Serial magnetic resonance imaging shows separate medial and lateral medullary infarctions resulting in the hemimedullary syndrome.
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Adult↗
Biomedical subjects
M Hirayama
Publications and source records attributed to M Hirayama.
CAG repeat number correlates with the rate of brainstem and cerebellar atrophy in Machado-Joseph disease.
We compared the CAG repeat length and the severity of the brainstem and cerebellar atrophy visualized by MRI in 30 patients with Machado-Joseph disease. We found a strong correlation between the CAG repeat number and the quotient of the degree of atrophy divided by age at examination. These results suggest that the rate of disease progression is dependent on the CAG repeat size and disease progression may commence at birth.
Adult↗
[Hearing recovery in sudden deafness with profound hearing loss].
We investigated the hearing recovery in 51 patients with sudden deafness with the initial averaged five-frequency hearing levels worse than or equal to 100 dB. Twenty-two patients were treated in an outpatient setting with a peri-oral steroid, vasodilator, metabolic activator, and vitamin B. The remaining 29 patients were treated in the hospital with additional hyperbaric oxygenation therapy and/or Satellite ganglion block. The results were as follows. 1) The ultimate averaged five-frequency hearing levels were mainly distributed from 55 to 80 dB. Only seven patients showed ultimate hearing levels worse than 100 dB. Two patients achieved complete recovery better than 20 dB. 2) There was no significant difference in the hearing recovery between the patients treated in an outpatient setting and in the hospital. 3) The time interval until the hearing recovery began was distributed broadly between 2 and 28 days from the onset. While most of the patients who began to recover within 14 days from the onset showed ultimate hearing levels better than 80 dB, the patients who recovered after 14 days had worse hearing levels.
Adolescent↗
A new antitumor antibiotic, BE-19412A, produced by a streptomycete.
A new antitumor substance, designated BE-19412A, was isolated from the culture broth of Streptomyces sp. A19412. The active principle was extracted from the mycelium by methanol and purified by Silica gel and Sephadex LH-20 column chromatographies. BE-19412B was prepared by methylation of BE-19412A. BE-19412A and B exhibited cytotoxic activity against murine and human tumor cell lines. BE-19412A prolonged the survival of CDF1 mice bearing i.p. implanted Ehrlich carcinoma cells.
Animals↗
[Encephalopathy induced by fleroxacin in a patient with Machado-Joseph disease].
Convulsive seizure with unconciousness is an adverse effect of new quinolone antibiotics including fleroxacin. A block of GABA receptor in CNS has been reported as pathomechanism. A 48-year-old female patient with Machado-Joseph disease (MJD) had encephalopathy induced by fleroxacin. She revealed unconsciousness after the administration of fleroxacin (200mg/day) for three days. Electroencephrogram (EEG) showed diffuse slow waves. The administration was discontinued and her consciousness became clear after a day. The abnormal findings on EEG disappeared gradually. The concentrations of fleroxacin were within normal limits in serum and cerebrospinal fluid. The patient with MJD might have a tendency to develop encephalopathy by fleroxacin, because the GABA-ergic nervous system could be involved in MJD.
Anti-Infective Agents↗
Cisplatin neurotoxicity presenting as reversible posterior leukoencephalopathy syndrome.
Visual disturbance, hypertension, convulsions, and unconsciousness developed in a 70-year-old man after cisplatin chemotherapy and upper-limb amputation for osteosarcoma. MR imaging revealed bilateral reversible abnormalities in the occipital, parietal, and frontal white matter. Clinical and neuroradiologic features corresponded to reversible posterior leukoencephalopathy syndrome (RPLS), which some immunosuppressive and chemotherapeutic drugs have been reported to trigger. Cisplatin may be among these drugs. Our patient also had hypomagnesemia, which may have figured in the pathophysiology.
Aged↗
[Coxsackie virus B4 encephalitis in a young female who developed mental symptoms, and consciousness disturbance, and completely recovered].
