Cerebral white matter lesions in acute motor axonal neuropathy.
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Biomedical subjects
Publications and source records attributed to N Yuki.
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OBJECTIVE: To determine whether the anti-GM1 antibody IgG subclass (IgG1 to 4) is associated with clinical profiles and patterns of recovery in Guillain-Barré syndrome (GBS). METHODS: The IgG subclassification of anti-GM1 antibody was examined and compared with clinical data on 42 GBS patients positive for the antibody. RESULTS: Frequent anti-GM1 antibody subclasses were IgG1 (76%) and IgG3 (31%). IgG1 antibody was associated with preceding gastroenteritis and Campylobacter jejuni serology, whereas IgG3 antibody was associated with preceding respiratory infection. Although the severity at nadir was similar for IgG1- and IgG3-positive patients, the percentage of patients who could not walk independently was greater for the IgG1-positive group 1 month (42 vs 0%; p = 0.02), 3 months (28 vs 0%), and 6 months (25 vs 0%) after onset. Rapid recovery within 1 month occurred frequently in the patients with the IgG3 antibody but rarely in those with the IgG1 antibody (67 vs 11%; p = 0.003). CONCLUSIONS: The IgG1 subclass of anti-GM1 antibody is a major subtype indicative of slow recovery, whereas isolated elevation of IgG3 subclass antibody titer suggests rapid recovery. Variation in subclass patterns may depend on which pathogen precipitates GBS.
We evaluated the effects of Bifidobacterium breve-fermented soymilk on probiotic function. An administered strain of B. breve strain Yakult was capable of growing in soymilk with no additives as high as 10(9) CFU/ml. During storage of the fermented soymilk at 10 degrees C for 20 days, viable counts of the strain did not change. The growth inhibition of the strain in a bile-containing medium was lessened by the addition of soy protein. In human feeding experiments, the administered B. breve was recovered at a level of over 10(9) CFU/g faeces, accompanied by an increase in the total number of bifidobacteria. These results indicate that fermented soymilk with B. breve strain Yakult could be a novel type of probiotic food.
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We report a patient with severe sensory ataxia, areflexia, and ophthalmoplegia with preservation of limb muscle strength. Electrophysiological examinations revealed peripheral sensory nerve involvement. A serological examination showed the elevation of IgG antibodies to various b-series gangliosides as well as GT1a. These indicated that this case is an overlap between acute sensory ataxic neuropathy and Miller Fisher syndrome. Autoantibody is implicated as potential pathogenic agents in some cases of acute sensory ataxic neuropathy.
We report a case of markedly asymmetric pharyngeal-cervical-brachial weakness. Acute progression of symptoms, albuminocytologic dissociation in cerebrospinal fluid, electrophysiologic evidence of demyelination and elevation of IgG anti-GT1 a antibody titer paralleled the clinical course, support the diagnosis of Guillain-Barré syndrome. Guillain-Barré syndrome should be considered in the differential diagnosis of cranial neuropathy, even in cases where there is marked asymmetry.
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To demonstrate the beneficial effects of synbiotic therapy (combined use of probiotics and prebiotics) in critically ill patients, we designed a new protocol for such therapy. Three agents were used as synbiotics: Bifidobacterium breve, Lactobacillus casei, and galactooligosaccharides. More than 1 x 10(9) of probiotic bacteria were contained in each 1.0 g pack. We administered 3.0 g per day of each agent to the patient.A critically ill 9-month-old girl with laryngotracho-esophageal cleft (type IV) was treated by our new synbiotic therapy. Abundant amounts of synbiotic bacteria were detected in the feces which suggests that these administered bacteria affected intestinal function in situ. Bowel movements resumed soon after the commencement of synbiotic therapy and considerable amounts of short chain fatty acids were detected in the feces. Growth of the patient was satisfactory under this treatment. Our new synbiotic therapy had a beneficial effect to improve intestinal function. We recommend synbiotic therapy for critically ill patients in intensive care units as an important immunonutritional therapy.
OBJECTIVE: To investigate the presence of serum anti-GT1a IgG in Guillain-Barré syndrome (GBS) and its relation to clinical manifestations. BACKGROUND: Several patients with GBS and bulbar palsy have been reported to have serum anti-GT1a IgG. Most, however, also have anti-GQ1b IgG. A previous study failed to detect GT1a in human cranial nerves, but GQ1b was abundant in human ocular motor nerves. Whether anti-GT1a IgG itself determines the clinical manifestations is not yet clear. METHODS: The association of clinical manifestations with the presence of anti-GT1a IgG and with its cross reactivity was investigated. An immunochemical study was performed to determine whether GT1a is present in human cranial nerves. RESULTS: Anti-GT1a and anti-GQ1b IgG were positive in 10% and 9% respectively of 220 consecutive patients with GBS. Patients with anti-GT1a IgG often had cranial nerve palsy (ophthalmoparesis, 57%; facial palsy, 57%; bulbar palsy, 70%), and 39% needed artificial ventilation. These features were also seen in patients with anti-GQ1b IgG. There was no significant difference between the two groups with respect to the frequency of clinical findings. An enzyme-linked immunosorbent assay showed that anti-GT1a IgG cross reacted with GQ1b in 75% of the patients, GD1a in 30%, GM1 in 20%, and GD1b in 20%. All five patients who carried anti-GT1a IgG that did not cross react with GQ1b had bulbar palsy, neck weakness, absence of sensory disturbance, and positive Campylobacter jejuni serology. Thin-layer chromatography with immunostaining showed that GT1a is present in human oculomotor and lower cranial nerves. CONCLUSIONS: These findings provide further evidence that anti-GT1a IgG itself can determine clinical manifestations. The distinctive clinical features of patients with anti-GT1a IgG without anti-GQ1b activity distinguish a specific subgroup within GBS.
