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Biomedical subjects

S Tsuji

Publications and source records attributed to S Tsuji.

At least 829 records · Page 46Linked to original sources

[Juvenile Sandhoff disease with local panatrophy--a case report].

A case of juvenile Sandhoff disease with Gowers' local panatrophy was reported. A mentally retarded 39 year-old man had been noticed to have localized skin atrophy on the right leg since 7 years of age, and was also aware of the atrophy of his right leg since his junior high school age. His parents were first cousins. He had multiple localized atrophic skin lesions on the right side of his nape, the right side of lower back, and the right thigh. These skin lesions did not show any signs of sclerosis. Although he was mentally retarded (IQ=44), the neurological examination was normal including funduscopy. Histological examination of the atrophic skin le led that the epidermal basal layer was hyperpigmented and that the dermis and subcutaneous fatty tissue were extremely atrophic. The nerves meandered across the dermis and subcutaneous tissue, and had membranous lipid storage cytosomes in the axon and Schwann cells. Biochemical studies revealed marked deficiency fo leukocyte hexosaminidase activity. Total hexosaminidase activity was decreased to 18% of normal controls, and hexosaminidase B activity was completely deficient. Other lysosomal enzymes in leukocytes were normal in activities. Both parent showed intermediate levels of leukocyte hexosaminidase A and B activities. Electron microscopy analysis of a rectal biopsy specimen showed neuronal accumulation of dense osmophilic deposits, as well as membranous cytoplasmic bodies in Meissner's plexus. On the basis of the patient's age at onset and the above findings, the patient was diagnosed to have juvenile Sandhoff disease. The local panatrophy observed in the present case could be the result of the accumulation of GM2-ganglioside in autonomic nervous system, and may well be a new clinical feature of GM2-gangliosidosis.

Adult↗

[A case of multiple sclerosis with polyradiculitis and spinal subarachnoid block].

A case of 53-year-old female with multiple sclerosis was reported. In August 1987, she suffered from weakness in her legs and urinary retention. These signs had progressed with incomplete remissions and exacerbations. In March 1988, she developed sensory loss of all modalities below C2 level, spastic paralysis of upper extremities and flaccid paraplegia of lower extremities. Electromyography showed evidence of denervation in affected muscles of all extremities and paraspinal muscles. On lumber puncture, spinal fluid pressure fell to 0 mmH2O after removal of the spinal fluid of 12 ml, and the CSF protein was 740 mg/dl, indicating subarachnoid space block. The oligoclonal band was positive. MRI showed swelling in the cervical and upper thoracic cord, and multiple lesions in the periventricular white matter in the cerebrum. We diagnosed this case as multiple sclerosis in combination with acute polyradiculitis. The spinal subarachnoid block was considered to be caused by the swelling of the spinal cord.

Acute Disease↗

[A case of recurrent multiple cerebral infarctions with protein C abnormality].

A 38-year-old man of recurrent episodes of multiple cerebral infarctions with protein C abnormality was reported. He had six episodes of visual disturbance within a year. The neurological examination revealed bilateral visual loss and impaired visual field except for small central area. However, there were no abnormalities in the deep tendon reflexes and muscle strength of all extremities and cerebellar examinations. CT scan revealed multiple low density areas in the bilateral posterior lobes, and cerebral angiography demonstrated the severe stenosis of the ganglionic portion and the supraclinoid portion of the right internal carotid artery. Plasma levels of antithrombin-III activity, plasminogen activity, protein C activity, protein C antigen and protein S antigen were 105, 106, 28, 166 and 120%, respectively. Because only protein C activity decreased markedly, a diagnosis of protein C abnormality was established. We started warfarin therapy under the administration of concentrates of factor IX complex, since then there has been no cerebral accident.

Adult↗

Double tracer autoradiographic method for sequential evaluation of regional cerebral perfusion.

A new double tracer autoradiographic method for the sequential evaluation of altered regional cerebral perfusion in the same animal is presented. This method is based on the sequential injection of two tracers, 99mTc-hexamethylpropyleneamine oxime and N-isopropyl-(125I)p-iodoamphetamine. This method is validated in the assessment of brovincamine effects on regional cerebral perfusion in an experimental model of chronic brain ischemia in the rat. The drug enhanced perfusion recovery in low-flow areas, selectively in surrounding areas of infarction. The results suggest that this technique is of potential use in the study of neuropharmacological effects applied during the experiment.

Amphetamines↗

[Molecular genetic approaches to neurologic diseases].

