Biomedical subjects

T Mitsuma

Publications and source records attributed to T Mitsuma.

At least 127 records · Page 7Linked to original sources

PubMed1995

[Cerebellar infarction in the territory of the superior cerebellar artery, presenting a predominant cerebellar symptom--with special reference to its pathophysiology].

Clinical features of cerebellar infarction in the territory of the superior cerebellar artery (SCA) were investigated in six male patients, ranging in age from 50 to 69 years. In all patients, there were MR images of infarction located in the area supplied by the SCA. The lesion was on the left-side in 2, right-side in 3 and bilateral (recurrent) in 1 patient. The onset of disease occurred with nausea, vomiting and floating sensation, with no overt brain stem signs other than symptoms of unilateral cerebellar ataxia and dysarthria. Five of the 6 patients had heart disease and cerebral angiography without definite evidence of SCA occlusion, strongly suggesting occlusion of the artery at its periphery due to cardiogenic embolism. A comparison of these 6 patients with those reported previously in Japan suggests that patients with SCA occlusion may be divided into two distinct subgroups: one manifesting diffuse brain stem signs in addition to cerebellar signs, and the other showing cerebellar signs as the only neurologic manifestation. In the former group, comprising the vast majority of patients, SCA occlusion occurred at the origin of the vessel due to a thrombus under a state of hypertension, diabetes mellitus or malignancy, producing signs of brain stem involvement, such as dissociating sensory disturbance and Horner's sign. While in the latter group, which included these 6 patients, paucity of brain stem signs, absence of definite cerebral angiographic evidence of SCA occlusion, and the presence of heart disease were distinguishing clinical features. Cardiogenic cerebral embolism was probably the underlying pathology in many of the cases and the functional prognosis was favorable.

Aged↗

PubMed1995

[Disturbance of hypothalamic-pituitary hormone secretion in familial chorea-acanthocytosis].

An endocrinological study was performed dopaminergic regulation of the hypothalamic-pituitary axis of 3 patients with familial chorea-acanthocytosis (females, 38 to 47 years of age). All 3 patients exhibited low basal levels of triiodo-thyronine (T3), and 2 patients had a slightly elevated baseline plasma prolactin (PRL) level. The patients had a delayed plasma thyroid stimulating hormone (TSH) response and plasma PRL excessive response to thyrotropin releasing hormone (TRH), and a low plasma growth hormone releasing hormone (GRF) response to L-dopa. These TSH, PRL and GRF responses represent a secretion pattern due to a hypothalamic disorder, suggesting impaired regulation of hormone secretion by the dopaminergic system, primarily in the hypothalamus. In addition, an increased growth hormone (GH) response secretion following TRH (paradoxical response) was observed in 2 patients, suggesting that the pituitary was also involved. An oral glucose tolerance test (75g-OGTT) revealed a diabetic pattern in all 3 patients, indicating frequent association with impaired glucose tolerance.

Acanthocytes↗

PubMed1995

Possibility of postprandial electrogastrography for evaluating vagal/nonvagal cholinergic activity in humans, through simultaneous analysis of postprandial heart rate variability and serum immunoreactive hormone levels.

OBJECTIVES: To investigate changes in postprandial electrogastrography (EGG) from the neurohormonal mechanisms. METHODS: We measured EGG indices [frequency amplitude of normal 3-cpm (EGG-3 cpm)], high frequency amplitude of heart rate variability which has reflected cardiac parasympathetic tone, and several serum immunoreactive (ir)-hormone levels in 12 fasted male volunteers (mean age 25.3 yr). RESULTS: Immediately after the liquid food intake (250 kcal), a transient decrease in EGG-3 cpm frequency (from 0.045 +/- 0.001 Hz to 0.040 +/- 0.001 Hz; p < 0.05) accompanied by an increase in high frequency amplitude (from 31.05 +/- 3.45 ms to 39.10 +/- 4.08 ms; p < 0.05), and a serum immunoreactive gastrin level increase (from 38.72 +/- 4.92 pmol/L to 54.00 +/- 10.45 pmol/L; p < 0.05) and an immunoreactive somatostatin level decrease (from 15.00 +/- 0.43 pmol/L to 13.70 +/- 0.46 pmol/L; p < 0.01) were observed, suggesting vagal excitement. EGG-3 cpm amplitude and serum immunoreactive human pancreatic polypeptide (hPP) levels significantly increased soon after ingestion, and these changes lasted for 30 min. Furthermore, there was a positive correlation between changes in EGG-3 cpm amplitude and those in serum immunoreactive hPP levels during the postprandial periods (r = 0.55, p < 0.001). CONCLUSIONS: Considering the reports that a cholinergic, nonvagal pathway is of major importance in food-stimulated hPP release, the present results suggest that postprandial changes in EGG-3 cpm frequency and amplitude might be a good tool for evaluating not only vagal but also nonvagal cholinergic activity in the human gut.