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Anti-ganglioside antibodies are consistently found in Guillain-Barré syndrome (GBS) patients from different geographical parts of the world. Several studies indicated differences in relative frequencies of anti-ganglioside reactivity and isotype distribution between GBS patients from Asia and from Europe. We investigated antibody reactivity against the gangliosides GM1, GM1b and GalNAc-GD1a in GBS patients from Japan and The Netherlands in two different laboratories. The proportion of GBS patients with anti-ganglioside antibodies did not differ between the two countries. GBS patients from The Netherlands more frequently had cross-reacting anti-GalNAc-GD1a/anti-GM1b antibodies and a stronger IgM anti-ganglioside response. Our findings indicate that geographical determined factors, dependent on either the host or the triggering infectious agent, determine the isotype distribution and fine specificity of anti-ganglioside antibodies in GBS patients.
The authors describe two patients with acute sensory ataxic neuropathy. Both had a profound loss of proprioception and generalized areflexia. High titers of monospecific anti-GD1b IgG antibody were detected in their sera during the acute phase. Sensory ataxia resolved within 2 weeks after the onset. Taken together with the induction of experimental sensory ataxic neuropathy sensitized with GD1b ganglioside, GD1b may be a target molecule for autoantibody in some patients with acute sensory ataxic neuropathy.
OBJECTIVE: To examine the association between Miller Fisher syndrome (MFS) and antecedent Haemophilus influenzae infection. BACKGROUND: Little is known about agents in prior respiratory tract infection of MFS, whereas antecedent upper respiratory symptoms are frequent. H. influenzae is a major pathogen that can cause human respiratory tract infection. METHODS: The authors used ELISA to detect serum antibody against the bacterium in 70 consecutive patients with MFS and 110 with Guillain-Barré syndrome (GBS). RESULTS: Serum anti-H. influenzae IgG and IgM antibody activities were significantly higher in the MFS group than in age- and sex-matched patients with other neurologic diseases (n = 62) and normal control subjects (n = 82). The GBS group showed no significant increase in any class of antibody activities compared with control groups. Serologic evidence of recent infection was found in five (7%) of the patients with MFS and two (2%) of 110 patients with GBS, all of whom had a history of antecedent respiratory tract infection. They frequently showed ophthalmoplegia, but other neurologic features were not remarkable. Serum anti-GQ1b IgG antibody that had cross-reactivity with GT1a ganglioside was detected in six of these seven patients. Thin-layer chromatography with immunostaining showed that serum IgG from H. influenzae-seropositive patients with high anti-GQ1b and anti-GT1a IgG antibody titers bound to the lipopolysaccharide fraction extracted from the type b H. influenzae serostrain. These bands were also stained by anti-GT1a monoclonal antibody (GMR11), indicating that the lipopolysaccharide bears the GT1a epitope. CONCLUSIONS: These findings point to H. influenzae being an agent associated with MFS. Epitopic overlap between H. influenzae and human nerve tissue may be involved in the development of MFS much as GBS is associated with Campylobacter jejuni enteritis.
The authors reviewed the clinical features and outcome of Miller Fisher syndrome (MFS) for 50 consecutive patients with MFS including 28 patients who received no immunotherapy. Besides the characteristic clinical triad (ophthalmoplegia, ataxia, and areflexia), pupillary abnormalities, blepharoptosis, and facial palsy are frequent in MFS, whereas sensory loss is unusual despite the presence of profound ataxia. Patients with MFS usually had good recovery and no residual deficits.
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.
Some reports have called sensory ataxic neuropathy (SAN) associated with IgM antibody against b-series gangliosides a chronic form of Miller Fisher syndrome (MFS), but this has yet to be established. We examined five patients with SAN and eight patients with IgG anti-GQ1b-positive MFS. Only one patient with SAN complained of diplopia, whose ocular movement was not limited. The other four patients had neither diplopia nor limitation of ocular movement. All the SAN patients had severe deep sense impairment, whereas one patient with MFS showed only mild vibratory sense impairment. All sera from the SAN patients had remarkably high IgM antibody titers to the b-series gangliosides GD3, GD2, GD1b, GT1b, GQ1b, GQ1b alpha, fucosyl-GD1b, and alpha galactosyl [alpha fucosyl] GD1b. An absorption study confirmed that the anti-GQ1b antibodies cross-reacted with GD3, GD2, GD1b, and GT1b. In contrast, only two samples from the MFS patients had IgG antibody to GD3, and no sample reacted with GD2, GD1b, or GT1b. SAN has different clinical or serological features from MFS, and therefore is not a chronic form of it.