Recent advances of molecular biology has brought neurologists new and powerful approaches for the studies of neurologic diseases. The molecular genetic approaches have been successfully applied for the elucidation of molecular mechanisms of Gaucher disease, a sphingolipid storage disease. First a full length cDNA clone for glucocerebrosidase, the missing enzyme in Gaucher disease, was isolated, which enabled us to isolate the mutant as well as normal glucocerebrosidase genes. With detailed nucleotide sequence analysis of mutant genes, we have discovered two single base mutations within exons of glucocerebrosidase genes, one in type 2 and the other in type 1 Gaucher disease. Screening of the mutations among three phenotypes of Gaucher disease, it has been shown that there is a good correlation of occurrence of each mutation with the phenotypes. Moreover, we have shown that we have successfully restored glucocerebrosidase activity in Gaucher fibroblasts by retrovirus-mediated transfer of a normal human glucocerebrosidase cDNA. The results indicate that current molecular biology techniques can be utilized not only for the study of molecular mechanisms of neurologic diseases but also for the development of treatment of devastating neurologic diseases.

DNA Mutational Analysis↗

[Motor and cortical sensory evoked potentials by magnetic stimulation].

Motor evoked potentials (MEP) by magnetic stimulation on the scalp and the spinous processes of the 7th cervical (C 7) and 5th lumbar (L 5) vertebrae were studied in 20 normal subjects and 10 patients with the pyramidal tract lesions. The magnetic stimulator composed of two flat helical coils with mean inner diameters of 12.0 and 2.2 cm. The evoked muscle action potentials were recorded from the thenar muscle in the hand and abductor hallucis muscle in the leg. The mean peak latencies of MEP recorded from the thenar muscle were 22.1 +/- 1.7 and 12.8 +/- 0.9 msec at the stimulations on the scalp and C 7, respectively. The central motor conduction time (CMCT) between the cortex and C 7 was 9.1 +/- 1.1 msec. On the other hand, the peak latencies of MEP were 41.0 +/- 3.2 and 21.6 +/- 2.3 msec at the stimulations on the scalp and L 5, respectively. CMCT between the cortex and L 5 was 19.3 +/- 2.3 msec. The patients with pyramidal tract involvements showed delayed peak latencies or absent MEP. The cortical somatosensory evoked potentials (SEP) by the noninvasive magnetic stimulation on the levels of Th 10, Th 12 and L 5 spines, gluteus and ankle were studied in 20 normal subjects and 7 patients with neurological diseases. Cortical components P 2 and N 2 were recorded clearly in all normal subjects.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Roles of intrinsic prostaglandins and leukotrienes in gastric mucosal damage].

While gastric mucosa contains prostaglandins and leukotrienes, the roles of these substances in the gastric mucosal protection and damage are not clarified. Using AA-861, a specific 5-lipoxygenase inhibitor, and indomethacin, a cyclooxygenase inhibitor, we investigated the roles of intrinsic prostaglandins and leukotrienes in the gastric circulatory change and the development of gastric lesion induced by ethanol. Rats were fasted for 48 hours and allowed free access to water. After light ether anesthesia, rats were orally given AA-861 (gift from Takeda Chem. Industry; 2.5, 10, 40 or 640 mg/kg), indomethacin (20 mg/kg), and the both or AA-861 (40 mg/kg) and indomethacin (20 mg/kg). Thirty min after the treatment, 2 ml of ethanol (30, 40, 70, 99.5%) was orally administered. Sixty min later, the rats were killed to measure the area of mucosal lesions. The gastric mucosa was damaged by ethanol (greater than or equal to 40%) in a dose-dependent manner. The treatment with AA-861 prohibited the gastric mucosal damage. However, the treatment with indomethacin did not show a significant influence to the mucosal damage. Combined treatment of AA-861 and indomethacin yielded no significant difference from that of single treatment with AA-861 in acute gastric mucosal ulceration. The mucosal blood flow velocity was monitored by laser doppler velocimetry before and after the ethanol administration in the groups treated with vehicle, AA-861 or indomethacin. The blood flow velocity decreased after the administration of 40% ethanol in the group treated with indomethacin, and in a group treated with vehicle.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗

[Somatosensory evoked potentials in patients with cervical spondylotic myelopathy].

Somatosensory evoked potentials (SEPs) to posterior tibial nerve (PTN) and median nerve (MN) stimulations were recorded in 30 patients with cervical spondylotic myelopathy. Measurements performed include N20-P2 interpeak latency (IPL) for PTN-SEPs, EP-N13 IPL and EP-N20 IPL for MN-SEPs. Limits of normal IPL were defined by the mean + 3S.D. of the normal control group. PTN-SEPs was more sensitive (with 73.3% abnormal) than MN-SEPs (with 33.3% abnormal) and strongly correlated with the clinical signs of posterior column, but not with those of anterolateral column indicated by superficial sensory disturbances and spasticity of lower limbs. Severities of cord compression (sagital diameter/transverse diameter ratio) calculated from the picture of metrizamide CT were not correlated with SEPs findings and clinical signs. In patients with cervical myelopathy, SEPs, especially PTN-SEPs, were thought to be very useful examination.

