Bickerstaff's brainstem encephalitis associated with cytomegalovirus infection.
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Adolescent↗
Biomedical subjects
N Yuki
Publications and source records attributed to N Yuki.
Longitudinal study of serum and cerebrospinal fluid (CSF) class-specific antibodies against Campylobacter jejuni and GM1 ganglioside in Guillain-Barré syndrome.
We report two Guillain-Barré Syndrome (GBS) patients with culture-proven Campylobacter jejuni (Penner19/Lior 7). Elevated anti-C.jejuni IgA antibody declined to the normal range within one month, elevation of anti-C.jejuni IgM antibody lasted for 3 months and the titers of anti-C.jejuni IgG antibody were still elevated at one year after the neurologic onset. Serological criteria for antecedent C.jejuni infection in GBS patients should require 1) high titer of serum IgA and/or IgM antibody against C.jejuni followed by seroconversion of the IgA and/or IgM and 2) a 4-fold decline of IgG serum dilution against C.jejuni. Anti-C.jejuni antibodies in cerebrospinal fluid (CSF) reached the maximum value at one month after their neurologic onset which may not be responsible for the main pathological mechanism in the development of GBS. Anti-GM1 antibodies in serum and CSF were parallel with those of anti-C.jejuni antibodies during the course of the disease.
Adult↗
[Campylobacter jejuni and autoimmune-mediated neurologic diseases: pathogenesis of Guillain-Barré syndrome and Fisher's syndrome].
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Animals↗
The significance of immunoglobulin M antibody response to hepatitis C virus core protein in patients with chronic hepatitis C.
The significance of immunoglobulin (Ig) M antibody to hepatitis C virus core protein (IgM anti-HCV core) was studied in 41 patients with chronic hepatitis C virus (HCV) infection diagnosed by the polymerase chain reaction (PCR). IgM anti-HCVcore was tested with a solid-phase enzyme-linked immunoassay. The results were correlated with clinical features, liver histology findings evaluated by the histological activity index, and virological features such as genotypes and viremic levels assessed by a branched DNA assay. IgM anti-HCVcore was found in 29 (71%) patients, and its occurrence was only related to viremic levels. A significant relationship was observed between viremic levels and IgM anti-HCVcore cut-off index (rs = .42, P < .01). Of the eight low viremic (branched DNA-negative) patients, only two (25%) tested positive for IgM anti-HCVcore with a low cut-off index of < 3, whereas 27 (82%) of the 33 highly viremic (branched DNA-positive) patients had IgM anti-HCVcore (P < .01). After a 28-week interferon-alpha course (IFN-alpha), sustained aminotransferase normalization after therapy withdrawal was achieved by only two (13%) of the 16 patients with IgM anti-HCVcore cut-off index > 3 compared with 11 (44%) of the 25 patients with that < 3 (P < .05). IgM anti-HCVcore cut-off index decreased after therapy in patients who cleared the virus in sera but increased again after reappearance of viremia. These findings suggest that IgM anti-HCVcore may serve as a simple serological indicator of active virus replication and have relevance to the outcome of antiviral therapy.
Adult↗
[Fisher's syndrome following Campylobacter jejuni enteritis--a case report and review of the literature].
A 13-year-old boy presented with ophthalmoplegia, cerebella ataxia and areflexia subsequent to watery diarrhea and pyrexia. Campylobacter jejuni (PEN 2: LIO 4) was isolated from the stools. The patient had a high titer of IgG anti-GQ1b IgG antibody titers which decreased during the clinical course. Although C. jejuni has recently been recognized as the most common microorganism causing infection preceding Guillain-Barré syndrome, only six patients with Fisher's syndrome after this bacterial infection have been reported. This is the first report of a patient with Fisher's syndrome in whom C. jejuni was identified as a causative organism in Japan.
Adolescent↗
[Guillain-Barré syndrome associated with anti-GalNAc-GD1a antibody subsequent to Campylobacter jejuni enteritis].
We report a 15-year-old man with Guillain-Barré syndrome (GBS) following Campylobacter jejuni enteritis. Neurologic examination revealed distal muscle weakness and mild disturbance of superficial sense on right plantar area. Serial electrophysiologic studies indicated that the predominant process was axonal degeneration of motor nerves. Thin-layer chromatography with immunostaining revealed that serum IgG strongly reacted with GalNAc-GD1a and weakly with GM2, but did not react with GM1. This is the first report of GBS subsequent to C. jejuni enteritis associated with anti-GalNAc-GD1a antibody.
