Raymond syndrome (alternating abducent hemiplegia) caused by a small haematoma at the medial pontomedullary junction.
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Adult↗
Biomedical subjects
J Kira
Publications and source records attributed to J Kira.
Chronic hypertrophic cranial pachymeningitis associated with HTLV-I infection.
Two patients presenting with recurrent multiple cranial neuropathy showed diffuse thickening and gadolinium enhancement of the dura mater on brain MRI. Both had anti-HTLV-I antibodies in serum. A quantitative polymerase chain reaction study of the peripheral blood disclosed that the HTLV-I proviral DNA loads increased considerably in one case and moderately in the other. Both showed a spontaneous proliferation of peripheral blood lymphocytes as well as an increase in helper/inducer T cells. Neither had any other underlying infections or autoimmune diseases. Thus it is possible that hypertrophic pachymeningitis developed as a result of multiorgan involvement of HTLV-I infection in these patients.
Adult↗
[Segmental muscular atrophy of unilateral upper limb associated with cervical disc herniation in a juvenile male].
A 25-year-old man first noticed atrophy of right upper arm at the age of 15. This symptom progressed for the following 3 years and then became stable. As he noted difficulty in elevating his right arm above the shoulder level, at the age of 25 he was admitted to our hospital. On neurological examination, he had right scapula alata, moderate atrophy and weakness of right triceps muscle. Right triceps reflex was absent. Other neurological examinations were normal. Needle electromyography revealed neurogenic patterns in the muscles of the C5-T1 segments. Cervical MRI and CT myelography revealed mild cervical disc protrusion toward the right side at the C5/6 level and atrophy of the middle and lower cervical cord on the right side, but nothing suggestive of the fexion myelopathy. The clinical manifestations of the patient resembled those of Hirayama disease, except for the distribution of the involved muscles. Since the most severely involved level of the cervical cord (C7 segment) corresponded to the level of the protruded cervical disc, it was suggested that the cervical disc herniation played an important role in the development of the myelopathy in this patient.
Adult↗
TNF-beta produced by human T lymphotropic virus type I-infected cells influences the proliferation of human endothelial cells and fibroblasts.
Human T lymphotropic virus type I (HTLV-I) is linked to adult T cell leukemia as well as to HTLV-I-associated myelopathy/tropical spastic paraparesis. In this report, we studied the effects of HTLV-I-infected cell supernatants on HUVEC, fibroblasts, and glioma cells. The HTLV-I-infected cell supernatants (HUT102 and MT-2) strongly inhibited the proliferation of HUVEC, although they enhanced the proliferation of the fibroblasts. Regarding the glioma cells, only the MT-2 supernatant showed weak inhibitory effects on the proliferation. However, the HTLV-I-uninfected cell supernatants showed no effects on these target cells. The biologic activities of both HUT102 and MT-2 supernatants were found to be dose dependent and were reduced by heat treatment at 100 degrees C for 5 min, but not at 56 degrees C for 30 min. These activities were not dependent on the concentrations of HTLV-I viral particles and were only minimally affected by the presence of anti-HTLV-I Abs. A bioassay of various cytokines revealed that the activity of TNF was much higher in the HUT102 and MT-2 supernatants than in the HTLV-I-uninfected cell supernatants (MOLT-4, Jurkat, and K-562). rTNF-alpha and rTNF-beta also showed strong inhibitory effects on HUVEC as well as on the enhancement of the fibroblast growth. With the use of Sephadex G-100 column chromatography, we obtained the highest activities from the 60- through 70-kDa fractions of the HUT102 supernatant and some activities from the 20- through 30-kDa fractions. The biologic activities of both the whole HUT102 supernatant and its active fractions were completely blocked by anti-TNF-beta mAb, although they were not blocked by anti-TNF-alpha mAb. In a Western blot assay, the 25- and 27-kDa bands of TNF-beta were shown clearly in the HUT102 supernatant, although no TNF-alpha bands appeared. These findings suggest that TNF-beta is present in either its oligomeric or monomeric form in the HTLV-I-infected cell supernatants and is also mainly responsible for the supernatants' effects on HUVECs and fibroblasts.
Cell Division↗
Sequence heterogeneity of HTLV-I proviral DNA in the central nervous system of patients with HTLV-I-associated myelopathy.