An 18-year-old female had common cold and insomnia in early March 1987. Later, abnormal speech and behavior, emotional incontinence, anorexia and consciousness disturbance appeared. On March 19, she was admitted to our hospital in semi-comatose state. Myoclonus-like movement on hands was observed, and epileptic attacks with tonic and clonic convulsions occasionally occurred. There were no neurological findings that suspected cerebral focal lesions. The respiration was assisted through tracheal intubation. Laboratory examinations showed inflammatory reactions (CRP+2, WBC 10,600) and transient high levels serum CK (6,215 IU). As she had bradycardia (30-40/min) with complete AV block on ECG, the pacemaker was implanted. The complication of myocarditis was suspected. EEG showed bilateral slow waves (3-6Hz), dominantly in frontal areas. Brain CT and CSF examinations were normal. After the combined administration of ara-A, dexamethasone and anti-convulsant, the consciousness level was recovered within a month. The serum antibody against coxsackie virus B4 alone was significantly increased. We concluded that coxsackie virus B4 caused acute encephalitis with mental symptoms and myocarditis with AV block. Recently, cytomegalovirus was reported to be the causative virus in a young female with non-HSV encephalitis who showed mental symptoms with good prognosis, but coxsackie virus B4 should also be considered as one of the causative viruses.
Adolescent↗
What kind of patients are suitable for evaluating the therapeutic effect of sudden deafness?
OBJECTIVE: This study aimed to find out appropriate patients to evaluate the therapeutic effect of sudden deafness. STUDY DESIGN: The study design was a retrospective case review. SETTING: The study was performed at a university hospital. PATIENTS: A series of 443 patients with idiopathic sudden sensorineural hearing loss who visited the authors within 1 week from the onset participated. INTERVENTIONS: Relationship between interval from the onset and treatment prognosis was investigated. RESULTS: The significant difference in prognosis related to the days from the onset to the initial visit was noted only in the patients with the initial hearing level from 50-65 dB. The prognosis of the patients who visited the authors on the next day from the onset was excellent. Whereas, in the patients with initial hearing level worse than 70 dB, no significant difference in prognosis was noted among the days of visit. CONCLUSIONS: The patients with the initial hearing level worse than 70 dB and who visited the authors within 8 days from the onset were appropriate for candidates in evaluating the therapeutic effect of sudden deafness.
Adult↗
GM1 gangliosidosis type 3 with severe jaw-closing impairment.
The patient had adult GM1 gangliosidosis (type 3) with severe impairment of mastication caused by dystonia of anterior digastric muscles (jaw-opener) on clenching. This is the first report on jaw dystonia severe enough to cause the masticatory impairment in adult GM1 gangliosidosis. The discordance of closing and opening muscles during mastication might be caused by a basal ganglia lesion in this disease.
Adult↗
Chorea-acanthocytosis with polyclonal antibodies to ganglioside GM1.
A patient with chorea-acanthocytosis presenting with axonal neuropathy showed an elevation in IgM polyclonal antibodies to the GM1 ganglioside, which were estimated by enzyme-linked immunosorbent assay and complement-mediated liposome immune lysis assay (LILA). This is the first demonstration of such antibodies in chorea-acanthocytosis. Anti-GM1 antibodies might have directly caused the axonal neuropathy by binding to GM1 or cross-reactive antigens in the nerves.
Acanthocytes↗
Relationship between respiratory failure and plasma noradrenaline levels in amyotrophic lateral sclerosis.
We evaluated plasma noradrenaline (NA) levels at test and during head-up tilt test in 20 patients with sporadic amyotrophic lateral sclerosis (ALS). Their fasting plasma NA levels ranged from 195 to 4227 pg/ml. The average plasma NA level was 483 pg/ml in five ambulatory patients, 341 in two wheelchair-bound patients, 1264 in 11 bedridden patients, and 208 in two respirator-dependent patients whose disability grading was the worst among the four groups. Arterial carbon dioxide (PCO2) was evaluated as a measure of respiratory function. The coefficient of correlation between PCO2 and plasma NA was r = 0.654 (p < 0.01). Either respiratory failure or lower motor neuron dysfunction may relate to the elevation of plasma NA levels. In the two bedridden patients, plasma NA levels and heart rate at rest increased significantly as the disease progressed. Cardiovascular responses to head-up tilting were normal. These data suggest that the elevation of plasma NA levels may be related to progression of respiratory failure and lower motor neuron dysfunction. In conclusion, sympathetic hyperactivity in ALS is considered to be not primary, but secondary to somatic motor disabilities and respiratory failure.
