Bickerstaff's Brainstem Research Articles

Clinical correlates of serum anti-GT1a IgG antibodies

Patients with the pharyngeal-cervical-brachial variant (PCB) of Guillain–Barré syndrome (GBS) have anti-GT1a IgG with or without GQ1b reactivity, whereas those with Fisher syndrome (FS) or Bickerstaff's brainstem encephalitis (BBE) have anti-GQ1b IgG antibodies which cross-react with GT1a. The nosological relationship between these conditions has yet to be established. To investigate the relationships between each manifestation and between clinical features and the coexistence of anti-GQ1b IgG, we reviewed neurological signs present during illnesses of 140 patients who had anti-GT1a IgG. Based on our criteria, FS was diagnosed for 64 (46%) patients, GBS for 22 (16%), BBE for 14 (10%), and PCB for 6 (4%). Overlapping conditions were diagnosed for some patients: FS and GBS (5%), PCB and FS (5%), BBE and GBS (4%), and PCB and BBE (1%). Patients who initially had bulbar palsy developed not only PCB but FS or BBE. The population of anti-GT1a-positive patients frequently had ophthalmoplegia, ataxia, and areflexia, whereas the subpopulation who had anti-GT1a IgG without GQ1b reactivity frequently had preceding diarrhea as well as oropharyngeal, neck, and limb weakness. Patients with anti-GT1a IgG presented a variety of clinical conditions, indicative of a continuous clinical spectrum. A major part of this clinical variation was due to the coexistence of anti-GQ1b IgG. The presence of a common autoantibody (anti-GT1a IgG) and overlapping illnesses suggests that PCB is closely related not only to GBS but to FS and BBE as well.

Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barre syndrome

Bickerstaff's brainstem encephalitis: clinical features of 62 cases and a subgroup associated with Guillain-Barre syndrome

Bickerstaff's brainstem encephalitis associated with IgM antibodies to GM1b and GalNAc-GD1a

This is the first report of a case of Bickerstaff's brainstem encephalitis (BBE) associated with IgM antibodies to GM1b and GalNAc-GD1a. Subsequent to Campylobacter jejuni enteritis, the patient rapidly developed consciousness disturbance and hyperreflexia in addition to external ophthalmoplegia and cerebellar-like ataxia. EEG showed transient 7 Hz monorhythmic θ activities, predominantly in the front-central area. He received high doses of immunoglobulin intravenously and had completely recovered 3 months later. High anti-GM1b and anti-GalNAc-GD1a IgM antibody titers present during the acute phase decreased with his clinical improvement. An absorption study showed the anti-GM1b and anti-GalNAc-GD1a IgM antibodies to be cross-reactive. Anti-GM1b and anti-GalNAc-GD1a antibodies have been detected in some patients who developed Guillain–Barré syndrome after C. jejuni enteritis, whereas the anti-GQ1b IgG antibody is associated with BBE. Infection by C. jejuni bearing a GM1b-like or GalNAc-GD1a-like lipooligosaccharide may trigger the production of anti-GalNAc-GD1a and anti-GM1b IgM antibodies. It is not clear why our patient developed BBE rather than Guillain–Barré syndrome. These antibodies may, however, prove useful serological markers for identifying BBE patients who do not have the anti-GQ1b IgG antibody.

Clinical deterioration in Bickerstaff's brainstem encephalitis caused by overlapping Guillain-Barré syndrome

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.

High-dose Intravenous Immunoglobulin Therapy in a Child Case of Bickerstaff's Brainstem Encephalitis

High-dose Intravenous Immunoglobulin Therapy in a Child Case of Bickerstaff's Brainstem Encephalitis

Involvement of the central nervous system in Miller Fisher syndrome: a case report

Miller Fisher syndrome (MFS) is characterised by ophthalmoplegia, ataxia and areflexia. Reports on cerebellar ataxia and supranuclear oculomotor derangement in MFS suggested an additional involvement of the central nervous system (CNS), resembling Bickerstaff's brainstem encephalitis (BBE). In the present report, a patient with a monophasic acute illness, early recovery and specific clinical-laboratory findings suggested both intrinsic brainstem and peripheral nerve disease (MFS and BBE). In pons and medulla oblangata, blurred to discrete T2-lesions were revealed by cranial MRI, while involvement of peripheral nerves was detected with EMG. The CSF showed no increase in protein or cell content, such as occurs in brainstem encephalitis.

