Anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma: a case report / 中华神经科杂志

Paraneoplastic neurological syndromes (PNS) are heterogeneous disorders caused by autoimmune responses of cancer, which can affect any part of the nervous system. Anti-amphiphysin antibody is one of the high-risk PNS antibodies, which is usually associated with small cell lung cancer and breast cancer. However, extrapulmonary neuroendocrine carcinoma is rare in patients with anti-amphiphysin antibody. A case of anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma is reported. The tumor was detected by fluorine 18 fluorodeoxyglucose positron emission tomography and pathologically confirmed by gastroscopic biopsy. The patient′s neurological symptoms were partially improved after treatment of intravenous immunoglobulin and glucocorticoids. However, the disease prognosis is closely related to the accompanying tumor.

Síndrome anti-GQ1b en un niño con síndrome de Miller-Fisher-Bickerstaff / Anti-GQ1b syndrome. A child with Miller-Fisher-Bickerstaff syndrome

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-Fisher­Bickerstaff 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 específico

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 twelve-year-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 determination

Sobreposición del síndrome de Miller Fisher y encefalitis de Bickerstaff en pediatría: reporte de caso / Overlap of Miller Fisher syndrome and Bickerstaff encephalitis in pediatrics: Case report

RESUMEN Se describe el caso en pediatría de la sobreposición del síndrome de Miller Fisher y la encefalitis de Bickerstaff en presencia de perfil de anticuerpos positivos para anti-GQ1b en un niño de 6 años, quien presenta un compromiso tronco-encefálico y luego entra en una encefalopatía con compromiso de nervio periférico. El presente caso es relevante en relación con los escases de artículos semejantes en la literatura pediátrica, con pocos precedentes en la literatura publicada hasta la fecha.

SUMMARY To describe the pediatric case of the overlap of Miller Fisher syndrome and Bickerstaff encephalitis in the presence of an anti-GQ1b positive antibody profile in a 6-year-old boy who presents with a brainstem compromise and progress to encephalopathy with peripheral nerve compromise, the present case is relevant in relation to the scarcity of similar articles in pediatric literature with few precedents in the literature published to date.

Analysis of clinical features of brainstem encephalitis manifested as Parkinson syndrome / 中国神经精神疾病杂志

Objective To analyze the clinical manifestations and imaging features of human brainstem encephalitis with Parkinson syndrome as the main manifestations. Methods Combined with related literature, the clinical, laboratory and imaging features of three patients with brainstem encephalitis, which were mainly manifested as Parkinson syndrome, were analyzed. Results The 3-hour video EEG showed normal in 2 cases and abnormal in one case with more delta wave in each leads. Cerebrospinal fluid examination revealed normal cell numbers in one case, increased cell numbers in 2 case and elevated cerebrospinal fluid proteins in all 3 cases. serum and CSF IgG anti-NMO antibodies as well as autoimmune encephalitis antibodies were negative in 3 cases. Brain MRI showed symmetrical abnormal signal in bilateral midbrain and pia mater enhancement increased in 3 cases. After hormone, and madopar treatment, Parkinson syndrome symptoms were significantly improved in two patients. Conclusion Brainstem encephalitis-induced symmetrical midbrain lesion can manifest as Parkinson syndrome. The laboratory tests reveal an involvement of immune system and hormone therapy may be effective.

The probability and timing of Miller-Fisher syndrome progressing to Guillain-Barre syndrome or Bickerstaff brainstem encephalitis in childhood / 临床儿科杂志

Objective To investigate the probability and timing of childhood Miller-Fisher syndrome (MFS) progressing to Bickerstaff brainstem encephalitis (BBE), classical Guillain-Barre syndrome (GBS), and pharyngeal-cervical-brachial (PCB-GBS). Methods The clinical data of 128 children with confirmed MFS diagnosis were retrospectively analyzed. Results Among 128 children, 60 cases were simple MFS (ocular muscle paralysis, ataxia, reflexes diminished or disappeared, without limbs weakness and lethargy; laboratory tests suggest cerebrospinal fluid protein-cell separation and/or serum anti-GQ1b antibody positive), 28 cases developed MFS/PCB-GBS (met MFS diagnosis criteria, accompanied by weakness of pharynx, neck and upper limb, weakened or disappeared of upper limb reflex, without weakness of lower limb), 22 cases developed MFS/GBS (met MFS diagnosis criteria, accompanied by weakness of limb), 18 cases developed MFS/BBE (met MFS diagnosis criteria, accompanied by lethargy, pyramidal tract positive). There were no differences in the age at onset, the interval from onset to the start of the treatment, Hughes functional grading, and the percentage of cases having a history of preceding infections, the rate of positive serum anti-GQ1b antibody, the ratio of albumin cytological dissociation in cerebrospinal fluid among 4 groups (P>0.05). The interval from MFS onset to progression to MFS/PCB-GBS, MFS/GBS, or MFS/BBE was within 10 days. Conclusions In children with MFS, 50% developed PCB-GBS, GBS, or BBE, which occurred within 10 days after onset. Clinicians should pay attention to the time window and adjust the medicine rationally.