Aged↗
Predictors of the progression of diabetic nephropathy and the beneficial effect of angiotensin-converting enzyme inhibitors in NIDDM patients.
A progressive decline in glomerular function occurs in diabetic nephropathy. The predictive effects of progression promoters were examined in 182 non-insulin-dependent diabetic patients from a baseline serum creatinine concentration of 133 mumol/l. During a total of 605 person-years follow-up, 107 patients developed end-stage renal failure requiring dialysis. The rate of decline of renal function was highly variable. Urinary protein excretion was the strongest predictor correlated to the rate of decline, followed by diastolic and systolic blood pressure, total cholesterol and platelet count, while the protective effects were seen in serum albumin and haematocrit. Adjustment for urinary protein excretion revealed that diastolic blood pressure, familial predisposition to hypertension, serum albumin, and smoking were independent significant predictors. Angiotensin converting enzyme inhibitors (ACE-I) significantly retarded the development of end-stage renal failure compared to antihypertensives other than ACE-I (mostly nifedipine), and the effect was evident particularly in patients with proteinuria below the median (2.5 g/24 h) (presumably those who responded to ACE-I). A complex effect of proteinuria in association with blood pressure elevation, familial predisposition to hypertension, hypoalbuminaemia, and smoking may play an important role in the progression of nephropathy.
Angiotensin-Converting Enzyme Inhibitors↗
Salvage therapy for relapsed or refractory childhood acute lymphocytic leukemia by alternative administration a lymphoid- and myeloid-directed chemotherapeutic regimen consisting of dual modulation of ara-C, hydroxyurea, and etoposide.
Risk-directed chemotherapeutic regimens in recent use have improved the prognosis of children with acute lymphocytic leukemia (ALL). However, many patients relapse during or shortly after cessation of the initial continuation chemotherapy. Since achievement of a second complete remission (CR) is the initial step in successful retreatment effort, it is important to develop salvage protocols for children with relapsed or refractory ALL. In the present study, we developed a new salvage protocol (MLL-93) and applied the concept of dual chemical modulation of cytarabine, hydroxyurea, and etoposide with the alternative administration of high doses of myeloid- and lymphoid-directed agents. We also planned to perform allogeneic bone marrow transplantation (BMT) following a CR if patients had HLA-identical donor(s). The six patients treated with the MLL-93 protocol achieved a second CR. One patients in CR died of interstitial pneumonia after an unrelated allogeneic BMT. The other five patients have been in CR for 12-41 months. We suggest that the concepts of alternative administration of lymphoid- and myeloid-directed drugs and biochemical modulation are useful in the treatment of children with relapsed or refractory ALL.
Antineoplastic Combined Chemotherapy Protocols↗
Two cytotoxic pathways of natural killer cells in human cord blood: implications in cord blood transplantation.
Using K562 cells as a target we investigated cord blood (CB)-natural killer (NK) cytolytic pathways. The cytotoxicity of fresh CB-NK cells was significantly lower than that of peripheral blood mononuclear cells (PB MNCs). When CB was incubated with IL-2, the level of CB-NK cytotoxicity was increased and boosted to the level observed in PB-NK cells. Fresh CB-NK cells induced apoptosis in target cells. Activated CB cells induced apoptosis and necrosis in target cells, at the same level as PB MNCs. CB stem cell transplantation may also induce graft-versus-host disease (GVHD)/graft-versus-leukaemia (GVL), similar to bone marrow transplantation.