The nucleotide sequence of human T-lymphotropic virus type I (HTLV-I) in central nervous system tissue was determined in 3 autopsy cases with HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP) and 1 seropositive carrier without HAM/TSP but with multiple sclerosis. All HAM/TSP samples (3 spinal cords and 2 brains) and the sample from the seropositive carrier without HAM/TSP (brain) were positive for HTLV-I env (5146-6681), pX5' (6549-7494), and pX3' (7354-8276) regions by the two-step polymerase chain reaction method. A nucleotide sequence analysis of the pX5' and pX3' polymerase chain reaction products from nucleotides 6631 to 8259 revealed heterogeneity of the HTLV-I genome in all cases. It is notable that 13 of 50 clones derived from the pX3' polymerase chain reaction products were defective in the tax open reading frame while 7 were defective in the rex open reading frame in the HAM/TSP samples. All 17 clones from 1 HAM/TSP case were defective in the pX open reading frame II. One nucleotide insertion at 7784 creating a frame shift in both tax and rex was seen in all 3 HAM/TSP cases but not in the HTLV-I carrier without HAM/TSP. The pX-defective mutants found frequently in the central nervous system may contribute to the neural damage, since the pX gene products are essential for the transactivation of various cellular genes as well as for viral replication.
Aged↗
Recurrent opticomyelitis associated with anti-DNA antibody.
Two patients had frequent attacks of acute myelitis and optic neuritis. One patient lacked any other organ involvement whereas the other developed systemic manifestations of systemic lupus erythematosus 14 years after the onset. Both patients developed antinuclear and antidouble strand DNA antibodies after the onset of neurological involvement. These patients, whose neurological manifestations were indistinguishable from multiple sclerosis, were thus considered to have systemic lupus erythematosus related collagen disease.
Adult↗
Vitamin B12 metabolism and massive-dose methyl vitamin B12 therapy in Japanese patients with multiple sclerosis.
Serum vitamin B12 levels and unsaturated vitamin B12 binding capacities were measured in 24 patients with multiple sclerosis (MS), 73 patients with other neurological disorders and 21 healthy subjects. There was no decrease in the vitamin B12 levels, however, a significant decrease in the unsaturated vitamin B12 binding capacities was observed in patients with MS when compared with other groups. A massive dose of methyl vitamin B12 (60 mg every day for 6 months) was administered to 6 patients with chronic progressive MS, a disease which usually had a morbid prognosis and widespread demyelination in the central nervous system. Although the motor disability did not improve clinically, the abnormalities in both the visual and brainstem auditory evoked potentials improved more frequently during the therapy than in the pre-treatment period. We therefore consider that a massive dose methyl vitamin B12 therapy may be useful as an adjunct to immunosuppressive treatment for chronic progressive MS.
Adult↗
[The nervous system involvement in human retroviral infection].
Human T-lymphotropic virus type I (HTLV-I) is a pathogenic retrovirus associated with a chronic progressive myelopathy, termed HTLV-I-associated myelopathy (HAM). A chronic inflammatory process has been implicated in HAM by a pathological study, but the exact mechanism still remains to be elucidated. Our quantitative polymerase chain reaction study indicated that the large increase in the HLTV-I proviral DNA in peripheral blood is associated with the development of HAM. The nucleotide sequence analysis of HTLV-I in central nervous system (CNS) tissue of HAM patients revealed that the sequences of HTLV-I genome were heterogenous in all cases, and that the pX-defective mutants were found frequently in the CNS. Thus, HTLV-I exists as quasispecies in vivo, as shown in the case of human immunodeficiency virus. It is possible that the HTLV-I pX microvariants contribute to the neural damage, since the pX gene products are essential for the transactivation of various cellular genes as well as for viral replication.
Animals↗
[Acute relapsing sensory-dominant polyneuropathy associated with anti-GQ1b antibody and autoimmune hepatitis].
