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Biomedical subjects

M Uchino

Publications and source records attributed to M Uchino.

At least 163 records · Page 9Linked to original sources
PubMed1995

Genetic abnormalities and pathogenesis of familial amyloidotic polyneuropathy.

Familial amyloidotic polyneuropathy (FAP) is an autosomal inherited disease, characterized by extracellular amyloid deposits and by peripheral neuropathy. Amyloid fibrils derived from most types of FAP consist of variant transthyretin (TTR) with single amino acid substitutions, and methionine 30 TTR is the most common variant TTR. TTR is mainly produced in the liver and the choroid plexus. Biochemical and molecular biological techniques have been revealing the amyloidogenicity of variant TTR in vitro and in vivo using the transgenic mouse as a model. It will be important for the development of effective therapy to find out the factors, other than variant TTR, which affect amyloid deposition and define the tissue specificity of amyloid.

Adolescent↗
PubMed1995

Headache associated with aseptic meningeal reaction as clinical onset of Fabry's disease.

This report concerns an 18-year-old boy who is hemizygote for Fabry's disease. Varying degrees of nonpulsating headache crises, lasting from a few hours to several days, began when he was 16 years of age. Painful crises in the extremities, characteristic of Fabry's disease, were not present. Although only occasional, he had several episodes of throbbing headache with vomiting without aura. The meningeal signs were equivocal, although the patient had noninfectious pleocytosis, intracranial hypertension, delayed radioisotope clearance on cisternography, and multiple old cerebral infarcts. Nonsteroidal anti-inflammatory drugs, antidepressants, carbamazepine, and glycerol were of no benefit for his headache. Although its mode of action remains obscure, prednisolone was effective for treating the headache and the aseptic meningeal reaction.

Adolescent↗
PubMed1995

Involvement of the central nervous system in rheumatoid arthritis: its clinical manifestations and analysis by magnetic resonance imaging.

A rheumatoid arthritis (RA) patient showed high signal intensity in the subcortical region of the frontal and occipital lobes on T2-weighted magnetic resonance imaging (MRI). Histopathological examination in the autopsy specimen revealed severe systemic vasculitis. Additional radiological and laboratory studies revealed that transient cerebral ischemia induced by vasculitis occurred in this patient.

Arthritis, Rheumatoid↗
PubMed1995

Neurologic features of chronic Minamata disease (organic mercury poisoning) certified at autopsy.

To better understand the neurologic events related to chronic Minamata disease (organic mercury poisoning), we studied data from 77 patients with Minamata disease as certified at autopsies performed from 1976 to 1994 (mean age: 72.3 years). Major neurologic findings included: sensory impairment in 80.5% of the patients which was limited to the extremities in 42.9%. Impairment of lower extremity coordination was present in 35.8% of the patients, constriction of the visual fields in 28.8%, and retrocochlear hearing loss in 15.3%. There was no correlation between the degree of cerebellar incoordination and the methylmercury concentration in the cerebellum. Compared with the classic type of Minamata disease, the incidence of major neurologic findings was markedly decreased. In light of these findings, supplemental examinations including brain computed tomography (CT), magnetic resonance imaging (MRI), short latency somatosensory evoked potential (SSEP), or tremogram may be necessary to clinically diagnose Minamata disease, especially in atypical or mild cases.

Adult↗
PubMed1995

Change in the age of onset in patients with familial amyloidotic polyneuropathy type I.

To investigate the influence of environmental factor(s) on the pathogenesis of familial amyloidotic polyneuropathy (FAP) type I, the age of onset in 33 FAP patients in the Arao district of Kumamoto Prefecture in Japan and in 20 of their ancestors to the 3rd prior generation was carefully examined. The age of onset in FAP patients progressively became younger. This tendency was more apparent in female FAP patients than in male patients. Since the pathogenesis of amyloid formation in FAP as well as other amyloidosis diseases is not still elucidated, this phenomenon may be of interest.

Adolescent↗
PubMed1995

Adult T cell leukemia following HTLV-I-associated myelopathy/tropical spastic paraparesis: case reports and implication to the natural course of ATL.

Adult T cell leukemia (ATL) is the T cell malignancy caused by human T lymphotropic virus type I (HTLV-I), and HTLV-I is also the causative agent of HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP). Although HTLV-I causes both diseases, concomitant occurrence is reported to be rare. This paper describes two cases of HAM/TSP that developed into lymphoma-type ATL after the onset of HAM/TSP. In one case, the same HTLV-I infected clone could be detected by polymerase chain reaction in peripheral blood obtained when the patient was diagnosed as HAM/TSP. This finding showed that the HTLV-I clone already existed at the stage of HAM/TSP. Since frequent detection of clonal proliferation of HTLV-I infected cells has been reported previously in patients with HAM/TSP, careful follow-up is needed for patients with HAM/TSP.

Base Sequence↗
PubMed1995

[A case of the brainstem tactile hallucinosis due to pontine hemorrhage].

