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Masaaki Odaka

Publications and source records attributed to Masaaki Odaka.

At least 19 recordsLinked to original sources
PubMed2006

Isolated abducens nerve palsy as a regional variant of Guillain-Barré syndrome.

The authors reviewed clinical profiles and laboratory findings for 100 cases of abducens nerve paresis without impairment of the other cranial nerves, limb weakness, and ataxia throughout the clinical course. Review of the medical records of 9300 patients referred to our neuoroimmunological laboratory for serum anti-ganglioside antibody testing. Information was obtained from each primary physician on symptoms of preceding infection; initial symptoms; neurological signs during the illness; the clinical course; treatment provided; and outcome. Isolated abducens nerve paresis was present in 100 patients and bilateral paresis in 29. Tentative diagnoses made by the primary physicians on request of anti-ganglioside antibody testing were abducens nerve palsy (n = 68), Fisher syndrome (n = 14), acute ophthalmoparesis without ataxia (n = 14). Symptoms of infection anteceded in 63. Tendon reflexes were absent or decreased in 27. Distal paresthesias were experienced by seven. Serum anti-GQ1b antibody was positive in 25. These findings suggest that some cases of isolated abducens nerve palsy can be categorized as a regional variant of Guillain-Barré syndrome or mild form of Fisher syndrome.

Abducens Nerve↗
PubMed2006

Chronic inflammatory demyelinating polyneuropathy: a treatment protocol proposal.

Guidelines for diagnostic criteria and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) have been proposed by a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society, based on available evidence and expert consensus. These should prove practical for the clinical management of CIDP. Intravenous immunoglobulin followed by corticosteroids should be considered as the initial treatment, however no clear second drug of choice for patients who do not respond to the initial treatment is given. The author reports the long-term therapeutic efficacy of ciclosporin for patients with CIDP who did not show sustained improvement under steroid therapy. Ciclosporin should be tried for patients with intractable CIDP who require repeated intravenous immunoglobulin. An adequate initial dose of ciclosporin is 3 mg/kg/day, with plasma trough concentrations between 100 and 150 ng/ml. If patients respond to ciclosporin, remission can be maintained for 2 years, after which the dose can be slowly reduced over 1 year. Eventual withdrawal should be considered. This review proposes a treatment strategy that includes long-term maintenance therapy for CIDP based on published clinical trials and the author's clinical experience. Current concepts concerning the clinical spectrum of CIDP and diagnostic approaches are also considered.

Clinical Protocols↗
PubMed2006

[A case of acute lymphocytic leukemia relapsed as meningoradiculoneuropathy after bone marrow transplantation].

We report a 21-year-old woman who had acute lymphocytic leukemia with a relapse in the peripheral nervous system after bone marrow transplantation. She developed gait disturbance and numbness of the lower limb extremities, with gradual worsening. Neurological examination detected paraparesis associated with areflexia and stocking-type paresthesia. An examination of the cerebro-spinal fluid detected leukemicells. T2-weighted MRI of the lumbar spine showed multiple hyperintense signal lesions at the bodies of vertebra, spinous processes, and pedicles. Based on the diagnosis of recurrent acute lymphocytic leukemia with tumor infiltration to the meninges (meningeal leukemia), she received chemotherapy, after which her neurological symptoms and signs gradually improved. We would like to emphasize that neurological examination is important to detect CNS relapse in a patient with leukemia, even in hematological complete remission.

Adult↗
PubMed2006

[Case of spinal thoracic-lumbar dural arteriovenous fistula with polyneuropathy type sensory impairment].

A 57-year-old man had progressive paresthesias ascending from both legs together with paraparesis. Distal paresthesias of the upper extremities developed earlier than segmental sensory impairments. At transfer to our hospital, a neurological examination detected bilateral lower limb weakness predominant in the distal part; severe glove and stocking paresthesias in addition to superficial sensory impairment below the Th8 level; and micturition problems. T2-weighted thoracolumbar MRI showed a hyperintense spinal cord lesion between Th5 and L1. At the L4 level, a spinal arteriogram showed enlarged and tortuous vessels extending from the lumbar artery which drains to the spinal vein. These findings led to the diagnosis of spinal dural arteriovenous fistula. Anatomical substrates for this sensory impairment may be produced by development from the side to forward parts of lesions in the outer circumference of the anterior funiculi, resulting in the dominating sensory impairment in the distal parts of the upper extremities. Physicians need to be aware of patients who have polyneuropathy-like sensory impairment without segmental sensory signs, and must consider the possibility of spinal dural arteriovenous fistula.