Adolescent↗
[Guillain-Barré syndrome associated with external ophthalmoplegia, consciousness disturbance, and extensor plantar responses: an overlap between Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis].
A 14-year-old girl developed diplopia and ataxic gait following upper respiratory tract infection. On day 3, she became lethergic, but was easily roused on stimulation. Neurologic examinations showed external ophthalmoplegia, tetraplegia, tendon areflexia, and extensor plantar responses. CSF protein was 93 mg/dl with normal cellurality on day 25. Serum in the acute phase of her illness had autoantibodies to gangliosides GQ1b and Ga1NAc-GD1a. An EEG showed bilateral theta activity. Results of ABR, SEP and blink reflexes suggested the presence of intramedullary brainstem lesion. She received plasmapheresis on days 4, 5 and 6. Neurological signs immediately improved. Five months after onset of neurological symptoms, she had no neurological abnormalities except for tendon areflexia. Electrophysiologic studies returned to normal concurrently with her clinical improvement. This patient demonstrates an overlap between Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis.
Adolescent↗
[Immunoadsorption therapy on Fisher's syndrome--removal ability of anti-ganglioside antibodies by tryptophan-linked immunoadsorbent].
There have been several reports describing that immunoadsorption therapy improves the neurologic involvement in Fisher's syndrome (FS). However, few studies have assessed the usefulness of immunoadsorption therapy in view of the removal ability of anti-GQ(1b) antibody, which may function the development of FS. We examined the ability of immunoadsorbents for the anti-GQ(1b) antibody in a patient with FS. A 28-year-old woman developed diplopia and giddiness following a cough, fever and diarrhea. On admission (day 22), neurologic examination showed bilateral moderate oculomotor paralysis and bilateral complete abducens paralysis. She had areflexia, numbness of middle and ring fingers on the left and mild ataxic gait. Her serum had IgG anti-GQ(1b) and anti-GD(1b) antibodies. We examined the absorption of anti-ganglioside antibodies onto a polyvinyl alcohol gel (PVA), a phenylalanine-linked PVA (PH-350) and a tryptophan-linked PVA (TR-350) by the batchwise adsorption method. TR-350 absorbed the autoantibodies, but the removal ability of autoantibody by PVA and PH-350 was not proved. The FS patient was treated with TR-350 (days 29, 34 and 43) and PH-350 (day 39). Anti-GQ(1b) and anti-GD(1b) antibodies were significantly removed by the TR-350, in accordance with the results of the in vivo study. There was little loss of albumin as compared with the immunoglobulins and complements. The numbness and ataxia disappeared on day 44. The diplopia disappeared on day 106. TR-350 would be better than PH-350 in the treatment of FS by immunoadsorption therapy.
Adult↗
[Axonal Guillain-Barré syndrome associated with anti-GalNAc-GD1a antibody subsequent to Campylobacter jejuni (PEN 43) enteritis].
We reported a 16-year-old boy who had Guillain-Barré syndrome (GBS) after suffering diarrhea. Campylobacter jejuni was isolated from his stool, and the serotype belonged to PEN 43. Neurologic examination revealed distal-dominant muscle weakness atrophy, and mild sensory disturbance. Motor and sensory nerve conduction velocities were normal, but compound muscle action potentials were markedly reduced. Serum from the patient had high titers of anti-FalNAc-GD1a antibodies. He had HLA-A24, B51, DRB1*04 and DRB1*09. His elder sister showed diarrhea and serum anti-C. jejuni antibody, but did not showed GBS and serum anti-ganglioside antibody. Her HLA types were A24, B51, DRB1*09 and DRB1*14.
Adolescent↗
[Pathogenesis of Guillain-Barré syndrome and Fisher's syndrome: molecular mimicry between antecedent infectious agents and components of nerve tissues].