A 53-year-old man with hypertension was admitted to our hospital, for somnolence, horizontal gaze palsy, right hemiparesis and right sensory disturbance. Brain CT scan revealed a high density area from the left lower pontine tegmentum to the left tegmentum of the lower midbrain. As he became alert, he reported visual hallucination. Two weeks after onset of stroke, he complained tactile hallucination on his right half body with sensory disturbance. After disappearance of the visual hallucination, the tactile hallucination had been persisted. This is the first report of peduncular hallucinosis with long-persisted tactile hallucination due to brainstem bleeding. Tactile hallucination was suggested to be associated with sensory disturbance and extensive destruction of the brain stem tegmentum.

Cerebral Hemorrhage↗
PubMed1995

[A case of central retinal artery occlusion followed by cerebral infarction due to internal carotid artery disease].

Reported is a 68-year-old male with left central retinal artery occlusion (CRAO) followed by cerebral infarction due to left internal carotid artery disease. He was referred to our hospital diagnosed as having left CRAO. In view of severe stenosis of ipsilateral internal carotid artery by ultrasound duplex scan, carotid endarterectomy (CEA) was indicated. While waiting for the operation, he suffered from monoparesis in his right lower limb despite the administration of antiplatelet agents. Brain MRI showed cerebral infarction in the left frontal cortex. It was suggested that artery-to-artery embolism from the lesion of the internal carotid artery might be the common mechanism of CRAO and cerebral infarction in this case. According to theses results, CEA should be considered in patients with severe stenosis of the internal carotid artery before it is too late.

Aged↗
PubMed1995

[A case of transient ischemic attacks preceded by postprandial hypotension].

A 78-year-old man with a history of total gastrectomy, myocardial infarction and hypertension was admitted to our hospital because of frequent transient attacks of consciousness disturbance, aphasia and right hemiparesis after meals. His attacks occurred one to three times a day, 30 to 60 minutes after meals and lasted 120 to 180 minutes. At the time of the attacks his blood pressure decreased, but his pulse rate did not change. Cerebral angiography demonstrated atherosclerotic occlusion of the left internal carotid artery. There was no myocardial uptake of 123I-metaiodobenzylguanidine (MIBG). These results suggest that our patient had an autonomic dysfunction. Duplex carotid ultrasonography revealed that the blood flow velocity fell about 30% at the time of the attacks. His autonomic dysfunction and major artery occlusion may have produced a hemodynamic TIA following postprandial hypotension.

Aged↗
PubMed1995

[A case of recanalized basilar artery following basilar artery occlusion due to spontaneous dissection].

An 85-year-old man who did not have any previous history of heart disease was admitted to our hospital with the chief complaints of disturbance of consciousness, tetraparesis, and bilateral Babinski signs. On admission, intravenous digital subtraction angiography (DSA) showed bilatral vertebral arteries without filling the basilar artery. On the second hospital day, MRI showed a septum in the basilar artery, and no infarctions were noted in the brain stem. He gradually improved with heparin therapy. On the tenth hospital day, intravenous DSA disclosed filling of the basilar artery. On the sixteenth hospital day, conventional cerebral angiography revealed filling of the basilar artery from the left vertebral artery, and string sign and linear shadow (intimal flap) was noted in the basilar artery. He was diagnosed to have the basilar artery dissection. The patient became ambulatory and was discharged independently. This is very rare case of the basilar artery dissection with a good prognosis.

Aged↗
PubMed1995

[Familial amyloidotic polyneuropathy (FAP) type I and the therapies].

Since autonomic dysfunction in familial amyloidotic polyneuropathy (FAP) Type I is recognized in the early stage of FAP patients and restricts their daily life, we developed various therapies for various autonomic manifestations: Loading glucose was often effective for faintness caused by hypoglycemia and erythropoietin was also good therapy for orthostatic hypotension as well as anemia found in the end stage of FAP patients. Stoma and nasal drop of L-threo-DOPS were useful to control diarrhea and orthostatic hypotension. Duplex ultrasonography of the 4 vessels revealed that reverse flow was always recognized in FAP patients with faintness, which was effectively treated by the administration of L-threo-DOPS. Orthotopic liver transplantation revealed effective therapy for autonomic dysfunction in FAP patients. Precise analysis of protein metabolism in FAP patients revealed that among apolipoproteins, only apolipoprotein AII decreased as the progression of the disease and high density lipoprotein gained the negative charge by agarose electrophoresis study. Concerning low density lipoprotein (LDL) study, only variant transthyretin in the circulation associated with LDL in FAP patients, suggesting that LDL may play an important role in the amyloid formation of FAP.

Amyloid Neuropathies↗
PubMed1995

[A case of internal carotid artery occlusion with recurrent amaurosis fugax].

Reported is the case of a 61-year-old man with a 2-year history of recurrent amaurosis fugax in the left eye attributable to left internal carotid artery occlusion. Cerebral angiography showed occlusion of origin of the left internal carotid artery, collateral pathway from the left external carotid artery to the left internal carotid artery through the left ophthalmic artery, 25% stenosis of the right internal carotid artery, and collateral pathway to left internal carotid arterial system through the Willis ring. We concluded that emboli through the left external carotid artery from the site of left internal carotid artery occlusion might cause amaurosis fugax in this case, because of the findings described below. 1. sudden onset and rapid recovery of visual loss. 2. ipsilateral occlusion of the internal carotid artery and retrograde filling of the ophthalmic artery by cerebral angiography. 3. the diminishment of attacks of amaurosis fugax by the administration of antiplatelet drug.