A 43-year-old male with 2 episodes of sensory impairments in four extremities and liver dysfunction, developed an acute exacerbation of both sensory impairments and liver dysfunction after administration of interferon-alpha. On admission, neurological examination revealed a mild distal weakness of four extremities, moderate impairment of superficial sensation in hands and severe impairment of deep sensation and areflexia in all extremities. Routine laboratory tests were normal except for a mild liver dysfunction. His serum was positive for antinuclear antibody, but negative for anti-DNA antibody and LE-test. Since he was seropositive for hepatitis B (HB) c antibody but seronegative for HBs antigen and antibody, HBe antigen and antibody, he was considered to be a seroconverted carrier of HB virus. Liver biopsy revealed chronic active hepatitis with marked lymphocytic infiltration. CSF examinations were within normal limits. Sensory conduction studies of median and sural nerves showed no response, but motor conduction studies of median and peroneal nerves were within normal limits. Light and electron microscopic examination of biopsied sural nerve disclosed a moderate decrease in large myelinated fibers, but not in either small myelinated or unmyelinated fibers. Thin-layer chromatography with immunostaining showed the presence of anti-GQ1b antibody in his serum. The anti-GQ1b antibody did not react with GT1a. Oral administration of prednisolone alleviated liver dysfunction, muscle weakness and superficial sensory impairment of four extremities, but not in deep sensation.
Acute Disease↗
In vivo infection of human T-cell leukemia virus type I in non-T cells.
Using three different procedures, immunomagnetic isolation, FACS and panning, each of the T4 cell, T8 cell, monocyte, and B cell population was fractionated from peripheral blood mononuclear cells (PBMC) obtained from 10 human T cell leukemia virus type I (HTLV-I)-associated myelopathy (HAM)/tropical spastic paraparesis (TSP) cases, 3 adult T cell leukemia and lymphoma (ATLL) cases and 9 HTLV-I-infected healthy carriers. DNA from each fraction was then subjected to the quantitative polymerase chain reaction. Although T4 cells possessed the highest copy number of HTLV-I in general, we also detected a significant amount of HTLV-I provirus in T8 cells, monocytes, and B cells. To confirm virus infection of the non-T cell populations, especially the monocyte fraction, we performed further experiments and the following results were obtained: (1) HTLV-I tax RNA expression was confirmed simultaneously in the RNA of monocytes that were negative for T cell receptor beta chain (TCR beta) expression in vivo. (2) We cultured these monocytes for a short period of time and detected HTLV-I antigen expression in non-specific esterase-positive monocyte/macrophage cells. Our data indicate that HTLV-I has a broad host range in vivo and that monocytes or cells of monocyte lineage such as tissue macrophages might comprise a virus reservoir in vivo.
B-Lymphocytes↗
Chronic progressive spinocerebellar syndrome associated with antibodies to human T-lymphotropic virus type I: clinico-virological and magnetic resonance imaging studies.
The relationship between HTLV-I infection and the development of spinocerebellar degeneration (SCD) in adulthood was studied. The frequency of anti-HTLV-I antibodies among patients with SCD (8 of 43, 19%) was found to be higher than the seroprevalence rate in the northern part of Kyushu island (6%). All HTLV-I carriers with SCD showed chronic progressive cerebellar ataxia as a predominant feature and cerebellar atrophy was evident in 6 of 8 patients on magnetic resonance imaging (MRI) of the brain. However, SCD patients with anti-HTLV-I antibodies showed a significantly higher frequency of cerebral white matter lesions on brain MRI (6 of 8) and pyramidal tract signs in lower extremities (6 of 8) than did those without the antibodies (35%, P < 0.05 and 26%, P < 0.01, respectively). Moreover, the HTLV-I carriers with SCD exceeded 14 HTLV-I carriers having neither SCD nor HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP) (21%, P < 0.05), and 59 HTLV-I seronegative patients without brain disorders (19%, P < 0.001) in the frequency of white matter lesions. On the other hand, the HTLV-I carriers with SCD were distinct to the HAM/TSP patients in the predominance of cerebellar involvement. Therefore, the peculiar features of cerebellar, pyramidal and white matter involvement in the HTLV-I-seropositive SCD patients as well as the increased frequency of HTLV-I infection among SCD patients suggest that this syndrome may be a distinct subtype of HTLV-I-associated neurologic diseases.
Aged↗
Primary progressive versus relapsing remitting multiple sclerosis in Japanese patients: a combined clinical, magnetic resonance imaging and multimodality evoked potential study.
