A case of recurrent Miller-Fisher syndrome with multiple anti-ganglioside antibodies / 中国综合临床

Guillain-Barré syndrome (GBS) defines a kind of Immune-mediated acute inflammatory peripheral neuropathy. Miller-Fisher Syndrome (MFS) is a special variant of GBS, with mostly one-way course and rare clinical recurrence. Only a few recurrent cases have been reported in China. Here we report a case of a young male patient with double vision and progressive aggravation of limb numbness, acute onset, with symptoms of upper respiratory tract infection before onset, accompanied by pupil abnormalities and autonomic nervous dysfunction, who was was admitted to our hospital for similar symptoms 3 years ago and was improved by immunotherapy. The patient had a triad of “ataxia, areflexia and ophthalmoplegia”. Cerebrospinal fluid showed protein-cell separation. Serum anti-Sulfatides antibody IgM, anti-GT1a antibody IgG, anti-GQ1b antibody IgG and anti-GM3 IgM were positive. Recurrent MFS was diagnosed and the symptoms improved after immunotherapy. This case suggests that MFS is clinically heterogeneous, a few patients can present with relapse and generally have a better prognosis with immunotherapy. Pre-existing infection and anti-GQ1b antibody production may be predisposing factors for MFS recurrence.

Clinical features of 19 children with Bickerstaff brainstem encephalitis / 中华儿科杂志

Objective@#To summarize the clinical features of Bickerstaff brainstem encephalitis (BBE) in children.@*Methods@#In this retrospective study, data of 19 patients with BBE (11 males and 8 females) were collected from Department of Neurology, Beijing Children′s Hospital from October 2015 to January 2018. The clinical features, treatment and prognosis were analyzed.@*Results@#The onset age of BBE ranged from 1 year and 8 months to 12 years and 11 months. There were 18 cases with preceding infection. The most common infection was upper respiratory tract infection (9 cases), followed by simple fever (5 cases). The most common initial neurological symptoms were lethargy or disturbance of consciousness (8 cases), followed by limb weakness (5 cases). There were 6 cases of simple BBE and 13 cases of BBE overlapping Guillain-Barré syndrome (GBS). Besides the characteristic triad of altered mental status, ataxia, and ophthalmoplegia, there were other symptoms including convulsion (5 cases), diplopia (3 cases), nystagmus (7 cases), facial muscular weakness (7 cases),bulbar palsy (13 cases) and autonomic nerve symptoms (9 cases). Hypo or areflexia was seen in 16 cases. Positive Babinski′s signs were seen in 8 cases. Hyponatremia was present in 10 cases in whom 4 showed severe hyponatremia. Albumin-cytological dissociation of cerebrospinal fluid was seen in 10 cases. The autoimmune antibodies were examined in all 19 patients. Anti-ganglioside antibodies including anti-GM1 IgG antibody was positive in 2 patients and one of whom was also found with positive anti-GD1b IgG antibody. Anti-GQ1b IgG antibody was present in 2 patients. Electromyography was performed in 14 cases and 8 cases, who were all BBE overlapping GBS, showed neurological damage. A total of 16 cases were monitored by video electroencephalography and 8 cases showed slow waves of background. In addition to, interictal focal discharge was detected in 2 cases. T2 fluid-attenuated inversion recovery (FLAIR) sequence abnormal signals were detected in 3 of 18 cases performed brain magnetic resonance imaging (MRI), and lesions involved with brainstem, basal ganglia, thalamus, cerebellum, corpus callosum and cerebral cortex. Lesions involved cervical and thoracic spinal cord were found in 1 out of 11 cases for whom spinal cord MRI was performed. All of the 4 cases who underwent enhanced MRI of spinal had partial nerve roots enhancement. All of the 19 patients received 1 to 2 courses of intravenous immunoglobulin therapy, and 2 cases also received plasma exchange. Fifteen cases received steroid therapy. The following-up period ranged from 3 months to 2.5 years. Two cases were lost to follow-up. Twelve cases achieved a full recovery within 3 months. Three cases recovered within 6 months. One case still had slight limb weakness and ataxia after 1 year and 8 months of follow-up, and another case had left autonomic nerve symptoms in the follow-up of 2 years and 3 months. Both of them were BBE overlapping GBS.@*Conclusions@#Children′s BBE is similar to that in adults, and is frequently found overlapped with GBS. Furthermore, it is sometimes accompanied by central nervous system demyelination disease. The antiganglioside antibodies are not often detectable. Immunoglobulin therapy could usually achieve good response. The prognosis of simple BBE is good in most situations. For BBE overlapping GBS, the more severe the limb weakness during the peak of disease is, the slower the recovery would be.