Adult↗

[CT findings of bone metastasis].

Computed tomograms of 150 cases with bone metastasis of various malignant neoplastic diseases are reviewed. According to CT findings bone metastasis is classified into six types, namely osteoblastic, mixed, osteolytic with sclerotic rim, osteolytic with sclerotic center, osteolytic, and infiltrative. Presumably many bone metastases show initially osteoblastic type on CT, and change progressively to mixed type or osteolytic type. If chemotherapy or radiotherapy is effective for bone metastasis, CT demonstrates changes of inverse direction. In detection of bone metastasis CT is superior to radionuclide bone scan. To find out the bone metastasis CT of cancer patients must be evaluated with bone window.

Adult↗

[A case of HTLV-I associated myelopathy (HAM) complicated by mononeuritis multiplex].

A 42-year-old woman with progressive myelopathy and mononeuritis multiplex is reported. The neurological examination on admission revealed hyperreflexia of the four extremities with pathological reflexes and moderate muscle weakness of the lower extremities with spasticity. Sensory disturbance was distributed on the areas of the bilateral lateral cutaneous femoral, the superficial peroneal and sural nerves. The antibody to HTLV-I in the serum and cerebrospinal fluid was more than 8192X and 512X, respectively. No sensory potential was recorded in the sensory conduction study of bilateral lateral femoral cutaneous nerves. Corticosteroid therapy caused a marked improvement of the sensory and urinary disturbances and had a slight effect on the spastic gait. Our nerve conduction study found small sensory potentials with normal conduction velocities in the bilateral lateral femoral cutaneous nerves. These results suggested the presence of an axonal degeneration in the peripheral nerves in this case. There have been no reports in the literature regarding a case of HAM with mononeuritis multiplex.

Adult↗

Phasic coronary artery flow velocity determined by Doppler flowmeter catheter in aortic stenosis and aortic regurgitation.

Phasic coronary artery flow velocity was recorded in 14 patients with aortic regurgitation (AR), 4 with aortic stenosis, 61 with other heart diseases and in 2 normal subjects by means of a bidirectional Doppler flowmeter catheter. The normal pattern of the phasic coronary artery flow velocity was characterized by a small forward flow during systole (S wave) and a large forward flow during diastole (D wave). The phasic coronary artery flow velocity in patients with AR showed increased S wave and decreased D wave. The area under the S-wave curve divided by the area under the D-wave curve (S/D ratio) in patients with AR increased (left coronary artery flow velocity 0.66 +/- 0.39, p less than 0.05; right coronary flow velocity 0.79 +/- 0.36, p less than 0.01) as compared with the S/D ratio in patients with other heart diseases (left coronary flow velocity 0.32 +/- 0.12; right coronary artery flow velocity 0.38 +/- 0.17). There was a tendency toward a relative positive correlation between S/D ratio values and AR cineangiographic grades. Decreased S/D ratios were observed in 4 patients with aortic stenosis. It is believed that no reports exist on phasic coronary flow velocity recorded in conscious patients who had aortic valve disease.

Adult↗

Somatosensory potentials evoked by magnetic stimulation of lumbar roots, cauda equina, and leg nerves.

Cortical somatosensory evoked potentials (SEPs) were studied by noninvasive magnetic stimulation at T-10, T-12, and L-5 vertebral levels and in mid-gluteus muscle and ankle in 27 normal subjects and 7 patients with neurological diseases. Cortical components P2 and N2 were recorded in all normal subjects. The mean peak latencies of P2 were 20.3 +/- 0.9 (standard deviation), 21.1 +/- 1.2, 23.5 +/- 1.4, 27.9 +/- 2.0, and 38.1 +/- 1.8 msec at the T-10, T-12, L-5, midgluteal and ankle sites of stimulation, respectively. No substantial difference in morphology of P2 and N2 was seen between magnetic and electrical stimulation at T-12. Amplitudes of P2 and N2 were maximal after magnetic stimulation at motor threshold. P2 and N2 may originate from the sensory cortex. P2 and N2 evoked by T-10 and T-12 stimulation were normal in peak latency and morphology in patients with polyneuropathy or polyradiculoneuropathy. Peak latencies of P2 and N2 evoked by T-10 and T-12 stimulation were significantly delayed in patients with myelopathy. The patients with radiculopathy showed a delayed peak latency and conduction time of P2 evoked by L-5 stimulation. Magnetic stimulation of spinal root is able to detect lesions of spinal cord noninvasively.

Adolescent↗

Muscarinic regulation of two ionic currents in the bullfrog sympathetic neurone.