Central Nervous System Vascular Malformations↗
PubMed2005

Overexpression of GD1a ganglioside sensitizes motor nerve terminals to anti-GD1a antibody-mediated injury in a model of acute motor axonal neuropathy.

Anti-GD1a ganglioside antibodies (Abs) are the serological hallmark of the acute motor axonal form of the post-infectious paralysis, Guillain-Barre syndrome. Development of a disease model in mice has been impeded by the weak immunogenicity of gangliosides and the apparent resistance of GD1a-containing neural membranes to anti-GD1a antibody-mediated injury. Here we used mice with altered ganglioside biosynthesis to generate such a model at motor nerve terminals. First, we bypassed immunological tolerance by immunizing GD1a-deficient, beta-1,4-N-acetylgalactosaminyl transferase knock-out mice with GD1a ganglioside-mimicking antigens from Campylobacter jejuni and generated high-titer anti-GD1a antisera and complement fixing monoclonal Abs (mAbs). Next, we exposed ex vivo nerve-muscle preparations from GD1a-overexpressing, GD3 synthase knock-out mice to the anti-GD1a mAbs in the presence of a source of complement and investigated morphological and electrophysiological damage. Dense antibody and complement deposits were observed only over presynaptic motor axons, accompanied by severe ultrastructural damage and electrophysiological blockade of motor nerve terminal function. Perisynaptic Schwann cells and postsynaptic membranes were unaffected. In contrast, normal mice were not only unresponsive to immunization with GD1a but also resistant to neural injury during anti-GD1a Ab exposure, demonstrating the central role of membrane antigen density in modulating both immune tolerance to GD1a and axonal susceptibility to anti-GD1a Abmediated injury. Identical paralyzing effects were observed when testing mouse and human anti-GD1a-positive sera. These data indicate that anti-GD1a Abs arise via molecular mimicry and are likely to be clinically relevant in injuring peripheral nerve axonal membranes containing sufficiently high levels of GD1a.

Animals↗
PubMed2005

Characterisation of the immunoglobulin variable region gene usage encoding the murine anti-ganglioside antibody repertoire.

Neuropathogenic murine antibodies reactive with terminal disialylgalactose epitopes are innate and preferentially encoded by the VH7183.3b gene. Here we have studied antibodies reactive with internal galactose-linked disialosyl epitopes and the terminal trisaccharide of GT1b. Antibodies were of moderate affinity and unmutated. Anti-GD1b antibodies were often encoded by the VH10.2b heavy and gj38c light chain genes. Anti-GT1b antibodies with broader glycan binding patterns were encoded by VHQ52 and VHJ558 family genes. These data indicate that the discrete specificities of ganglioside-binding antibodies are dictated by particular patterns of V gene usage residing within the innate B cell repertoire.

Amino Acid Sequence↗
PubMed2005

Ganglioside mimicry as a cause of Guillain-Barré syndrome.

PURPOSE OF REVIEW: Campylobacter jejuni is the most frequent agent of antecedent infection in an axonal variant of Guillain-Barré syndrome, acute motor axonal neuropathy, and anti-GM1 or anti-GD1a IgG antibody is also associated with acute motor axonal neuropathy. Molecular mimicry has been found between human GM1 ganglioside and the lipo-oligosaccharide of C. jejuni isolated from an acute motor axonal neuropathy patient. Progress has been made in Guillain-Barré syndrome research, especially on acute motor axonal neuropathy subsequent to C. jejuni enteritis. RECENT FINDINGS: Sensitization of rabbits with C. jejuni lipo-oligosaccharide, as well as GM1, induced the production of anti-GM1 IgG antibody, and the subsequent development of acute flaccid paralysis. Pathological changes in rabbit peripheral nerves were identical to those seen in human acute motor axonal neuropathy. These findings provide conclusive evidence that molecular mimicry is a cause of human autoimmune disease. Ganglioside-like lipo-oligosaccharide is synthesized by sialyltransferase Cst-II, N-acetylgalactosaminyl-transferase CgtA, and galactosyltransferase CgtB. There is a strong association between the simultaneous presence of these genes and Guillain-Barré syndrome-associated C. jejuni strains. Knockout mutants of C. jejuni genes involved in lipo-oligosaccharide sialylation had reduced reactivity with anti-GM1 sera from Guillain-Barré syndrome patients, and did not induce an anti-GD1a IgG antibody response in mice. Lipo-oligosaccharide biosynthesis genes appear to be essential for the induction of anti-GM1 or anti-GD1a IgG antibody and the subsequent development of acute motor axonal neuropathy. SUMMARY: The concept that carbohydrate mimicry causes autoimmune disease provides a clue to the resolution of the pathogenesis of other immune-mediated diseases.