Blindness↗
PubMed1994

Decrease in Cu/Zn- and Mn-superoxide dismutase activities in brain and spinal cord of patients with amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is a degenerative disorder of motor neurons in the central nervous system (CNS). Mutation of the Cu/Zn-superoxide dismutase (SOD) gene on chromosome 21 has been found in some families with autosomal dominant familial ALS (FALS). We sought to determine whether there may be differences in the distribution and activity of SOD in the CNS of patients with sporadic ALS, and of control patients without neurological disorders. The frontal cortex, cerebellum, and spinal cord were obtained at autopsy on 5 patients with ALS and from 10 controls. Immunohistochemically, in the controls, the cytosols of the large pyramidal neurons of the cerebral cortex, anterior and posterior horn cells, and neurons of the nucleus thoracicus of spinal cord were stained homogeneously with anti-human Cu/Zn-SOD antibody, and in a granular manner with anti-human Mn-SOD antibody. Pia mater and epithelial cells of choroid plexus also stained well. Conversely, in the CNS of the ALS patients, most neurons were stained faintly, or not at all with both anti-Cu/Zn- and Mn-SOD antibodies, whereas the pia mater and the epithelial cells of choroid plexus stained intensely. There was no difference in total SOD activity in the entire CNS between ALS patients and controls, as determined by enzyme assay. Results suggest that, in cases of sporadic ALS, the activities of Cu/Zn- and Mn-SOD are decreased and superoxide produced within the neurons accumulates because of an insufficient elimination, leading to the development or acceleration of cell damage, ultimately producing neuronal degeneration and necrosis.

Adolescent↗
PubMed1994

Role of nitric oxide in the peripheral vessels of patients with familial amyloidotic polyneuropathy (FAP) type I.

In order to establish the generation of endothelial derived relaxating factor (EDRF) in patients with familial amyloidotic polyneuropathy (FAP) type I, the muscle temperature of the lower limb was measured with a deep portion thermometer. The temperature of the gastrocunemius muscle in FAP patients was significantly decreased compared with control subjects. In order to investigate the generation of EDRF in FAP patients, we administered NG-monomethyl-L-arginine (L-NMMA), specific inhibitor of nitric oxide, from the brachial artery and measured the changes in blood flow. Although 61.7 +/- 18.2% of the blood flow was transiently decreased after administration of L-NMMA in control subjects, FAP patients showed poor responses. In contrast, in the same way as in control subjects, significant vasodilatation was seen in FAP patients after administration of L-arginine. The urinary secreted NO2-/NO3- levels per day, which reflect the synthesis of nitric oxide in the systemic circulation, was a great deal lower in FAP patients than in control subjects. These results suggest that, although peripheral vessels can be dilated when a large amount of the substrate for NO synthase, L-arginine, is supplied, production of nitric oxide may be suppressed, and, as the result of this phenomenon, blood flow is decreased in the peripheral tissues of FAP patients in a static state.

Adult↗
PubMed1994

Role of superoxide dismutase and nitric oxide on the interaction between brain and systemic circulation during brain ischemia.

To elucidate the critical role of superoxide dismutase (SOD) and nitric oxide in brain injury and systemic circulation during brain ischemia, we performed bilateral carotid artery ligation (BCAL) on rats and evaluated the effects of NG-monomethyl-L-arginine (L-NMMA) and a long-acting SOD derivative (SMA-SOD). After administration of L-NMMA, specific inhibitor against nitric oxide synthase (NOS), most of BCAL rats died within 6 h while no BCAL rats without L-NMMA died at all. Administration of SMA-SOD exhibited no effect on the life span of BCAL rats. Magnetic resonance imaging (MRI) and microscopic analysis for the ischemic brain revealed that, although administration of L-NMMA showed no significant effect on the ischemic brain of BCAL rats, SMA-SOD effectively prevented the ischemic changes based on permeability edema in the frontal lobe. Measurement of changes in the blood flow of the ischemic brain revealed that administration of L-NMMA decreased the blood flow in the BCAL rats while no remarkable changes were seen after administration of SMA-SOD. Urinary secretion of NO2-/NO3-, the metabolites of nitric oxide, was increased by challenging BCAL, and the presence of L-NMMA or SMA-SOD diminished this elevation. Blood pressure was increased by performing BCAL to rats, and administration of L-NMMA showed further elevation of the blood pressure. On the contrary, administration of SMA-SOD decreased post-ischemic hypertension. These results suggest that SOD may play a protective role for brain ischemia by suppressing increased vascular permeability, while nitric oxide showed beneficial effect on the ischemic brain by increasing the blood flow in the ischemic brain.

Amino Acid Oxidoreductases↗