minimal alterations in the neurological examination.

Topics: Adolescent; Adult; Aged; Antibodies, Anti-Idiotypic; Brain Stem; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Magnetic Resonance Imaging; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia

IgG anti-GQ1b antibodies are associated with Fisher syndrome (FS), Bickerstaff brainstem encephalitis (BBE), acute ophthalmoparesis and overlap of FS or BBE with Guillain--Barré syndrome (GBS) (FS/GBS or BBE/GBS). It has not been clearly established if the primary pathology of these disorders is demyelinating or axonal in nature. Rapid resolution of conduction slowing or block without signs of demyelination--remyelination has been reported in axonal subtypes of GBS that are associated with IgG anti-GM1 or -GD1a antibodies. We hypothesised that such reversible conduction failure would be also observed in FS and related disorders.. Serial nerve conduction studies were prospectively performed in 15 patients with FS and related conditions.. Neither conduction block nor abnormal temporal dispersion was observed in any of the nerves at any point in all the patients. Conduction velocities for none of the nerves were in the demyelinating range. The amplitude of sensory nerve action potential was decreased in three FS, one FS/GBS and two BBE/GBS patients. Compound muscle action potential amplitudes were decreased in the two BBE/GBS patients. These decreases in amplitudes of sensory nerve action potential and compound muscle action potential promptly resolved without significant change in duration on serial studies.. Reversible conduction failure was seen in six of the 15 patients with FS and related disorders on serial nerve conduction studies. There were no signs of demyelination or remyelination in the 15 patients. The pathology appears to be primarily non-demyelinating. We believe these conditions form a continuous spectrum with axonal GBS.

Topics: Action Potentials; Adult; Autoantibodies; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Middle Aged; Miller Fisher Syndrome; Neural Conduction; Ophthalmoplegia

Information regarding the epidemiological background of Bickerstaff brainstem encephalitis (BBE) is limited.. We conducted a nationwide survey of BBE in the Japanese population in two steps: the first aimed to identify patients with brainstem encephalitis for the specified 3 year period and the second to evaluate whether the clinical picture met our diagnostic criteria for BBE.. The number of patients with brainstem encephalitis was estimated as 704 (95% CI 478 to 930) over the 3 years. The annual onset of BBE was roughly estimated as 100 cases, which accounted for 43% of brainstem encephalitis. BBE was slightly male predominant and often young onset. Among brainstem encephalitis patients, BBE was characterised by antecedent infectious symptoms, oropharyngeal palsy and sensory disturbance at the distal extremities with absent or decreased tendon reflexes, in addition to a triad of symptoms (external ophthalmoplegia, ataxia and impaired level of consciousness) and shorter duration to the peak, with good outcome. Anti-GQ1b antibodies were present in 75% of cases. Several BBE patients with atypical clinical features or without anti-GQ1b antibodies were also identified. These cases often had marked CSF pleocytosis, abnormal brain MRI findings and a longer duration to peak symptoms, sometimes with considerable residual deficits.. BBE is a rare disorder but accounts for a major proportion of brainstem encephalitis. 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 characterised by delayed recovery, negative anti-GQ1b antibodies, and abnormal CSF and brain MRI findings with other possible pathogeneses.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Brain Stem; Child; Child, Preschool; Electroencephalography; Encephalitis; Female; Gangliosides; Health Surveys; Humans; Japan; Magnetic Resonance Imaging; Male; Middle Aged; Nervous System Diseases; Physicians, Primary Care; Surveys and Questionnaires; Treatment Outcome; Young Adult

Bickerstaff's encephalitis is a syndrome of ophthalmoplegia, ataxia and impaired consciousness commonly associated with serum GQ1b antibodies. We describe a patient with seropositive Bickerstaff's encephalitis who did not respond either to plasma exchange or intravenous immunoglobulin but recovered following adjunct treatment with the anti-CD 20 monoclonal antibody, Rituximab. There was a concomitant reduction in serum GQ1b antibodies associated with improvement. Interestingly, GQ1b antibody was also detected in CSF. These findings have potentially significant clinical and immunopathological implications.