The mechanism of slow muscarinic excitation in bullfrog sympathetic ganglia was studied using a single-electrode voltage clamp technique. The membrane current induced by muscarine (0.01-30 microM: Imus) consisted of a voltage-dependent component (dIM) induced by the inhibition of K+-current (known as IM), a voltage-independent cation-selective current (ID), or both. In the last case, the magnitude of either component varied in different cells. Generation of both dIM and ID were equally suppressed by pirenzepine with a dissociation constant (Ki) of 30 nM, while they were inhibited less by AF-DX116 with a Ki of 600 nM. Kd values for muscarinic induction of dIM and ID were 0.35 and 5 microM respectively. This difference was also seen even after reducing the receptor population by pretreatment with propylbenzilylcholine mustard. ID was enhanced after blockade of dIM by Ba2+ (4 mM), with a little change in Kd. This effect was larger when the control ID was smaller. These results suggest that a single subtype (M1) of muscarinic receptor generates both the dIM and ID with different efficacies and that Ba2+, known as a blocker of IM (accordingly of a high-efficacy current, dIM), potentiates a low-efficacy current (ID).

Animals↗

Quantitative assessment of cerebral blood flow using technetium-99m-hexamethyl-propyleneamine oxime: Part I, Design of a mathematical model.

To design a mathematical model for quantifying cerebral blood flow using 99mTc-hexamethyl-propyleneamine oxime (HM-PAO), basic studies were performed in animals and human volunteers. Microautoradiography revealed that HM-PAO crossed the blood-brain barrier. Thin layer chromatographic studies demonstrated the rapid disappearance of free HM-PAO in the brain tissue. Back diffusion from brain to blood was found negligible. From these observations, the familiar microsphere model was employed in the measurements of blood flow with HM-PAO. This, however, resulted in much lower flow values than simultaneously obtained values with the labeled microspheres. This underestimation was ascribed to the high affinity of HM-PAO to blood cells and serum protein. Taking the binding of HM-PAO to blood components into consideration, the following model equation was designed for quantifying cerebral blood flow: Ce(t) = Ca(t)-kCa(t)*exp(-kt), Cb(T) = F integral of T0 Ce(t)dt, where Ce and Ca are the free HM-PAO concentration in the intravascular space and the arterial whole-blood concentration of HM-PAO, respectively, as a function of time (t), Cb is the brain activity concentration, k is the rate constant for the binding of HM-PAO to the blood components, F is the blood flow value, T is time of measurement, and * denotes the operation of convolution. In clinical studies, Ca(t) and Cb(T) are obtainable from a dynamic single photon emission computerized tomographic study of the brain and multiple arterial blood sampling, respectively. The values for F and k can be estimated using a nonlinear least squares fitting method.

Animals↗

Combined features of myasthenia gravis and Eaton-Lambert syndrome: anti-ganglioside antibodies in serum.

We studied 2 patients with Eaton-Lambert syndrome showing some features of myasthenia gravis. Among 9 different glycolipid antigens examined, serum titers of IgG antibodies to GT1b, GD1a, and sialylparagloboside were increased. Those antibodies may be involved in the pathogenesis of the presynaptic neuromuscular block that was presumed to be present in these patients.

Autoantibodies↗

Determination of flow and rate constants in a kinetic model of [99mTc]-hexamethyl-propylene amine oxime in the human brain.

The values for flow and rate constants for a kinetic model of [99mTc]-hexamethylpropyleneamine oxime (HM-PAO) distribution in the human brain were determined. The single-pass extraction ratio of HM-PAO was also determined in the rat brain by the indicator diffusion method; a value of 0.90 +/- 0.02 (mean +/- SEM, n = 5) was obtained. Time course data of brain activity and arterial blood activity of the tracer were fitted to a four compartment model: Values of blood flow and the first-order rate constants for backdiffusion of the diffusible tracer from brain to blood (k2), conversion of the lipophilic tracer to the hydrophilic one in brain (k3), and conversion of the diffusible tracer to the nondiffusible one in blood (k5) were determined. Conversion of hydrophilic tracer back to a lipophilic form in both blood and brain was assumed to be negligible during the course of the experiment. The values obtained for blood flow, k2, and k3 were, respectively, 0.40 +/- 0.03 ml/g/min, 0.38 +/- 0.04 min-1, and 0.92 +/- 0.05 min-1 in the gray matter (n = 4), and 0.23 +/- 0.01 ml/g/min, 0.17 +/- 0.01 min-1, and 1.01 +/- 0.05 min-1 in the white matter (n = 2) in patients with cerebrovascular disorder. The k5 value was 1.14 +/- 0.06 min-1 (n = 4). These experimentally determined values agree well with the theoretical ones previously reported by Lassen et al. The results suggest the relative constancy of the k3 and k5 values and the more prominent initial backdiffusion of the lipophilic HM-PAO from brain to blood in high flow regions compared to low flow regions.

Animals↗