Differential diagnosis of posterior fossa multiple sclerosis lesions--neuroradiological aspects.

Various infratentorial pathological conditions can mimic multiple sclerosis (MS) both clinically and radiologically. We review the inflammatory, vascular, neoplastic and metabolic conditions which show features similar to those of MS on magnetic resonance imaging (MRI). Behcet's disease, Lyme disease, progressive multifocal leukoencephalopathy, neurosarcoidosis, Whipple's disease, listeria rhombencephalitis, Bickerstaff's brainstem encephalitis, vasculitis due to systemic lupus erythematosus, and acute disseminated encephalomyelitis produce inflammatory lesions similar to those of MS in the brainstem and cerebellum. Neoplastic diseases, in particular pontine gliomas and lymphomas, can mimic MS. Vascular ischaemic lesions, either due to infarction produced by occlusion of a major posterior circulation artery or due to small vessel vasculopathy, can lead to posterior fossa lesions. The MRI changes of central pontine myelinolysis can also mimic MS. Diffuse axonal injury, radiation and chemotherapy induce lesions that resemble MS, however the clinical history will exclude these possibilities. Finally, we discuss a few conditions which are similar to MS in clinical presentation but have different MRI appearances, such as brainstem cavernomas, posterior fossa tumoural lesions, aneurysms and vascular loops producing neurovascular conflicts. Analysis of the MRI findings with clinical history and laboratory data helps to narrow down the diagnosis of the infratentorial pathology.

Overlapping Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis associated with anti-GQ1b IgG antibody after herpes simplex virus infection

Herpes simplex virus (HSV) is a rare, antecedent infectious agent in Guillain-Barré syndrome (GBS). We report a patient with overlapping GBS and Bickerstaff's brainstem encephalitis (BBE). The patient had a vesicular lesion on her nose. Antecedent HSV type 1 (HSV-1) infection was confirmed by isolation of the virus and detection of the presence of serum anti-HSV-1 IgM antibody during the acute phase. Her serum IgG had high anti-GQ1b antibody titer. External ophthalmoplegia has been noted in 2 of 4 reported cases of HSV-associated GBS. Herpetic brainstem encephalitis cases of poor prognosis are known, but only 2 cases of benign brainstem encephalitis secondary to HSV infection, in which there was acute ophthalmoplegia and clinical features consistent with those of BBE have been reported.

Fine specificity of anti-GQ1b IgG and clinical features

Anti-GQ1b IgG frequently is present in sera of patients with Miller Fisher syndrome (MFS), Guillain-Barré syndrome (GBS) with ophthalmoplegia, Bickerstaff's brainstem encephalitis (BBE), and acute ophthalmoparesis (AO) in the acute phase. Why various clinical signs develop under these conditions, however, has yet to be clarified. We investigated the fine specificity of anti-GQ1b IgG and its clinical correlation in sera from 82 patients: 56 with MFS, 11 with GBS, 13 with BBE, and 2 with AO. Anti-GQ1b IgG antibodies were absorbed by GT1a in 80 (98%) of the 82 sera, by GD1b in 11 (13%), and by the other b-series gangliosides GD3, GD2, or GT1b in 24 (29%). The most frequent pattern of fine specificity was the cross-reaction with GT1a alone, seen in 56 (68%) samples. Of the 11 patients with anti-GQ1b IgG, cross-reacting with GD1b, 6 (55%) had impaired deep sense, and the association was significant ( p=0.02). This is the first study to show that the fine specificity of anti-GQ1b IgG is heterogeneous and that the difference is correlated with the presence of a particular clinical sign.

Anti-GQ1b IgG antibody syndrome: clinical and immunological range

OBJECTIVESTo clarify the nosological relation among Miller Fisher syndrome (MFS), Guillain-Barré syndrome (GBS) with ophthalmoplegia, Bickerstaff's brain stem encephalitis (BBE), and acute ophthalmoparesis without ataxia. Serum samples from patients with...