Topics: Adult; Antibodies, Monoclonal, Murine-Derived; Autoantibodies; Encephalitis; Gangliosides; Humans; Immunoglobulin G; Immunologic Factors; Male; Rituximab; Severity of Illness Index; Treatment Outcome

A 25-years-old man experienced fever and diarrhea. Ten days later he noticed difficulty walking (day 1). On admission neurological examination revealed lethargy, dysarthria and weakness of limbs. Oculocephalic response was not be elicited and extensor toe signs were positive. In spite of treatment with aciclovir and methylprednisolone, he continued to show progressive deterioration developing to coma with decorticate posture. Autonomic symptoms (hyperhidrosis, hypersalivation and fever) and groaning were observed. Brain magnetic resonance image and brainstem evoked potential presented no abnormality, but electroencephalographic study showed a spindle pattern indicating spindle coma. Laboratory tests including cerebrospinal fluids showed no specific results. High-dose immunoglobulin was administered from day 6, and his consciousness level improved. External ophthalomoplegia and ataxic gait were observed after he became more alert. Because he had IgG type anti-GQ1b antibodies in the serum, a diagnosis was made of Bickerstaff's brainstem encephalitis (BBE). Six months after discharge he had complete resolution of his symptoms. This is the first report of spindle coma observed in a case of serologically confirmed BBE.

Topics: Adult; Autoantibodies; Biomarkers; Brain Stem; Coma; Decerebrate State; Drug Therapy, Combination; Electroencephalography; Encephalitis; Gangliosides; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Male; Methylprednisolone; Treatment Outcome

A 21-year-old healthy female suffered from an upper respiratory tract infection and 2 days later developed diplopia, unsteady gait, dysarthria and a profound disturbance of consciousness with rapid development of coma. Brain MRI and Tc99m brain perfusion SPECT, EEG, neurophysiological tests and CSF analysis results were unspecific. The detection of serum anti-GQ1b IgG autoantibodies at high titre led to the diagnosis of Bickerstaff's brainstem encephalitis (BBE). Clinical symptoms resolved after treatment with plasma exchange and the outcome was good. Brain MRI was normal, and Tc99m brain perfusion SPECT demonstrated hypoperfusion of the whole cerebral hemispheres and basal ganglia with relative sparing of the thalami and the brainstem. Similar to brain MRI, the sensitivity of Tc99m brain perfusion SPECT in detecting brainstem lesions in typical BBE patients seems to be low.

Topics: Brain Stem; Encephalitis; Female; Gangliosides; Humans; Magnetic Resonance Imaging; Organotechnetium Compounds; Plasma Exchange; Tomography, Emission-Computed, Single-Photon; Young Adult

A 39-year-old man acutely developed diplopia, vertigo, unsteady gait, and disturbance of consciousness following an upper respiratory infection. Neurological examination showed ophthalmoplegia, facial paralysis, tetraplegia and loss of deep tendon reflexes. Babinski reflex was positive on the left and there were bilateral flexor withdrawal reflexes. He also developed ballism-like involuntary movements in all extremities, loss of proprioception predominantly on the left, and severe truncal ataxia. Anti-GQ1b IgG antibody was selectively elevated in serum, and CSF protein was elevated to 53 mg/dl with cell count of 12/mm3. Nerve conduction study showed decreased amplitude of compound motor action potentials in all extremities, and no response in facial muscles. Cranial MRI showed no abnormalities whereas EEG was severely abnormal with lack of posterior dominant rhythm and the presence of continuous diffuse theta-waves. This case presented clinical characteristics of three syndromes concurrently-Fisher syndrome, Bickerstaff brainstem encephalitis, and Guillain-Barré syndrome-that may be collectively called 'anti-GQ1b IgG antibody syndrome'. The unique feature of the present case was development of deep coma and ballism-like movements, associated with selective increase of serum anti-GQ1b IgG antibody. It is thus conceivable that anti-GQ1b IgG antibody might underlie the pathogenesis of all three conditions.