Sera from patients with Guillain-Barré syndrome (GBS) following Campylobacter jejuni infection have autoantibody to GM1 ganglioside. We have investigated whether GM1-oligosaccharide structure is present in the lipopolysaccharide (LPS) of C. jejuni (PEN 19) that was isolated from a GBS patient. The LPS showing the binding activity of cholera toxin, which specifically recognizes the GM1-oligosaccharide, was purified by a column chromatography. Proton NMR methods have revealed that the LPS has the oligosaccharide structure [Gal beta 1-3GalNAc beta 1-4 (NeuAc alpha 2-3) Gal beta 1-], which is identical to the terminal tetrasaccharide of GM1 ganglioside. Fisher's syndrome (FS) is very frequently associated with anti-GQ1b ganglioside antibody. We isolated C. jejuni from 2 patients with FS subsequent to enteritis. Crude LPS fractions were extracted from the bacteria and separated by thin-layer chromatography. Monoclonal antibodies to GQ1b ganglioside (GMR 13 and 7F5) reacted with both LPS fractions, indicating that the LPSs bear GQ1b epitope. These findings strongly support that "molecular mimicry" between infectious agents and components of nervous system function in the development of post-infection disorders.
Autoantibodies↗
Fisher's syndrome, anti-GQ1b antibody, and HLA-B39 antigen.
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Autoantibodies↗
Molecular mimicry between GQ1b ganglioside and lipopolysaccharides of Campylobacter jejuni isolated from patients with Fisher's syndrome.
We isolated Campylobacter jejuni from 2 patients with Fisher's syndrome subsequent to enteritis. Crude lipopolysaccharide fractions were extracted from the bacteria and separated by thin-layer chromatography. Monoclonal antibodies to GQ1b ganglioside (GMR13 and 7F5) reacted with both lipopolysaccharide fractions, indicating that the lipopolysaccharides bear the GQ1b epitope. This is the first report of molecular mimicry between neural tissue components and the antecedent infectious agents of Fisher's syndrome.
Antibodies, Monoclonal↗
Pathogenesis of axonal Guillain-Barré syndrome: hypothesis.
Pathologic studies of acute motor axonal neuropathy show strong evidence of the presence of primary axonal Guillain-Barré syndrome (GBS). The pathogenesis of axonal GBS is speculated to be as follows: (1) Infection by an organism induces the high production of a cross-reactive antibody between an infectious agent and the motor nerve axon in patients with a particular immunogenetic background. (2) The antineural antibody binds to the motor nerve terminals, thereby inhibiting motoneuron excitability and causing muscular weakness. (3) Binding of the antineural antibody, or subsequent functional impairment of the motoneurons, causes the motor axon to degenerate from the terminals. (4) In severe cases, extensive axonal loss and central chromatolysis of the motoneurons occur. These inhibit recovery and lead to a poor functional prognosis.
Animals↗
Motor neuron disease with multi-system involvement presenting as tetraparesis, ophthalmoplegia and sensori-autonomic dysfunction.
We carried out a postmortem examination on two Japanese patients, 64- and 80-year-old men whose survival was prolonged with an artificial respirator. They had no family history of neuropsychiatric disorders and were suspected, clinically, as having a motor neuron disease that differed from amyotrophic lateral sclerosis (ALS). As well as upper and lower motor neuron impairment, they showed a variety of symptoms, such as sensory disturbances, hypohidrosis, impotence, ophthalmoparesis and/or atonic neurogenic bladder, and their protein content in cerebrospinal fluid was elevated markedly. Pathological examination revealed the following extensive nervous system involvement: (1) the upper and lower voluntary motor systems, including the IIIrd, IVth and VIth cranial nerve nuclei: (2) the reticular formation and its major afferent pathways; (3) the vestibulospinal and tectospinal systems; (4) the spinocerebellar system and the exteroceptive somatic afferent pathways; (5) the dentatorubral and pallidoluysian systems; and (6) the substantia nigra, locus ceruleus and intermediolateral and Onufrowicz's nuclei. Neither Bunina bodies, Lewy body-like hyaline inclusions nor ubiquitin immunoreactive skein-like structures were observed. The distribution of the lesions was quite different from that in patients with ALS and the other known related diseases. Recently, seven autopsied cases with clinical and histopathological similarities to our patients have been reported in Japan. Our conclusion is that our two and these seven patients should be classified as having a new motor neuron disease entity, which can be is differentiated from ALS.
Aged↗
Guillain-Barré syndrome associated with IgM anti-GM1 antibody following Campylobacter jejuni enteritis.