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.

Síndrome de Miller Fisher en un niño de 12 años / Miller Fisher Syndrome in a 12 year old boy

El Síndrome de Miller Fisher (SMF) es una variante del Síndrome de Guillain Barré (SGB), caracterizado por la tríada clínica de oftalmoplejía, ataxia y areflexia. Se presenta el caso de un niño de 12 años de edad, examinado con un tiempo de enfermedad de 4 días y con una variedad de síntomas que incluían ptosis palpebral, somnolencia, marcha tambaleante y debilidad muscular, asociados a antecedente de infección respiratoria de vías altas. El examen clínico demostró paresia del III, IV, y VI nervios craneales de ambos ojos, arreflexia y debilidad distal en extremidades. Se instaló tratamiento con Inmunoglobulina intravenosa que condujo a una evolución clínica satisfactoria.

The Miller Fisher Syndrome (MFS) is a variant of the Guillain Barre Syndrome (GBS), characterized by the clinical trial of ophthalmoplegia, ataxia and areflexia. The case of a 12 year old boy is examined with a 4-day long history characterized by symptoms such as palpebral ptosis, somnolence, ataxia and muscle weakness, associated with a history of upper respiratory infection. Clinical examination showed paresis of III, IV, and VI cranial nerves of both eyes, areflexia, and distal weakness in the extremities. Treatment with intravenous immunoglobulin was established, leading to a satisfactory clinical evolution.

Clinical analysis of four cases of anti GQ1b syndrome / 中华儿科杂志

Objective@#To investigate the clinical manifestations, laboratory findings, treatment and outcome of anti-GQ1b antibody syndrome.@*Method@#The clinical manifestations, laboratory examination, diagnosis, treatment and prognosis of (4 patients 4 male patients, from 4 to 12 years) with anti-GQ1b syndrome in Beijing Children's Hospital affiliated to Capital Medical University from 2015 to 2016 were retrospectively analyzed.@*Result@#All 4 children presented with ataxia. Case 1 showed impaired speech, ptosis and weakness of arms; case 2 and 3 had external ophthalmoplegia, weakness of limbs; case 4 presented hypersomnia, irritability and hallucinations. Serum anti-GQ1b-IgG antibody was positive in all cases. Case 1-3 received lumber puncture at the course of 1-2 weeks, CSF presented albuminocytological dissociation, case 4 had CSF pleocytosis and increased protein level. Brain MRI of Case 1-2 were normal; Case 3 showed long T1 and T2 signal in cerebellar dentate nucleus, pons and corpus callosum; Case 4 showed long T1 and T2 signal in bilateral centrum semiovale, basal ganglia, external capsule, insula and cerebellum. Electromyograms of case 1-3 showed peripheral axonal lesion. All children were treated with IVIG. After treatment, condition of all patients were improved. According to the clinical manifestation, laboratory examination, and outcome after treatment, case 1 was diagnosed as anti-GQ1b antibody syndrome (Pharyngeal-Cervical-Brachial weakness overlapped with Miller Fisher syndrome), case 2 and 3 were diagnosed as anti-GQ1b antibody syndrome (Miller Fisher syndrome overlapped with Guillain Barré syndrome) and case 4 was diagnosed as anti-GQ1b antibody syndrome (acute ataxia hypersomnolence).@*Conclusion@#When patients with the presence of prodromic infections, monophasic course, drowsiness, ataxia, ophthalmoplegia, weakness and the symptoms/signs are relatively symmetric, anti-GQ1b antibody syndrome should be considered. Anti-GQ1b antibody has important significance for diagnosis. Most children have a good prognosis. Early correct diagnosis can avoid unnecessary examinations and guide appropriate use of immunotherapy.