Topics: Adult; Brain Stem; Dyskinesias; Encephalitis; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulins, Intravenous; Male; Miller Fisher Syndrome

Whether Bickerstaff's brainstem encephalitis (BBE) is a distinct disease or a subtype of Fisher syndrome (FS) is unclear as there have been no clinical studies with sufficiently large numbers of patients with FS or BBE. Our aim was to clarify the nosological relationship. Medical records of patients suffering acute ophthalmoplegia and ataxia within four weeks of onset were reviewed. BBE was the diagnosis for patients with impaired consciousness, FS for those with clear consciousness and areflexia. Clinical features, neuroimages, and laboratory findings were analyzed. Patients were grouped as having BBE (n = 53), FS (n = 466), or as unclassified (n = 62). 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 2 %), and abnormal EEG findings (57 % versus 25 %). BBE (n = 4) and FS (n = 28) subgroups underwent detailed electrophysiological testing. Both groups frequently showed absent soleus H-reflexes, but normal sensory nerve conduction (75 % versus 74 %) and a 1-Hz power spectrum peak on postural body sway analysis (67 % versus 72 %). Common autoantibodies, antecedent infections, and MRI and neurophysiological results found in this large study offer conclusive evidence that Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum with variable CNS and PNS involvement.

Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Autoantibodies; Brain Stem; Campylobacter Infections; Causality; Child; Child, Preschool; Electroencephalography; Encephalitis; Female; Gangliosides; Humans; Infant; Infant, Newborn; Japan; Magnetic Resonance Imaging; Male; Middle Aged; Miller Fisher Syndrome; Peripheral Nerves; Reflex, Abnormal; Sex Distribution; Terminology as Topic

In this report, we describe the case of two patients with Bickerstaff's brainstem encephalitis (BBE) who developed delirium manifested as emotional incontinence, restlessness, and aggressive behavior from disease onset. Serum anti-GQ1b and anti-GT1a IgG antibodies were detected in both patients. When unusual psychiatric symptoms are observed, in addition to acute ophthalmoplegia and ataxia, neurologists should take into account the possibility of BBE. Brain MRI findings were normal in both patients and SPECT was performed on only patient 1. SPECT of patient 1 showed reversible hypoperfusion in the brainstem, bilateral thalami, and medial frontal lobe. Brain SPECT appears to be useful for detecting lesions of the brainstem as well as the basal ganglia or cerebrum in BBE.

Topics: Adult; Brain Stem; Delirium; Encephalitis; Female; Gangliosides; Humans; Immunoglobulin G; Male; Middle Aged; Ophthalmoplegia; Tomography, Emission-Computed, Single-Photon; Ultrasonography

Twenty-eight patients suffered Campylobacter jejuni enteritis after eating raw chicken. Among them, only one patient developed Bickerstaff's brainstem encephalitis, who carried anti-GQ1b IgG antibodies. In contrast, none of the others did the autoantibodies. C. jejuni was cultured from all stool samples from five patients with enteritis alone. All the isolates had the same genotype, cst-II (Asn51), which are characteristic of strains isolated from Bickerstaff's brainstem encephalitis. These findings suggest that host susceptibility may play a role in inducing the production of anti-ganglioside antibodies and the development of Bickerstaff's brainstem encephalitis.

Topics: Adolescent; Adult; Antibodies; Brain Stem; Campylobacter Infections; Campylobacter jejuni; Disease Outbreaks; Encephalitis; Female; Gangliosides; Humans; Male; Serotyping

Bickerstaff brainstem encephalitis is characterized by the occurrence of ataxia, ophthalmoplegia, motor weakness with areflexia and central nervous system symptoms, with drowsiness, pyramidal syndrome and sensorial symptoms. Diagnosis is based on MR findings and GQ1b antibodies. Treatment is not well known.. We report a patient aged 39 years native of Laos who presented weakness, loss of reflexes, and drowsiness. Brain MR showed hyperintense signals in the brain stem. GQ1b antibodies were positive. The course was characterized by decrease of the weakness, normalization of MR and negativity of GQ1b antibodies.. This observation underlines common features of Bickerstaff brainstem encephalitis, Miller Fisher syndrome and Guillain Barre syndrome. A favorable course and GQ1b antibodies are shared by these syndromes.