We report a 4-year-old girl diagnosed as having Guillain-Barré syndrome after infection by Penner serotype 19 of Campylobacter jejuni. The patient had the HLA-B35 antigen. Neurological examination revealed distal-dominant weakness and intact sensation. Serial electrophysiological studies indicated that the predominant process was axonal degeneration involving motor nerves. An enzyme-linked immunosorbent assay revealed the presence of high titres of serum IgM antibodies to gangliosides GM1 and GM2. The IgM auto-antibody titres decreased concurrently with the clinical course of the illness and no switching from IgM to IgG secretion took place.
Antibodies, Anti-Idiotypic↗
Expression of a blood group B antigen-related glycoepitope in human dorsal root ganglion cells.
Carbohydrate epitopes of glycoconjugates are expressed on sensory neurons of dorsal root ganglion (DRG). A possible role of antibodies directed at carbohydrate determinants of the glycoconjugates has been suggested in some patients with sensory neuropathy. We investigated expression of blood group antigen-related epitopes in human DRG immunohistochemically using monoclonal antibodies to A, B, and H antigens. A blood group B determinant [Gal alpha 1-3(Fuc alpha 1-2)Gal beta-]-related glycoepitope was demonstrated in the neurons and surrounding satellite cells of DRG obtained from subjects with any ABO blood group phenotype. The treatment with trypsin or chloroform/methanol prior to the immunostaining suggested that the glycoconjugate exhibiting the blood group B determinant-related epitope consisted mainly of glycoprotein and included glycolipid. The glycoconjugates with the blood group B determinant-related epitope may play a role in the physiological function and pathophysiology of human DRG neurons.
ABO Blood-Group System↗
Hepatitis C virus replication and antibodies to structural and nonstructural viral proteins in chronic hepatitis C.
The correlation between hepatitis C virus replication and antibodies to both structural (core) and nonstructural (C100-3) hepatitis C virus proteins (anti-HCVcore and anti-C100-3, respectively) was assessed. The concentration of serum hepatitis C virus RNA was determined by a competitive reverse transcription-polymerase chain reaction assay, and antibody titers were determined by endpoint dilution. No correlation was found between viremic levels and antibody titers in 42 chronic hepatitis C patients. At the end of a 6-month course of interferon-alpha therapy, 18 patients became negative for hepatitis C virus RNA. In the other 24 patients, post-treatment viremic levels ranged from 10(-6.5)-10(0.5) of pretreatment levels. Both anti-C100-3 and anti-HCVcore frequently decreased in patients whose viremic levels dropped to the negative range or to < 10(-2) of pretreatment levels. Anti-C100-3 decreased in all such cases (25/25), while anti-HCVcore decreased in 18/25 (72%) (p < 0.01), indicating that anti-C100-3 is more likely to decrease following suppression of viral replication than anti-HCVcore. These data suggest that hepatitis C virus antibodies may serve as a marker of suppression of viremia following interferon therapy even in patients who do not clear the virus.
Adult↗
Hepatitis C virus antibody and hepatitis C virus replication in chronic hepatitis B patients.
We assessed hepatitis C virus infection in 156 chronic hepatitis B patients using second-generation hepatitis C virus antibody (anti-HCV). Active virus replication was further investigated in anti-HCV-positive cases by means of polymerase chain reaction assay for the detection of serum hepatitis C virus RNA. Anti-HCV prevalence was higher in patients negative for hepatitis B e antigen (HBeAg) (10/48, 21%) than in HBeAg-positive patients (10/108, 9%) (p < 0.05), and the reactivity (cut-off index) in anti-HCV enzyme-linked immunosorbent assay of the positive cases was significantly higher in HBeAg-negative patients (4.1 +/- 0.1) than in -positive ones (3.6 +/- 0.6) (p < 0.05). The prevalence of hepatitis C virus RNA in anti-HCV-positive cases was also higher in the HBeAg-negative group (9/10, 90%) than in the -positive group (3/10, 30%) (p < 0.01). Viremia was found in association with high reactivity in anti-HCV ELISA (cut-off index > 3.5) in both groups. Nine (90%) of 10 such cases were viremic in the HBeAg-negative group compared with three (43%) of seven in the HBeAg-positive group (p < 0.05). These results suggest that hepatitis C virus replication may be influenced by hepatitis B virus replicative states, indicating possible interference between hepatitis B and C viruses.
Adult↗