Adult↗

PubMed1995

[Expression of MHC and cell adhesion molecules in muscular sarcoidosis].

Biopsied skeletal muscles from 5 patients with muscular sarcoidosis (nodular type; 1, and myopathic type; 4) were immunocytochemically examined. All biopsies presented granulomatous changes. Atrophic or regenerating muscle fibers adjacent to granuloma demonstrated compression or ischemic changes. In the center of the granuloma, CD68+ epitheloid cells and giant cells, and CD4+ T cells were localized. At the periphery of the granuloma, CD4+ T cells, CD8+ T cells, CD20+ B cells, and CD68+ macrophages were found. Expression of HLA-A,B,C was diffuse in the muscle fibers. Expression of HLA-DR and ICAM-1 was more prominent near the granuloma or perifascicular fibers, and that of LFA-3 was moderate in those lesions. VCAM-1 was expressed in endothelial cells and macrophages near the granuloma. Those findings indicate that interferon-gamma or TNF-alpha produced by infiltrating inflammatory cells may induce expression of these immunologic markers or adhesion molecules. Immunocytochemical differences between the nodular and myopathic forms of sarcoidosis are not evident, but either localization or abundance of granuloma in muscle bulks is relevant to weakness or atrophy of clinically affected muscle.

Aged↗

PubMed1995

[Phenotypic heterogeneity in Japanese Charcot-Marie-Tooth disease type 1A patients with PMP-22 gene duplication].

We studied phenotypic heterogeneity in 18 Japanese patients with Charcot-Marie-Tooth disease type 1A (CMT1A) with PMP-22 gene duplication, together with heterogeneity of duplication size. In order to detect the duplication of PMP-22 gene region, the PMP-22 cDNA and a polymorphic marker VAW409R3 were used as probes for Southern blot analysis. As the clinical phenotypes, we assessed the degree of foot deformity, muscular weakness and atrophy, tendon reflexes, sensory impairment and electrophysiologic and sural nerve biopsy findings. Although the degree of muscular weakness and atrophy was slightly more severe in the advanced age, there was a patient with calf hypertrophy in the older age or a patient with marked muscular atrophy of the leg in the younger age. The incidence of foot deformities and sensory impairment was high and these phenotypes did not relate to aging. Diminished or absent tendon reflexes and slowing of motor conduction velocities were commonly seen, but the motor conduction velocity varied greatly among the patients. The clinical phenotypes were extensively variable among the CMT 1A patients with the same gene mutation of PMP-22 gene duplication, suggesting that there is a factor other than PMP-22 gene duplication, which influences phenotypic manifestation in CMT 1A.

Adult↗

PubMed1995

[Relations between autonomic dysfunction and skin sympathetic nerve activity in Guillain-Barré syndrome: a microneurographical assessment].