Topics: Adrenal Cortex Hormones; Autoantibodies; Autoantigens; Blindness; Brain Stem; Coma; Combined Modality Therapy; Demyelinating Autoimmune Diseases, CNS; Electroencephalography; Encephalitis; Female; Gangliosides; Humans; Magnetic Resonance Imaging; Middle Aged; Optic Atrophy; Plasmapheresis; Quadriplegia; Reflex, Abnormal; Syndrome

Topics: Adult; Autoantibodies; Brain Stem; Cerebellar Ataxia; Disorders of Excessive Somnolence; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Intracellular Signaling Peptides and Proteins; Mental Status Schedule; Neurologic Examination; Neuropeptides; Ophthalmoplegia; Orexins

The Miller Fisher syndrome, Guillain-Barre syndrome and Bickerstaff's brainstem encephalitis are related conditions in which anti-GQ1b antibody positivity occur in varied frequencies. This report demonstrates the presence of corticobulbar and corticospinal dysfunction in BBE, by means of a novel transcranial magnetic stimulation technique. It further supports the presence of protean manifestations in anti-GQ1b IgG antibody-positive spectrum of disorders.

Topics: Adult; Autoantibodies; Brain Stem; Dysarthria; Encephalitis; Evoked Potentials, Motor; Gangliosides; Guillain-Barre Syndrome; Humans; Hypoglossal Nerve; Hypoglossal Nerve Diseases; Male; Motor Neurons; Neural Conduction; Neurologic Examination; Pyramidal Tracts; Reaction Time; Reflex, Abnormal; Remission, Spontaneous; Syndrome; Tongue; Transcranial Magnetic Stimulation

Bickerstaff's brain-stem encephalitis is usually a monophasic post-viral inflammatory illness characterized by progressive ophthalmoplegia, ataxia and disturbance of consciousness (or hyper-reflexia). Since the clinical spectrum of Bickerstaff encephalitis may overlap with the Miller-Fisher and Guillain-Barré syndromes, the presence of anti- GQ1b antibodies and abnormal brain MRI can help to support its diagnosis. 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. We report a case of recurrent Bickerstaff's brainstem encephalitis with no identifiable antecedent illness, and overlapping features of Miller Fisher and Guillain-Barré syndromes, in the presence of negative anti-GQ1b antibodies and repeatedly normal MRI of the brain.

Topics: Brain Stem; Consciousness; Electroencephalography; Encephalitis; Gangliosides; Glasgow Coma Scale; Humans; Male; Middle Aged; Miller Fisher Syndrome; Neurologic Examination; Ophthalmoplegia; Paralysis; Physical Therapy Modalities; Recurrence

Topics: Adult; Autoantibodies; Brain Stem; Encephalitis; Female; G(M1) Ganglioside; Gangliosides; Guillain-Barre Syndrome; Humans; Miller Fisher Syndrome; Muscle Weakness; Ophthalmoplegia

A 37-year-old man developed an acute encephalitic condition after respiratory infection. His condition rapidly deteriorated, and he experienced ophthalmoplegia, tetraplegia, loss of brainstem reflexes and deep tendon reflexes, and deep coma. Electrophysiological evaluations indicated involvement of the peripheral nerve as well as the brainstem. Follow-up studies found acute progression of peripheral nerve damage. Serum anti-GQ1b IgG antibody was present. The initial condition was diagnosed as Bickerstaff's brainstem encephalitis, and subsequent overlapping of Guillain-Barré syndrome probably was responsible for the clinical deterioration. When unusual worsening is observed in clinically suspected encephalitis, neurologists must take into account the possibility of associated Guillain-Barré syndrome and related disorders.