Diagnosis strategy of Guillain-Barré syndrome spectrum disorders / 中华实用儿科临床杂志

Guillain-Barré syndrome(GBS) has clinical characteristics:flaccid,symmetrical,ascending paralysis.Cranial nerves and respiratory muscle related,albuminocytologic dissociation in cerebrospinal fluid,and electrophysiological changes.GBS was believed to be an autoimmune perineuropathy.Recently,there were more and more reports about GBS spectrum disorders or GBS variant correlated with anti-GQ1b antibody or anti-GM1 IgG antibody et al.The GBS Classification Group presented the new clinical criteria in 2014,to enable neurologists and non-neurologists to diagnose GBS and all its variants using a simple yet all-inclusive classification system.

Oculopharyngeal Palsy in Patients with Coexistence of Anti-GQ1b and GT1a Antibodies

The presence of antiganglioside antibodies is closely associated with the clinical characteristics of Guillain-Barre syndrome (GBS), as evidenced by the presence of anti-GQ1b antibody in Miller-Fisher syndrome and anti-GT1a antibody in a pharyngeal-cervical-brachial variant of GBS. We report herein three patients harboring both anti-GT1a and anti-GQ1b antibodies who all exhibited oculopharyngeal palsy and additional features of ataxia, facial palsy, internal ophthalmoplegia, and visual disturbance. The findings of this study suggest that oculopharyngeal palsy is a common clinical manifestation determined by the coexistence of anti-GQ1b and GT1a antibodies.

An Adult Case of Fisher Syndrome Subsequent to Mycoplasma pneumoniae Infection

Reported herein is an adult case of Fisher syndrome (FS) that occurred as a complication during the course of community-acquired pneumonia caused by Mycoplasma pneumoniae. A 38-yr-old man who had been treated with antibiotics for serologically proven M. pneumoniae pneumonia presented with a sudden onset of diplopia, ataxic gait, and areflexia. A thorough evaluation including brain imaging, cerebrospinal fluid examination, a nerve conduction study, and detection of serum anti-ganglioside GQ1b antibody titers led to the diagnosis of FS. Antibiotic treatment of the underlying M. pneumoniae pneumonia was maintained without additional immunomodulatory agents. A complete and spontaneous resolution of neurologic abnormalities was observed within 1 month, accompanied by resolution of lung lesions.

A Case of Miller Fisher Syndrome in a Patient with Systemic Lupus Erythematosus / 대한내과학회지

Neuropsychiatric events are common in patients with systemic lupus erythematosus (SLE). The estimated incidence of neuropsychiatric SLE (NPSLE) is 30 to 40%. However, NPSLE poses a difficult diagnostic challenge because a variety of conditions should be considered in the differential diagnosis, especially when patients present with uncommon or rare NPSLE features. We herein describe a 49-year-old man with SLE who initially presented with diplopia, ptosis, and gait disturbance that had developed 1 week after an upper respiratory tract infection. He was finally diagnosed with Miller Fisher syndrome (a variant of Guillain-Barre syndrome) according to clinical symptoms, anti-GQ1b antibody positivity, and neurological study results. The patient recovered without sequelae with intravenous immunoglobulin therapy. This is the first report to describe a case of Miller Fisher syndrome that developed in a patient with SLE in Korea and suggests that Miller Fisher syndrome should be included as a differential diagnosis of NPSLE.