Apoptosis↗
Cytotoxic T-lymphocytes recognizing P-glycoprotein in murine multidrug-resistant leukemias.
A multidrug-resistant murine lymphoid leukemia P388/ADR overexpresses P-glycoprotein (P-gp), an active transporter that pumps cytotoxic drugs out of cells and a product of mdr1 gene. Cytotoxic T lymphocytes (CTL) that showed cytotoxicity against P388/ADR were generated from mixed lymphocyte tumor cell culture. CTL do not kill drugsensitive parental P388 (P388/parent) that does not express P-gp. Monoclonal antibody against P-gp inhibited cytotoxic activity. Similar results were obtained in another multidrug-resistant cell line P388/VP-16. Cytotoxic activity was mediated by Thy1+ CD4- CD8+ T-cells. When P388/ADR was treated with murine IL-4, expression of P-gp was downregulated. Monoclonal antibody against interleukin-4 (IL-4) abrogated the IL-4-induced suppression of P-gp. Cytolytic activity of CTL against IL-4-treated P388/ADR was dose dependently inhibited. These results suggest that P-gp is immunogenic and can be a target of CTL in this murine leukemia model.
ATP Binding Cassette Transporter, Subfamily B, Mem↗
Characterization of a water-soluble polysaccharide fraction with immunopotentiating activity from Bifidobacterium adolescentis M101-4.
The soluble and insoluble fractions obtained after sonication and centrifugation of Bifidobacterium adolescentis M101-4 cells were examined, and both of these fractions exhibited mitogenic activity in an assay of murine splenocytes and Peyer's patch cells in vitro. The soluble fraction was further treated by a 6-step procedure involving proteinase K-treatment, ultrafiltration with a 50-kDa cut-off molecular-sieving membrane, anion-exchange chromatography, dialysis, ultrafiltration through a 6-kDa cut-off membrane filter, and gel-filtration to yield a soluble high molecular weight fraction (SHF) which was effective for stimulating the proliferation of murine splenocytes. Almost three quarters of this fraction by weight was found to consist of carbohydrates containing glucose and galactose as major constituents, and the average molecular weight was estimated to be between 60,000 and 2,460,000, with the main peak at 1,550,000 Da, by the retention time of gel permeation chromatography. A structural analysis by 1H- and 13C-nuclear magnetic resonance and methylation indicated that SHF contained polysaccharides consisting of -4Galp1-, -4Glcp1-, and -6Glcp1- as the major residues, and Galf1- and -6Galf1- as the minor residues. Immunopotentiating SHF was found to contain galactofuranosyl residues as characteristic constituents which had not been previously detected in other soluble fractions from Gram-positive bacteria.
Adjuvants, Immunologic↗
Malignant lymphoma involving the penis following malignant pleural mesothelioma.
A 74-year-old man who had been diagnosed with malignant mesothelioma developed malignant lymphoma of B-cell origin involving the penis. He had a history of occupational exposure to asbestos as a construction worker. The association of malignant mesothelioma with lymphoma is rare, and the possibility of asbestos exposure as a common etiology is discussed. The intense stimulation of B lymphocytes and decreased T lymphocyte activity in asbestos-exposed populations may result in development of B-cell malignancies. Though the relationship between asbestos exposure and malignant mesothelioma is firmly established, the relationship between asbestos exposure and lymphoma remains to be investigated.
Aged↗
[Impaired associated movement in Parkinson's disease].
We analyzed the intended voluntary movement and simultaneously occurring automatic associated movement in Parkinson's disease(PD). We studied wrist dorsiflexion as a movement associated with grip and Bell's phenomenon(upward rolling of the eyeballs on forcible closure of the eyelids) in patients with PD and normal controls. The patients showed significantly smaller wrist dorsiflexion and upward rolling of the eyeballs. Patients with PD have a greater reduction of automatic associated movement than intended voluntary movement. This discrepancy between the associated movement and voluntary movement may be one of the bases of clinical symptoms of PD patients in early stage.
Basal Ganglia↗