Animals↗
PubMed2005

[Clinical features and treatment of Fisher syndrome].

To clarify the clinical features of patients with Fisher syndrome, we reviewed detailed clinical profiles and laboratory findings in 267 cases. The men:women ratio was about 3:2, median age at onset 42 years, and the two peaks were 30-39 and 50-59. Sixty two percent of the patients had an antecedent illness with upper respiratory infectious symptoms. As initial symptoms, 63% had diplopia, 33% ataxic gait, and 17% dysesthesia of the upper and/or lower limbs. Besides ophthalmoplegia, ataxia, and areflexia, blepharoptosis (42%), internal ophthalmoplegia (35%), facial palsy (25%), and bulbar palsy (16%) were present. Limb weakness was significant in 25% and sensory impairment in 52%. CSF albuminocytological dissociation was present in 80% after the second week. Most patients were given intravenous immunoglobulin (59%). None died, and almost all had a monophasic remitting course and a generally good outcome.

Adult↗
PubMed2004

Carbohydrate mimicry between human ganglioside GM1 and Campylobacter jejuni lipooligosaccharide causes Guillain-Barre syndrome.

Molecular mimicry between microbial and self-components is postulated as the mechanism that accounts for the antigen and tissue specificity of immune responses in postinfectious autoimmune diseases. Little direct evidence exists, and research in this area has focused principally on T cell-mediated, antipeptide responses, rather than on humoral responses to carbohydrate structures. Guillain-Barré syndrome, the most frequent cause of acute neuromuscular paralysis, occurs 1-2 wk after various infections, in particular, Campylobacter jejuni enteritis. Carbohydrate mimicry [Galbeta1-3GalNAcbeta1-4(NeuAcalpha2-3)Galbeta1-] between the bacterial lipooligosaccharide and human GM1 ganglioside is seen as having relevance to the pathogenesis of Guillain-Barré syndrome, and conclusive evidence is reported here. On sensitization with C. jejuni lipooligosaccharide, rabbits developed anti-GM1 IgG antibody and flaccid limb weakness. Paralyzed rabbits had pathological changes in their peripheral nerves identical with those present in Guillain-Barré syndrome. Immunization of mice with the lipooligosaccharide generated a mAb that reacted with GM1 and bound to human peripheral nerves. The mAb and anti-GM1 IgG from patients with Guillain-Barré syndrome did not induce paralysis but blocked muscle action potentials in a muscle-spinal cord coculture, indicating that anti-GM1 antibody can cause muscle weakness. These findings show that carbohydrate mimicry is an important cause of autoimmune neuropathy.

Animals↗
PubMed2004

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.

Adolescent↗
PubMed2004

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 theta 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.

Ataxia↗
PubMed2004

Ataxic Guillain-Barré syndrome associated with anti-GM1b and anti-GalNAc-GD1a antibodies.

Ataxic Guillain-Barré syndrome (GBS) associated with anti-GQ1b IgG antibody has been reported. We, however, have had a patient with ataxic GBS who had IgG antibodies to the minor gangliosides GM1b and GalNAc-GD1a, and we therefore retrospectively investigated the clinical features of patients who had antibodies to GM1b or GalNAc-GD1a, but not to GQ1b. Information on patients' antecedent illnesses, initial symptoms, neurological signs, and CSF findings was reviewed in those with ataxic GBS or Fisher syndrome (FS) with anti-GM1b or anti-GalNAc-GD1a IgG antibodies. We tested whether the anti-GM1b and anti-GalNAc-GD1a antibodies are cross-reactive and constructed three-dimensional structural models of GM1b and GalNAc-GD1a. Ataxic GBS was diagnosed in 1 of 65 patients who had both anti-GM1b and anti-GalNAc-GD1a antibodies and in 3 of 159 patients who had anti-GM1b antibody without anti-GalNAc-GD1a antibody: FS was diagnosed in 1 of the 159 patients and in 1 of 35 who had anti-GalNAc-GD1a antibody without anti-GM1b antibody. All the patients' antibodies to GM1b or GalNAc-GD1a were associated with the IgG isotype. The clinical features of patients with ataxic GBS associated with anti-GM1b or anti-GalNAc-GD1a IgG antibodies did not differ from those of patients who had anti-GQ1b IgG antibody. Absorption study findings for serum from the patient who had both anti-GM1b and anti-GalNAc-GD1a IgG antibodies showed significant absorbance of anti-GM1b IgG antibody by GalNAc-GD1a and of anti-GalNAc-GD1a IgG antibody by GM1b, indicating that these antibodies are cross-reactive. This is the first report of ataxic GBS or FS associated with anti-GM1b or anti-GalNAc-GD1a IgG antibodies. These autoantibodies, as well as anti-GQ1b IgG antibody, may function in the development of some patients with ataxic GBS and FS.