We investigated the skin sympathetic nerve activity (SSNA) in 4 patients with Guillain-Barré syndrome (CBS) using microneurography. All patients showed transient hypertension, tachycardia and palmoplantar hyperhidrosis in the acute phase of illness, but these symptoms disappeared in the chronic phase. Microelectrode recordings of SSNA performed at the median nerve in the acute phase when hypertension, tachycardia and palmoplantar hyperhidrosis were present and then in the recovery phase. In the acute phase, the level of SSNA was significantly higher than the control levels obtained from 16 healthy subjects, while in the chronic recovery phase the SSNA showed no significant difference from that of the control. The correlation between the SSNA and corresponding changes in the sweat rate and skin blood flow was kept constant both in the acute phase and chronic phase. These findings suggest that the sympathetic nerve dysfunction observed in the acute phase of GBS is partly the consequence of hyperactivity of the skin sympathetic nerve.

Adolescent↗

PubMed1995

[Expression of the heat shock protein 70 in inflammatory myopathies].

Heat shock protein 70 (HSP 70) expression was immunohistochemically observed in diseased muscle fibers of 35 patients with dermatomyositis (DM) and 7 with polymyositis (PM). In DM, HSP 70 was localized in the sarcoplasm of type 1 fibers adjacent to the small vessels showing deposits of complement components in 13 patients and in the atrophic fibers at perifascicular regions in 7. HSP 70 was also expressed more preferentially in the small vessels rather than in the sarcoplasm in 13 DM patients. In PM, the expression of HSP 70 was blurred in all fibers including non-necrotic fibers invaded by T cells. In conclusion, HSP 70 is likely more frequently to be expressed in the sarcoplasm of DM than PM due to probable ischemic insults.

Dermatomyositis↗

PubMed1994

Androgen receptor mRNA with increased size of tandem CAG repeat is widely expressed in the neural and nonneural tissues of X-linked recessive bulbospinal neuronopathy.

We detected androgen receptor (AR) mRNA expression in various tissues in the patients with X-BSNP and controls using reverse transcription polymerase chain reaction (RT-PCR) and Northern blot analysis. The AR mRNAs were expressed in a wide variety of tissues including the testis, scrotal skin, liver, skeletal and cardiac muscles, sciatic nerve, sympathetic and dorsal root ganglia and spinal cord, and were all abnormally elongated in the size of the CAG repeat in the patients. The mutant AR gene with increased size of tandem CAG repeat was directly transcribed in various tissues, and would be related to a wide spectrum of phenotypic manifestations in X-BSNP.

Base Sequence↗

PubMed1994

cAMP-dependent differential regulation of extracellular matrix (ECM) gene expression in cultured rat Schwann cells.

cAMP-dependent regulation of the steady-state mRNA levels for the ECM components, laminin A, B1 and B2 chains, collagen types I, III and IV were examined by Northern blot analysis in cultured rat Schwann cells. ECM mRNAs of laminin B1 chain and collagen types I and IV were expressed at high levels in the control Schwann cells, while laminin B2 chain and collagen type III mRNA levels were low, and laminin A chain mRNA was not detectable. When Schwann cells were treated with forskolin or cAMP derivatives, the gene expression for the ECM molecules constituting the Schwann cell basement membrane, laminin B1 and B2 chains, and collagen type IV, was enhanced in time- and dose-dependent manners for exogenously administered forskolin or cAMP derivatives, while the mRNA levels for the ECM molecules, which are not the major components of the basement membrane, fibrillary collagen types I and III were significantly suppressed. This cAMP-dependent differential regulation of Schwann cell ECM gene expression may be related to the role of each ECM molecule in the peripheral nerve development and regeneration.

Adrenergic beta-Agonists↗

PubMed1994

Phenotypic heterogeneity of an adult form of adrenoleukodystrophy in monozygotic twins.

We describe genetically proven monozygotic twins with the adult form of adrenoleukodystrophy with significant phenotypic heterogeneity. Myeloneuropathy was common to both patients, but cognitive impairment and affective symptoms with extensive demyelination in the brain were prominent in the older twin, while adrenal insufficiency was predominant in the younger twin. The younger twin, however, exhibited affective symptoms similar to those displayed by his elder twin 10 years later. These findings suggest that nongenetic factors are important in determining the phenotypic variation of adrenoleukodystrophy gene.