Topics: Adult; Antibodies; Blotting, Western; Brain Diseases; Electrophysiology; Encephalitis; Evoked Potentials, Auditory; Follow-Up Studies; Gangliosides; Guillain-Barre Syndrome; Humans; Male; Neural Conduction; Ophthalmoplegia; Quadriplegia

We describe a 27-year-old woman who showed the clinical triad of Fisher syndrome (ophthalmoplegia, ataxia, and areflexia), a disturbance of consciousness, facial diplegia, and hemisensory loss. Her serum was positive for anti-GQ1b immunoglobulin G (IgG) antibody. The electroencephalographic findings (diffuse slow activity), median somatosensory evoked potential (absent cortical N20 with normal cervical N13), and blink reflex studies (absent R2) suggested central dysfunction, whereas results of facial nerve conduction 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. This case supports the hypothesized continuity between Fisher syndrome and Bickerstaff brainstem encephalitis. These two conditions may represent a single autoimmune disease mediated by anti-GQ1b antibody, usually involving the peripheral and occasionally the central nervous systems.

Topics: Adult; Brain Stem; Electroencephalography; Encephalitis; Evoked Potentials, Auditory, Brain Stem; Evoked Potentials, Somatosensory; Female; Gangliosides; H-Reflex; Humans; Immunoglobulin G; Miller Fisher Syndrome; Neural Conduction; Peripheral Nervous System; Plasmapheresis; Sleep Stages

Topics: Adolescent; Adult; Aged; Autoantibodies; Autoimmune Diseases of the Nervous System; Brain Stem; Child; Child, Preschool; Diagnosis, Differential; Encephalitis; Female; Gangliosides; Guillain-Barre Syndrome; Humans; Immunoglobulin G; Male; Middle Aged; Miller Fisher Syndrome; Ophthalmoplegia

Topics: Adult; Autoantibodies; Brain Stem; Encephalitis; Female; Gangliosides; Humans; Magnetic Resonance Imaging

Four patients with IgG GQ1(b)antibodies were admitted to the Gold Coast Hospital, a public hospital providing medical service to a population of approximately 400,000, over a 4-month period. This represents an unusual cluster of this syndrome, for which there is no apparent reason. Further, the four cases demonstrate the broad spectrum of the disorder from the benign ophthalmoplegia, ataxia and areflexia, Miller Fisher Syndrome, to the severe form with encephalitis (Bickerstaff's brainstem encephalitis).

Topics: Adult; Aged; Brain Stem; Cluster Analysis; Encephalitis; Female; Gangliosides; Humans; Immunoglobulin G; Male; Middle Aged; Miller Fisher Syndrome

A 54-year-old female developed dysarthria, left limb ataxia, and crossed dissociated sensory impairment, followed by external ophthalmoplegia, severe bulbar palsy and bilateral pyramidal tract involvement. A high titer of anti-GQ1b IgG antibody was detected in her serum. Brain MRI (T2 weighted image, diffusion weighted image) and peripheral nerve conduction study were normal. We diagnosed her as having Bickerstaff's brainstem encephalitis. In the past literature, we could not find any case of Bickerstaff's brainstem encephalitis showing crossed dissociated sensory impairment.

Topics: Autoantibodies; Biomarkers; Brain Stem; Diagnosis, Differential; Encephalitis; Female; Gangliosides; Humans; Immunoglobulin G; Lateral Medullary Syndrome; Magnetic Resonance Imaging; Middle Aged

A patient with Bickerstaff's brain stem encephalitis (BBE) associated with anti-GQ1b antibody developed coma, severe weakness, and respiratory distress. The patient required ventilatory support. After having failed to improve on steroids, she was treated with plasmapheresis. She improved concomitantly with the plasmapheresis treatment and made a complete recovery. BBE associated with anti-GQ1b antibody is generally considered to be benign, and specific treatments have not been established. The results with this patient suggest that the condition is not always benign, and plasmapheresis may be beneficial in this disorder.

Topics: Adult; Antibodies; Brain Stem; Coma; Electroencephalography; Encephalitis; Evoked Potentials, Somatosensory; Female; Gangliosides; Glucocorticoids; Humans; Immunoglobulin M; Infusions, Intravenous; Methylprednisolone; Muscle Weakness; Nerve Growth Factors; Plasmapheresis; Respiration, Artificial; Respiratory Insufficiency

The nosological relationship between Bickerstaff s brainstem encephalitis (BBE) and Guillain-Barre syndrome (GBS) has yet to be clarified. We report results of the autopsy of a patient for whom overlapping BBE and GBS was diagnosed clinically. This and similar cases support the original authors' hypothesis that BBE is closely related to GBS.