A Case of Miller Fischer Syndrome With Optic Nerve Involvement

PURPOSE: To report an extremely rare case of optic nerve involvement in Miller-Fisher syndrome. CASE SUMMARY: A 74-year-old woman presented to our clinic with decreased visual acuity in both eyes. Such symptoms started 1 month prior to her visit, after sudden onset of left-side motor power weakness and dysphasia. Following the initial symptoms, our patient consecutively experienced worsening dysphagia, ptosis, and lateral gaze limitation. We confirmed the diagnosis as Miller Fischer syndrome with a positive anti-GQ1b antibody serology. She received immunoglobulin treatment for 5 days in other departement. She visited our clinic due to continuing diplopia, decrease of visual acuity and gait disturbance. On initial examination, the corrected visual acuity was 0.5 in both eyes. Ptosis of both eyelids, esodeviation, limitation in ocular movement was noted. The fundoscopic examinations revealed relatively pale optic disc. There was decrease in color vision in both eyes. The visual evoked potential test showed low amplitude and delayed latency in P100 wave in both eyes. Two months after her initial visit to our department her symptoms started to improve, and after 4 months all the initial problems resolved completely and her corrected visual acuity checked out to be 0.8 in both eyes and VEP abnormality was recovered. CONCLUSIONS: It is extremely uncommon for Miller Fisher syndrome to involve the optic nerve. We present such a case of a 74-year-old woman whose symptoms improved 4 months after its first attack with treatment.

A Case of Acute Internal and External Ophthalmoplegia without Ataxia and Areflexia Associated with Anti-GQ1b Antibody

No abstract available.

Three Cases of Anti-GQ1b Antibody Syndrome with Internal Ophthalmoplegia

The anti-GQ1b IgG antibody is associated with Guillain-Barre syndrome (GBS) with ophthalmoplegia, Bickerstaff's brainstem encephalitis, acute ophthalmoparesis without ataxia, and ataxic GBS without ophthalmoplegia as well as Miller Fisher syndrome. Only limited numbers of patients with acute ophthalmoparesis without ataxia are known to show internal ophthalmoplegia. We report three patients with internal ophthalmoplegia from anti-GQ1b antibody syndrome.

Two Cases of Miller Fisher Syndrome without Ataxia

A classic triad of acute external ophthalmoplegia, areflexia and ataxia characterizes Miller Fisher syndrome(MFS). Diagnosis is based on clinical findings and supported by two laboratory findings; CSF albuminocytological dissociation and serum anti-GQ1b IgG antibody testing. Anti-GQ1b antibody is a key factor in the pathogenesis of Miller Fisher syndrome and a useful marker in laboratory diagnosis. Here we report 2 cases with Miller Fisher syndrome without ataxia, whose major symptom was acute external ophthalmoplegia. Case 1 was associated with preceding Epstein-Barr virus infection and negative anti- GQ1b antibody. Case 2 was positive for the anti-GQ1b antibody. Both received intravenous immunoglobulin and fully recovered within 2 months after the onset of disease.

A Case of Child with Miller Fisher Syndrome Diagnosed by Anti-GQ1b Antibody from Mycoplasma Pneumoniae

Miller Fisher syndrome, first reported by Miller Fisher in 1956, is characterized by a triad of external ophthalmoplegia, areflexia, and ataxia. Many features shared with Guillain-Barre syndrome; CSF usually shows elevated proteins and the syndrome is often is preceded by an infectious disorder. It is believed that the level of anti-GQ1b IgG antibody is elevated during an acute phase, increases and decreases rapidly during clinical recovery, that the level of anti-GQ1b IgG can be used as a diagnostic tool for Miller Fisher syndrome during an acute phase. We report an 8 year-old boy who showed typical clinical manifestations of Miller Fisher syndrome, with respiratory tract illness, associated with the seroconversion of Mycoplasma pneumoniae titers during the development of neurological symptom, with positive anti- GQ1b IgG.