Adrenoleukodystrophy↗

PubMed1994

Age-related changes of the myelinated fibers in the human corticospinal tract: a quantitative analysis.

A quantitative analysis was made of the myelinated fibers in the lateral corticospinal tract (LCST) at the levels of the 6th cervical, 7th thoracic and 4th lumbar spinal segments in 20 patients between 19 and 90 years old, and who died of non-neurological diseases. The diameter frequency histograms of myelinated fibers of LCST showed a bimodal pattern with a sharp peak of the small myelinated fibers and broad slope of the large myelinated fibers. The ratio of small fiber to large fiber densities was significantly higher in the 6th cervical (P < 0.05) and 4th lumbar segments (P < 0.01) than in the 7th thoracic segments. The density of small myelinated fibers was significantly lowered with advancing age (P < 0.05-0.001), while that of large myelinated fibers was not significantly decreased in the aged patients, although it showed a slight age-dependent declining tendency. Age-dependent decline of small fiber density was more prominent in the cervical and lumbar segments. Retraction of the axon-collaterals from large-diameter myelinated fibers, which are abundant in the cervical and lumbar segments, may contribute to the age-related diminution of the small myelinated fibers in the LCST.

Adult↗

PubMed1994

Disease-specific patterns of neuronal loss in the spinal ventral horn in amyotrophic lateral sclerosis, multiple system atrophy and X-linked recessive bulbospinal neuronopathy, with special reference to the loss of small neurons in the intermediate zone.

The ventral horn cells of the fourth lumbar segment were morphometrically analysed in six cases of amyotrophic lateral sclerosis (ALS; there common forms and three pseudopolyneuritic forms), six of multiple system atrophy (MSA) with autonomic failure, four of X-linked recessive bulbospinal neuronopathy (X-BSNP), and seven age-matched autopsy cases of non-neurological disorders. In the common form of ALS, large and medium-sized neurons of the medial and lateral nuclei were markedly lost; small neurons in the intermediate zone were slightly diminished but fairly well preserved. In the pseudopolyneuritic form of ALS, marked loss was present in the large and medium-sized neurons, and in the small neurons located in the intermediate zone as well. In the MSA, in contrast to ALS, there was a marked reduction in small neurons in the intermediate zone, and large and medium-sized neurons of the medial and lateral nuclei tended to be preserved. In X-BSNP, large and medium-sized neurons were almost completely lost and small neurons were also markedly depopulated. These findings indicated that the pattern of neuron loss in the ventral horn is distinct among these diseases depending on size, location and function of the ventral horn cell population. These disease-specific patterns of neuron loss suggest a difference in the process of neuronal degeneration of ventral horn cells among the disease examined.

Adult↗

PubMed1994

Modulatory effects of calcitonin gene-related peptide and substance P on human cholinergic sweat secretion.

Immunoreactivity to various peptides has been demonstrated in nerve terminals around the sweat glands, suggesting a regulatory function for these peptides on sweating. The present study evaluated the calcitonin-gene related peptide and substance P related regulation of sweating in man. Both calcitonin-gene related peptide and substance P, when administered alone, failed to cause sweat secretion, whereas sweating induced by methacholine chloride alone was four times greater when administered with calcitonin-gene related peptide and suppressed by 70% when administered with substance P. The degree of calcitonin-gene related peptide dependent augmentation and substance P dependent suppression of the methacholine chloride induced sweating was dependent on the concentration of calcitonin-gene related peptide and substance P. These findings suggest that calcitonin-gene related peptide enhances cholinergic sweating and substance P inhibits it.

Adult↗

PubMed1994

Familial amyloidotic polyneuropathy with late-onset and well-preserved autonomic function: a Japanese kindred with novel mutant transthyretin (Ala97 to Gly).