The role of tracheotomy in children with severe hand foot and mouth disease / 中国小儿急救医学

Objective To investigate the role of tracheotomy in children with severe hand foot and mouth disease.Methods Eight children with severe hand foot and mouth disease collected from July 2009 to August 2011 in PICU were retrospectively reviewed.Clinical manifestation and laboratory data were analyzed and followed up.Results Eight patients were enterovirus 71 (EV71) positive.Hormones,intravenous immune globulin,mechanical ventilation and vasodilators had been administered in patients with cardiopulmonary failure.They had severe neurologic sequelae,requiring prolonged intubation.MRI imaging of 7 cases showed abnormal brainstem,pons and medulla oblongata,1 case showed swelling of brain tissue.A total of 8 patients underwent a tracheotomy in mean (25.63 ± 8.86) d.The older was 22 months,the younger was 8 months and the mean age was (13.13 ±4.22) months.Four cases died,the other survived.Two cases were successfully decannulation,the other two were still in the tracheotomy.Conclusion Tracheostomy play an active role in reducing airway resistance,clearing airway secretions,providing the opportunity for subsequent rehabilitation.Eight patients were followed up for three years,neurological sequelae could not be restored.Four cases repeatedly suffer from pneumonia,atelectasis,leading to a serious decline in quality of life.

Overlapping Guillain-Barre syndrome and Bickerstaff's brainstem encephalitis associated with Epstein Barr virus / 소아과

A flaccid tetraparesis in Bickerstaff's brainstem encephalitis (BBE) is presumed to be a sign of overlapping Guillain-Barre 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.

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

No abstract available.

Severe hand foot and mouth disease in 68 children with brainstem encephalitis / 中国小儿急救医学

Objective To explore the clinical features and risk factors of children with severe hand foot and mouth disease (HFMD).Methods The clinical data of 68 children with HFMD associated with brainstem encephalitis were analysed retrospectively from Apr to Sep 2010.Results Among the 68 cases,63cases(92.6％ ) were younger than 3 years.The major symptoms and physical signs of 68 cases included rash (68 cases,100％ ),fever (68 cases,100％ ),fatigue (68 cases,100％ ),irritability (65 cases,95.6％ ),somnolence (42 cases,61.8％ ),vomiting (28 cases,41.2％ ),limb tremor (23 cases,33.8％ ),tendon reflexe hyperactivity (60 cases,88.2％ ),tachypnea or respiratory rhythm abnormality (63 cases,92.6％ ),tachycardia (65 cases,95.6％ ),and hypertension (54 cases,79.4％ ).Twenty-five cases (36.8％,25/68 ) had leucocytosis ( ＞ 12 × 109/L ),and 19 cases ( 27.9％,19/68 ) had hyperglycaemia.X-ray appearances:patchy and pathy shadows in single or bilateral pulmonary were seen in 46 cases( 67.6％,46/68 ).Forty-eight cases were examined by MRI,eight cases displayed ischemic lesions or demyelination.Spinal cord MRI was performed in 3 cases with flaccid paralysis,which showed demyelination.The etiology indicated that 38 cases ( 55.9％,38/68 ) were infected by enterovirus 71,25 cases( 36.8％,25/68 ) were infected by other enterovirus,5 cases (7.3％,5/68) were negative.All the cases were treated by mechanical ventilation with tracheal intubation,in whom 63 cases recovered well,4 cases improved,and 1 case gave up to die.Conclusion Ages ＜ 3 years,enterovirus 71 infection,continual fever,fatigue,somnolence,irdtability,vomiting,limb tremor,tendon reflexes hyperactivity,tachypnea or respiratory rhythm abnormality,tachycardia and hypertension are the high risks of critically ill children associated with severe HFMD.To reduce the fatality rate of HFMD,it is crucial to early judge the high risk factors,and take mechanical ventilations earlier.