Topics: Aged; Brain Stem; Encephalitis; Fatal Outcome; Female; Gangliosides; Humans; Immunoglobulin G; Polyradiculoneuropathy

There have been several case reports of acute ophthalmoparesis without ataxia subsequent to infection or immunization. The nosological position and therapy for acute ophthalmoparesis have yet to be established. Sera from patients are reported to have IgG anti-GQ1b antibody, which is frequently found in sera from patients with Fisher's syndrome. To establish the etiology of acute ophthalmoparesis, I tested sera from 8 patients with acute ophthalmoparesis for anti-GQ1b antibody. High IgG anti-GQ1b antibody titers were present in sera from patients in the acute phase of the illness. I describe the successful treatment with plasmapheresis and intravenous immunoglobulin. Some cases of acute ophthalmoparesis following infection or immunization may be categorized as an auto-immune disease related to Fisher's syndrome.

Topics: Acute Disease; Adult; Antibodies, Anti-Idiotypic; Brain Stem; Encephalitis; Female; Gangliosides; Humans; Immunoglobulin G; Immunoglobulins, Intravenous; Middle Aged; Nerve Growth Factors; Ophthalmoplegia; Plasmapheresis

Patients with Guillain-Barré syndrome (GBS) and Fisher's syndrome (FS) benefit from plasmapheresis. Specific treatments, however, have yet to be established for Bickerstaff's brain stem encephalitis (BBE). We showed earlier that sera from BBE patients, as well as from patients with FS and GBS with ophthalmoplegia, have autoantibody to GQ1b ganglioside in the acute phase of the disease. We report 2 BBE patients who had high IgG anti-GQ1b antibody titers and were treated successfully with plasmapheresis.

Topics: Adult; Aged; Brain Stem; Encephalitis; Female; Gangliosides; Humans; Immunoglobulins; Nerve Growth Factors; Plasmapheresis

Topics: Autoantibodies; Brain Stem; Encephalitis; Gangliosides; Humans; Immunohistochemistry

A 17-year-old girl was admitted to our hospital because of drowsiness, diplopia and gait difficulty. She had been well until ten days before admission when fever, drowsiness, headache and general fatigue developed. On admission, there were drowsiness, ophthalmoplegia, ataxia and hyporeflexia. CSF cells and anti-CMV antibody titers increased. CMV-DNA was detected in the CSF by the polymerase chain reaction (PCR). Serum anti-GQ1b antibody was positive. During recovery, forced laughing temporarily appeared. The neurological symptoms disappeared completely. CSF anti-CMV antibody titers became normalized and CSF CMV-DNA-PCR became negative. This is the first case report of Bickerstaff's brainstem encephalitis associated with CMV infection.

Topics: Adolescent; Autoantibodies; Brain Stem; Cytomegalovirus Infections; Encephalitis; Female; Gangliosides; Humans

We presented a case of probable Bickerstaff's brainstem encephalitis. A 39-year-old woman developed easy falling, progressive consciousness disturbance, total ophthalmoplegia, and bilateral pyramida tract sign. In serum anti-ganglioside antibody assay using enzyme-linked immunosorbent assay, high anti-GQ1b antibody was found. Electroencephalography was dominated by alpha activity, superimposed with occasional theta wave bursts. Brain MRI showed abnormal signal hyperintensities in the tegmentum of the midbrain to the upper pons on T2 weighted images and swelling of the midbrain and pons on T1 weighted images. Five days after the onset of the disease, she had respiratory arrest, which necessitated artificial ventilation for a few days. Then, she began to recover. However, she gained only partial recovery, leaving upward gaze palsy, right abducens nerve palsy, pseudbulbar palsy and quadriplegia.

Topics: Adult; Autoantibodies; Brain Stem; Encephalitis; Enzyme-Linked Immunosorbent Assay; Female; Gangliosides; Humans; Magnetic Resonance Imaging