We report the characteristics of one patient and two asymptomatic carriers from a Japanese family with familial amyloidotic polyneuropathy (FAP). The clinical features were somatic sensory and motor neuropathy with well-preserved autonomic function and late onset with slow insidious progression. These symptoms and signs are different from those of type 1 FAP. There were massive amyloid deposits with transthyretin (TTR) in the myocardium and the sural nerve. DNA sequencing of the TTR gene and amino acid sequence analysis of serum TTR revealed a new mutation in which Gly97 was substituted for Ala. We suggest that patients with somatic sensory and motor neuropathy of unknown origin without apparent autonomic dysfunction should be further studied for TTR mutation.

Age of Onset↗

PubMed1994

Aberrant androgen action and increased size of tandem CAG repeat in androgen receptor gene in X-linked recessive bulbospinal neuronopathy.

Plasma levels of testosterone, luteinizing hormone (LH) and follicle-stimulating hormone (FSH) after 3 or 6 days of administration of the synthetic androgenic hormone fluoxymesterone (10 mg/day) were measured in 26 patients with X-linked recessive bulbospinal neuronopathy (X-BSNP) and 22 age-matched male controls. The testosterone, LH and FSH levels in the controls were markedly suppressed after administration, but in the patients with X-BSNP, they were suppressed significantly less. The level of suppression varied considerably with the patients, and those of plasma testosterone and FSH were significantly correlated with the number of CAG repeats in the androgen receptor gene. These findings suggest that the androgen action was aberrantly transduced in the target organs in the patients with X-BSNP and which is related to the elongated CAG repeat in the androgen receptor gene.

Adult↗

PubMed1994

Nerve growth factor prevents neurotoxic effects of cisplatin, vincristine and taxol, on adult rat sympathetic ganglion explants in vitro.

Anti-cancer drugs, cisplatin, vincristine and taxol clinically induce toxic sensory as well as autonomic neuropathy. Administration of nerve growth factor (NGF) has been found to prevent experimental sensory neuropathies induced by these anti-cancer drugs, but the information about autonomic neuropathy is lacking. We developed an adult rat superior cervical ganglion (SCG) explant culture, which we treated with cisplatin, vincristine and taxol either in the presence or absence of NGF. The maximum length of regenerated neurites was shortened by cisplatin, vincristine and taxol in a dose-dependent manner. However cotreatment with NGF significantly promoted the regeneration of neurites in all drug-treated explants. The effect of NGF was clearly blocked by the anti-NGF antibody. These findings suggest that cotreatment of NGF prevents and reverses the toxic effects of the anti-cancer drugs on the sympathetic neurons.

Animals↗

PubMed1994

Gm haplotypes in chronic inflammatory demyelinating polyradiculoneuropathy in Japanese patients.

We studied the serum Gm allotype of 58 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and 236 nonrelated normal controls in Japan. The incidences of the Gm phenotype and haplotype in the patients were not significantly different from those in the normal controls. When CIDP was classified into two subgroups in terms of clinical course, the chronic relapsing (CR) and chronic nonrelapsing type (CNR), the distribution of all Gm haplotypes was significantly different between CR and CNR (total chi 2 = 8.319; corrected p < 0.05). Our findings suggest that the clinical course of CIDP may be associated with the Gm haplotype.

Chronic Disease↗

PubMed1994

Hypothalamic dysfunction in Parkinson's disease patients.

Ten patients with idiopathic Parkinson's disease (PD) (3 men and 7 women, group A) who had received no treatment for the disease; 102 patients with PD (36 men and 66 women, group B) who had undergone treatment and 45 healthy volunteers (15 men and 30 women, control group) were subject to thyrotropin-releasing hormone (TRH) tests and levodopa tests. In group A basal plasma prolactin (PRL) levels were significantly higher than in the controls both before and during treatment. Peak plasma PRL levels during TRH tests were significantly higher before treatment, but returned to the control levels during treatment. Nadir plasma PRL levels during levodopa tests were significantly increased before and during treatment. In group B basal plasma thyroid-stimulating hormone (TSH) and PRL levels were significantly higher than in the control group. Peak plasma PRL levels during TRH tests and nadir plasma PRL levels during levodopa tests were also significantly increased. The results strongly suggest a disturbance of pituitary hormone secretion due to hypothalamic dysfunction in PD patients.

Administration, Oral↗