Progress of severe hand, food and mouth disease / 国际儿科学杂志

The virus of hand, foot and mouth disease(HFMD) damages nerve system.Brainstem is the main site and has some specific receptors.Severe HFMD has a high mortality.The main causes of death are cardiopulmonary and brain failure, so it is very important that risk factors of severe HFMD are timely judged and treated. Pulmonary edema of severe HFMD is mainly caused by neurological damage. At present, although it has still no standardized treatment scheme, the diagnosis and treatment for different stages have a significant effect.

A Case of Brainstem Encephalitis Associated with Epstein-Barr Virus Infection / 대한소아신경학회지

A case of brainstem encephalitis associated with a reactivated infection of Epstein-Barr virus (EBV) in a 7-year-old boy is reported. The patient was diagnosed based on the compatible clinical, cerebrospinal fluid and radiological findings and the serological results for EBV, had a favorable clinical course and showed a discrepancy between the course of the neurological features and the evolution of the imaging alterations. The apparent diffusion coefficient imaging revealed the brain stem lesion suggesting an autoimmune mechanism in the pathogenesis of the illness.

Alucinações múltiplas devido a lesão no tronco cerebral: relato de caso / Multiple hallucinations due to brainstem injury: a case report

We report a case of a 43-year-old woman with brainstem encephalitis in the third trimester of pregnancy. She presented complex visual and auditory hallucinations in the acute disease phase (hearing opera arias and seeing room furniture upside-down). Hallucinations resolved with antiviral treatment.

Caso de uma mulher de 43 anos, gestante, que apresentou lucinações visuais e auditivas (árias de ópera e visão de móveis do quarto de cabeça para baixo) associadas a fase aguda de encefalite de tronco cerebral. Os sintomas desapareceram com resolução do quadro.

A Case of Brainstem Encephalitis Presented with Diplopia and Headache / 대한소아신경학회지

Brainstem encephalitis is a rare disease and patients typically present with symptoms of areflexia, ataxia, and ophthalmoplegia. We experienced a case of brainstem encephalitis in a 7 years old girl, who presented with diplopia and headache. It can be reliably confirmed by magnetic resonance imaging(MRI) and CSF analysis. We report a case of brainstem encephalitis in a child, with a brief review of literature.

Anti-GQ1b Antibody Syndrome; Is the Another Name of Miller Fisher Syndrome or Advent of a New Syndrome?

The Miller Fisher syndrome (MFS), characterized by ataxia, areflexia, and ophthalmoplegia, is a localized variant of Guillain-Barre syndrome (GBS). Bickerstaff's brainstem encephalitis (BBE) is a related syndrome in which central nervous system abnormalities accompany the classic triad. The discovery of the anti-GQ1b antibody and localization of GQ1b ganglioside in human nervous system enabled us to understand various kinds of symptoms in MFS and related diseases. Molecular mimicry of antigenic epitope from infective organisms such as Campylobacter jejuni with this ganglioside is likely the predominant pathogenic mechanism. This could explain the unusual conditions such as atypical MFS, GBS with ophthalmoplegia and BBE are various manifestations of post-infectious autoimmune neuropathies. Now, we can speculate them as the anti-GQ1b antibody syndrome in according to their immunological profiles. In addition to this, recent new concept of anti-ganglioside complex antibody will lead us to further understanding of these disorders.

Two Cases of Methyl Bromide Intoxication Mimicking Bickerstaff's Brainstem Encephalitis

BACKGROUND: Methyl bromide is toxic to the central and peripheral nervous systems. Two patients with occupational exposure to this agent are described. CASE: Two cases of previously healthy young men were involved. They had worked in a fumigating plant spraying fruits using methyl bromide for several months before admission. They showed general weakness, severe ataxia, bilateral dysmetria and bilateral lateral gaze limitation. And gaze-evoked nystagmus was observed in one of them. Brain MRI showed symmetrically increased signal intensity lesions including the brainstem, cerebellum and splenium of corpus callosum. After conservative treatment, they gradually improved over the next few weeks. CONCLUSIONS: Symmetry and topography of our patients??clinical and radiographic abnormalities support the proposal that methyl bromide exposure can produce symptoms similar to Bickerstaff's brainstem encephalitis.