Thirty-five Japanese patients with relapsing remitting multiple sclerosis (RRMS) and 11 Japanese patients with primary progressive MS (PPMS) were compared by a combined clinical, magnetic resonance imaging (MRI) and multimodality evoked potential (MEP) study. Patients with PPMS showed a more common occurrence of gait disturbance as the initial symptoms as well as a more common occurrence of cerebellar symptomatology than did those with RRMS, while spinal cord symptomatology was frequently observed in both conditions. On brain MRI, patients with PPMS had 3 times more lesions than did those with RRMS (P < 0.001, chi 2-test). The percentage of infratentorial lesions was also significantly higher in PPMS than in RRMS on MRI. Moreover, we found a significantly higher frequency of abnormal records in visual, brainstem auditory and somatosensory evoked potentials in PPMS than in RRMS. Interestingly, clinically unexpected abnormalities were significantly more common in PPMS than in RRMS throughout all modalities of MEPs. Thus, in Eastern MS, there exists a distinction between PPMS and RRMS in the MRI and MEP findings as well as in the clinical findings. Our result therefore suggest that there may be two distinct subtypes in MS.
Adult↗
Hypertrophic cranial pachymeningitis associated with mixed connective tissue disease; a comparison with idiopathic and infectious pachymeningitis.
This is the first report describing hypertrophic cranial pachymeningitis which developed in association with mixed connective tissue disease (MCTD). A 56-year-old man with a two-year history of MCTD gradually developed symptoms of headache and blurred vision. Bilateral papilledema and increased opening pressure and mild pleocytosis of the cerebrospinal fluid were noted. Magnetic resonance imaging of the brain revealed a diffuse thickening and a gadolinium-enhancement of the cranial dura mater. While five previous patients, diagnosed as idiopathic or infectious hypertrophic cranial pachymeningitis in our department, had either otitis media or sinusitis and showed multiple cranial nerve involvement, the present patient had neither otitis media nor sinusitis and did not show any cranial nerve involvement other than papilledema.
Adult↗
Presence of HTLV-I proviral DNA in central nervous system of patients with HTLV-I-associated myelopathy.
The polymerase chain reaction (PCR) method was used to determine the presence and amount of human T lymphotropic virus type I (HTLV-I) proviral DNA in central nervous system (CNS) tissue obtained at autopsy from 6 patients with HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP), 1 patient with adult T-cell leukemia (ATL) and CNS infiltration of leukemic cells, and 9 control subjects with other neurological disorders. HTLV-I pX and env but not pol DNA were detected in CNS tissue from 5 of 6 patients with HAM/TSP. The ATL samples were positive for pX, env, and pol DNA by PCR. None of the control samples was consistently positive for HTLV-I by PCR, but all showed positive bands on beta-globin PCR. Quantitative PCR combined with histological studies showed no correlation between HTLV-I proviral DNA amounts and extent of perivascular mononuclear cell infiltration in the HAM/TSP CNS. Also, the amounts of pX and probably env DNA were greater in the HAM/TSP samples than in the ATL sample, although the extent of mononuclear cell infiltration was far less in the HAM/TSP samples than in the ATL sample. Therefore, in addition to infiltrating mononuclear cells, constituent cells of the CNS may harbor the HTLV-I genome, at least in the pX and env regions, in patients with HAM/TSP.
Base Sequence↗
Antibody titers to HTLV-I-p40tax protein and gag-env hybrid protein in HTLV-I-associated myelopathy/tropical spastic paraparesis: correlation with increased HTLV-I proviral DNA load.
We studied the relationship between antibody titers to recombinant HTLV-I p40tax protein and gag-env hybrid protein in serum (by an enzyme-linked immunosorbent assay) and HTLV-I proviral DNA load in peripheral blood mononuclear cells (by a quantitative polymerase chain reaction method) in 18 patients with HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP), 17 HTLV-I carriers without HAM/TSP and 16 HTLV-I uninfected controls. The IgG and IgA antibody titers to either of the proteins correlated significantly with the HTLV-I pX (coding p40tax protein) and pol DNA amounts in HTLV-I infected subjects. HAM/TSP patients had significantly higher titers of IgG and IgA antibodies to the HTLV-I proteins than did the HTLV-I carriers without HAM/TSP. While the IgM antibodies to the HTLV-I proteins were found in only 6% of HTLV-I carriers without HAM/TSP, they were found in 40% of HAM/TSP patients, especially those having both a high HTLV-I proviral DNA load and high titers of the IgG and IgA antibodies. HAM/TSP patients with the IgM antibodies had a tendency to deteriorate more frequently on the Kurtzke's disability status scale and magnetic resonance imaging of the brain (leukoencephalopathy) than did those without in the two-year follow-up. Thus, the presence of IgM antibody and high titers of IgG and IgA antibodies to the HTLV-I proteins, together with the increased HTLV-I proviral DNA load, appears to distinguish HAM/TSP patients from HTLV-I carriers without HAM/TSP.