Adult↗
PubMed2004

[Clinical features of neuropathies in a group of patients associated with anti-GalNAc-GD1a antibody].

To clarify the clinical features of patients with anti-GalNAc-GD1a antibody, we retrospectively investigated which conditions were associated with anti-GalNAc-GD1a antibody in a large number of patients. Sixty-four out of 1,713 patients had anti-GalNAc-GD1a IgG antibody. Fifty-seven (89%) were diagnosed with Guillain-Barré syndrome (GBS) or atypical GBS with preserved deep tendon reflexes. These patients were characterized by young man, antecedent diarrhea, distal-dominant limb weakness, and absence of cranial nerve impairment and sensory disturbance. Some of sera from patients had anti-GM1, anti-GM1b, and anti-GD1a IgG antibodies associated with axonal GBS. Our study suggests that the anti-GalNAc-GD1a antibody testing is useful for supporting the diagnosis of GBS. In addition, fine specificity of the antibody also was clarified.

Autoantibodies↗
PubMed2004

[Details about the first medical examinations of patients with Guillain-Barré or Fisher syndromes].

Randomized controlled trials have shown beneficial responses to plasma exchange and intravenous immunoglobulin in Guillain-Barré syndrome (GBS), but details about the first medical examination of patients with GBS and Fisher syndrome (FS) have not been reported. We investigated the period from first consultations to treatment after the onset of patients with GBS and FS. A questionnaire was used to collect informations on 247 patients with GBS and 125 with FS, all of whom had been referred to our neuroimmunological laboratory informations between January 2001 and October 2001 for serum anti-ganglioside antibody tests. A letter of inquiry was sent to the primary physicians; 150 (61%) patients with GBS and 72 (58%) with FS responded validly. Patients with GBS generally consulted an internist [58%, neurologist (21%)] or orthopedist (17%), those with FS an internist [52%, neurologist (26%)] or ophthalmologist (25%). Consultation with a neurologist usually came after one with a physician. We conclude that physicians should be aware that patients may have GBS or FS, and that such recognition is useful for early diagnosis and treatment of these syndromes.

Guillain-Barre Syndrome↗
PubMed2004

[Anterior spinal artery syndrome due to cervical spondylosis presenting as cervical angina].

A 54-year-old woman developed acute progressive paraparesis after repeated precordial pain. Neurological examination revealed bilateral four-limb weakness predominant in the distal part of the upper limbs, upper limbs brisk tendon reflexes, superficial sensory impairment below the C8 level, and atonic bladder. T2-weighted cervical MRI disclosed hyperintense lesion with disc herniation in gray matter of spinal cord between C5 and C7. No vertebral artery abnormalities were detected. We hypothesized that she developed anterior spinal artery syndrome after cervical angina caused by cervical spondylosis. We conclude that physicians need to be aware of patients who experience chest pain without evidence of cardiac disease and that they take into consideration spinal cord infarction.

Angina Pectoris↗
PubMed2003

Axonal Guillain-Barré syndrome associated with axonal Charcot-Marie-Tooth disease.

We report the first case of axonal Guillain-Barré syndrome (GBS) associated with axonal Charcot-Marie-Tooth disease (CMT). A 30-year-old Japanese man, who had suffered leg atrophy and foot deformity since childhood, developed acute weakness in his four limbs following an upper respiratory tract infection. Nerve conduction studies showed low compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) amplitudes in all the nerves tested. Serial studies showed a rapid increase in CMAP amplitude, but no significant change in SNAP, which indicates that the acute event selectively involved motor axons and was superimposed on a baseline motor-sensory axonal neuropathy, probably CMT Type 2. Elevated serum IgG antibodies against GM1 and GM1b, an increase in CSF protein, and rapid clinical and electrophysiological recovery after plasma exchange support the diagnosis of a pure motor axonal form of GBS, acute motor axonal neuropathy. The association may be coincidental, but a particular susceptibility to axonal damage of CMT2 cannot be excluded.

Adult↗