Rehabilitation of Bickerstaff's Brainstem Encephalitis with Guillain-Barre Syndrome: A case report

We reported a 32-year-old man diagnosed as Bickerstaff's brainstem encephalitis with Guillian-Barre syndrome. After plasmapheresis, his consciousness and respiratory function and motor strength improved. He was discharged without rehabilitation treatment and could perform activities of daily living independently on wheel chair level. For reducing cocontraction of lower extremity muscles, neuromuscular reeducation using EMG biofeedback was performed after admission. After a month of treatment, gait pattern was improved. He received rehabilitative managements such as pool therapy with gait training and improved to 4/5 grade at proximal lower extremities, but the endurance and the quality for his walking was poor because of the cocontraction of muscles in lower extremities. EMG biofeedback for the neuromuscular reeducation leading to each muscle's isolated movements was done. After 2-month rehabilitation, he could walk over 20 meters even level independently without walking aids. This case could be a good model for the effective neuromuscular reeducation.

A Case of Recurrent Bickerstaff's Brainstem Encephalitis with an Overlap of Guillain-Barre Syndrome

Bickerstaff 's brainstem encephalitis (BBE) is characterized by acute ophthalmoplegia and ataxia. Some patients with BBE show mental changes, pyramidal signs and sensory disturbance. A forty-three year old woman was diagnosed as BBE in 1996. In 2000, she admitted again with complaints of ataxia and ophthalmoplegia. She was diagnosed as recurrent BBE and an overlap of Guillain-Barre syndrome (GBS). This is the first case of an overlap of BBE and GBS, as well as BBE in Korea.

A Case of Brainstem Encephalitis

We experienced a case of brainstem encephalitis that is possibly caused by herpes simplex virus infection in a 3 year-old girl. The diagnosis was made by clinical symptoms, neurologic exam, serologic test, brain CT, brain MRI and brainstem biopsy. Herpes simplex virus specific antibody was detected from cerebrospinal fluid and the titer was 11.78 by enzyme immunoassay. Stereotaxic brainstem biopsy was done for differenciate from brainstem glioma at 24th hospital day. Patient was treated with acyclovir and improved in association with neurologic sequallae at 3 months later. Brief review of literatures was made.

A Case of Herpes Simplex Virus Induced Focal Brainstem Encephalitis: A Case Report

Focal brainstem encephalitis due to Herpes simplex virus if a very rare infectious disease, occurs with a frequent of 1-2 million population per year. A 3 years old girl is described with infection due to Herpes simplex virus causing brainstem encephalitis. The diagnosis was established by enzyme immunosorbent assays of the cerebrospinal fluid and serum which demonstrated antibody responsed to Herpes simplex virus. The clinicopathological features, radiological findings, laboratories and brainstem biopsies are discussed in the context of the literature.

Clinical features and differential diagnosis of Miller-Fisher syndrome and Bickerstaff's brainstem encephalitis / 临床神经病学杂志

Objective To study the clinical characteristics and differential diagnosis of Miller-Fisher syndrome (MFS) and Bickerstaff's brainstem encephalitis (BBE).Methods The clinical data of 12 patients with diagnosis of MFS and 15 patients with diagnosis of BBE were analyzed retrospectively.Results In MFS group, the patients showed diplopia (12 cases), loss of pupillary light reflex (3 cases), peripheral facial paralysis (7 cases), hydroposia bucking (1 case), weakness of limbs (6 cases), sensory disability (4 cases) and ataxia (10 cases). In BBE group, the patients presented with diplopia (11 cases), central facial palsy (4 cases), tinnitus (4 cases), hydroposia bucking (6 cases), weakness of limbs (10 cases), sensory disability (2 cases), ataxia (10 cases), somnolence or coma (5 cases) and positive Babinski's sign (9 cases). CSF examination showed elevation of protein in 10 patients from BBE group and 10 patients from MSF group, however, leucocytosis was found in 6 cases from BBE group and 2 cases from MSF group. There were 4 patients of MFS and 1 patient of BBE with abnormal results of electromyogram (EMG) examination. 6 patients in BBE group had abnormal results of CT or MRI examinations, and the lesions mainly located in brainstem, thalamus and so on. Both groups had good prognosis after therapies. Conclusions MFS and BBE have similar and overlapped clinical characteristics. This indicates that these two diseases are closely related and may form a continuous spectrum. The main identifications of BBE are conscious disturbance, positive reflex of Babinski's sign and abnormal imaging results in some patients.