Adult↗
Increase of peripheral B lymphocytes committed to the production of monoreactive and high affinity antibodies to HTLV-1 in patients with HAM/TSP.
We quantitated the frequency of B lymphocytes capable of producing antibodies to HTLV-1 in the peripheral blood from patients with HAM/TSP, non-HAM/TSP HTLV-1 carriers and seronegative healthy subjects. Epstein-Barr virus (EBV) was used as a polyclonal activator of B lymphocytes in a limiting dilution condition. We found that B lymphocytes committed to the production of monoreactive-IgG and -IgA antibodies to recombinant HTLV-1 (gag + env) hybrid protein were significantly increased in a number in patients with HAM/TSP as compared to non-HAM/TSP HTLV-1 carriers and seronegative healthy subjects. By transforming these B lymphocytes with EBV and fusing them with human-mouse heteromyeloma (F3B6), a stable hybridoma producing IgG monoclonal antibody (mAb) to HTLV-1 (gag + env) protein was generated from a patient with HAM/TSP. This mAb (IgG1, kappa), designated F31.1, specifically bound to the amino acid residues from 235 to 254 of HTLV-1 envelope glycoproteins (gp46) with high affinity (Kd = 4.0 x 10(-9) mol/l). These data indicate that the antigen-driven process of B lymphocytes maturation by HTLV-1 antigens is markedly increased in patients with HAM/TSP.
Antibody Affinity↗
Hypertrophic cranial pachymeningitis due to Aspergillus flavus.
A 59-year-old woman suffered from occipital headache and bilateral cranial nerve VII, VIII, IX, X, XI and right XII deficit after developing otitis media. Magnetic resonance imaging (MRI) showed a thickening of the dura mater which was enhanced by gadolinium-DTPA (Gd). Aspergillus flavus was identified from the culture of otorrhea. She was treated with miconazole, flucytosin and fluconazole, which resulted in an improvement of the clinical symptoms and a thinning of the Gd-enhanced lesions on MRI. This is the first case of hypertrophic cranial pachymeningitis caused by Asp. flavus infection.
Aspergillosis↗
[A case of malignant rheumatoid arthritis with corticosteroid-reactive subacute myelopathy and multiple peripheral neuropathy].
We reported a 64-year-old man who had malignant rheumatoid arthritis (MRA) and developed subacute myelopathy and peripheral neuropathy. He had suffered from seropositive rheumatoid arthritis for 4 years, and developed weakness of four limbs, dysuria and constipation two months before the admission. Neurological examination revealed the diffuse muscle wasting and weakness in four limbs. Deep tendon reflexes were hyperactive in four limbs, but not in jaw jerk. Babinski sign was positive bilaterally. Deep sensation was decreased in four limbs and superficial sensation was decreased below the neck. Dysuria and constipation were noted, but anal and bulbocavernosus reflexes were present. On laboratory examination, RF and RAHA increased markedly. Serum complements decreased and immune complexes were positive. Nerve conduction study demonstrated multiple entrapment neuropathy in addition to mononeuritis multiplex. Histological examination of the biopsied sural nerve disclosed the obliterating endarteritis in the epineurium, and marked decrease in number of myelinated fibers. No compressive lesions were seen in the spinal canal by spine X-ray and MRI. Assuming that inflammatory process induced cervical myelopathy, corticosteroid therapy (predonisolone 60 mg/day) was administered and alleviated neurological symptoms, in accordance with the improvement of serological abnormalities. Therefore, an inflammatory process associated with MRA was supposed to damage the spinal cord as well as peripheral nerves in the present case.
Acute Disease↗