Brainstem involvement in a child with ophthalmoplegia, ataxia, areflexia syndrome

The anatomic location and pathogenesis of the ophthalmoplegia, ataxia, areflexia syndrome, which includes both the Miller-Fisher syndrome and Bickerstaff's brainstem encephalitis, continues to be a matter for debate. A patient with this syndrome is reported, and the brainstem location of the lesion and elevated titers of anti-GM1 antibodies in serum are demonstrated.

Cranial polyneuropathy with elevated serum antiganglioside antibody

We report a 6-year-old girl with cranial polyneuropathy with elevated serum levels of antiganglioside antibodies. She manifested herpetic vesicles around the right upper eyelid and mouth without antecedent infection. She developed facial asymmetry and double vision 5 days after the first apperance of the vesicles. Neurological examination on admission disclosed palsies of the bilateral sixth and twelfth cranial nerves and right third and seventh cranial nerves, but limb muscle weakness, ataxia, and areflexia were not observed. Cerebrospinal fluid examination and MRI of the brain showed no abnormalities. Serum antibodies to gangliosides GQ1b and GT1b, but not GM1, and those to Campylobacter jejuni were significantly increased on admission and on the hospital day 14. These observations suggest that the present case is a variant form of Miller Fisher syndrome or Bickerstaff's brainstem encephalitis subsequent to asymptomatic C. jejuni infection. We treated her with intravenous administration of high-dose methylprednisolone and acyclovir, but almost no effect was observed. All cranial nerve palsies, however, had resolved completely approximately 4 months later. This may be the first pediatric case in which cranial polyneuropathy and antiganglioside antibodies were associated.

Overlap of Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis

The nosological relationship between Bickerstaff's brainstem encephalitis (BBE) and Guilllain-Barré 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. © 1997 Elsevier Science B.V.

Atypical Miller Fisher syndrome with GQ1b antibodies

An atypical case of Miller Fisher syndrome is described in a patient with ophthalmoplegia and mild ataxia but no areflexia. High titres of acute phase antibodies to gangliosides asialo-GM1 and GQ1b were detected. Asialo-GM1 antibodies have not been previously reported in association with Miller Fisher syndrome. Considerable clinical recovery occurred in association with reduction in the ganglioside antibody titres. Ganglioside antibody assays may be helpful in the diagnosis of atypical cases of Miller Fisher syndrome. Detailed clinical, radiological and laboratory evaluation of suspected cases is warranted to improve our understanding of Miller Fisher syndrome. Such studies and the correlation with ganglioside antibody titres will also provide insights into the relationship between the classical and atypical cases of Miller Fisher syndrome, Guillain-Barré syndrome and Bickerstaff's brain stem encephalitis.

Successful plasmapheresis in Bickerstaff's brain stem encephalitis associated with anti-GQ1b antibody

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.

An immunologic abnormality common to Bickerstaff's brain stem encephalitis and Fisher's syndrome

The nosological position of Bickerstaff's brain stem encephalitis (BBE) has yet to be established, and its etiology is not clear. Because anti-G Q1b antibody frequently occurs in patients with Fisher's syndrome (FS) and there are clinical similarities between FS and BBE, we investigated anti-ganglioside antibodies in sera from 3 BBE patients who had transient long tract signs in addition to acute ophthalmoplegia and cerebellar-like ataxia in order to clarify the etiology and nosological position of BBE. High IgG anti-G Q1b antibody titers were present in all 3 sera samples but decreased with the clinical course of the illness. In contrast, no anti-G Q1b antibody was found in sera from patients with other neurologic diseases which were able to produce transient brain stem disturbance: multiple sclerosis, neuro-Behçet's disease, brain stem infarction, herpes simplex virus encephalitis, and Wernicke's encephalopathy. The finding that BBE and FS shared common autoantibody suggests that autoimmune mechanism common to FS is likely in BBE, and that both conditions represent a distinct disease with a wide spectrum of symptoms that include ophthalmoplegia and ataxia.

Bickerstaff's brainstem encephalitis associated with typhoid fever

A 14 year old boy developed the syndrome of Bickerstaff's brainstem encephalitis during the course of bacteriologically proved typhoid fever. The clinical course and the results of various neurological investigations are detailed. This report adds a further manifestation to the published neuropsychiatric complications of typhoid fever.