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Biomedical subjects

S Kuwabara

Publications and source records attributed to S Kuwabara.

At least 109 records · Page 6Linked to original sources

[A case of Isaacs' syndrome preceding the recurrence of malignant thymoma--generating site of ectopic activity and therapy].

We described a-44-year old male patient with Isaacs' syndrome occurring 8 months before the recurrence of malignant thymoma. Electrophysiological examination suggested that spontaneous muscle activities were generated at the distal terminals of the motor nerves. Double filtration plasmapheresis alleviated the symptoms of neuromyotonia for a short term. Administration of valproic acid had more prolonged effects lasting for 2 months until his death from relapsed thymoma. Our results support that autoimmune mechanisms, especially humoral factors, are playing an important role in the pathogenesis of Isaacs' syndrome and that anti-convulsants therapy is important.

Anticonvulsants↗

Partial deficiency of phosphoglycerate mutase with diabetic polyneuropathy: the first Japanese patient.

We report here findings in a 51-year-old Japanese man with non-insulin-dependent diabetes mellitus who complained of exercise-induced cramps. Muscle biopsy showed scattered regenerating fibers, small angular fibers and increased PAS positive particles. Electron microscopic examination revealed an abnormal accumulation of glycogen particles in subsarcolenmmal areas and between myofibrils while chemical studies showed an increased glycogen concentration and decreased phosphoglycerate mutase (PGAM), 46.9% of the normal mean value. Thus, partial PGAM deficiency, insulin resistance and mild diabetic sensory-motor polyneuropathy can induce severe cramps.

Biopsy↗

[High activity of antistreptolysin-O in a case of IgM myeloma].

A 56-year-old man was admitted to our hospital in November, 1991 because of hyperproteinemia and anemia. Total protein showed 12 g/dl and serum immunoglobulins were as follows; IgG 974 mg/dl, IgA 142 mg/dl, IgM 9270 mg/dl. M-component was identified as IgM-kappa with immunoelectrophoresis and serum viscosity indicated 6.9. Although the patient had no history of severe streptococcal infection, his serum showed very high activity of ASLO (6890 IU/ml). Bence Jones protein was detected in the urine and determined to be of kappa-type. Plasma cells occupied 43% of bone marrow nucleated cells and their cytoplasms were stained with FITC-labeled anti-IgM and anti-kappa antibodies. X-ray examination disclosed punched out lesions in the skull. Consequently, the patient was diagnosed as having IgM-myeloma. At first, VCAP regimens were given after plasmapheresis, but had no effect. Therefore, the patient was treated with CHOP protocol and the serum IgM decreased in amount and the proportion of bone marrow myeloma cells got down to 17%. ASLO titer also decreased in parallel with IgM. These findings suggest that IgM in this case had ASLO activity.

Antistreptolysin↗

[Double filtration and immunoadsorption plasmapheresis in Guillain-Barré syndrome].

We studied the effect of double filtration and immunoadsorption plasmapheresis in Guillain-Barré syndrome retrospectively in 70 patients. Thirty three patients had plasmapheresis, of whom 23 had double filtration and 10 immunoadsorption. The rest of 37 patients consisted of a control group. Clinical disability was evaluated using the functional grading scale by Hughes. In patients having a maximum disability of grade 4 and 5, the double filtration plasmapheresis group showed statistically significant improvement at 3 months after the treatment. There was no significant difference in prognoses between the immunoadsorption plasmapheresis and control groups. In mildly-illed patients having grade 2 and 3, the prognoses were not different between the three groups. In Guillain-Barré syndrome, double filtration plasmapheresis, as well as well-established plasma exchange, had benefit for severely-illed patients.

Adult↗

[A case of relapsing demyelinating multiple mononeuropathy with multifocal nerve enlargement].

We report a 23-year-old woman, who had a relapsing-remitting multiple mononeuropathy with multifocal nerve enlargement. The patient was characterized by asymmetrical, marked enlargement of multiple nerves in the arms (median and ulnar nerves) and neck (accessory nerve) bilaterally. Sequential nerve conduction studies revealed persistent demyelinative abnormalities, especially across the segment of nerve thickening. The MRI of the proximal arm confirmed the markedly enlarged median and ulnar nerves which showed high signal intensity on T2-weighted images and partial enhancement after administration of gadolinium. We consider that the patient had the clinical features of "multifocal pseudohypertrophic neuropathy" described by Adams et al, and that chronic demyelination and inflammation associated with impairment of the blood-nerve barrier might be underlying mechanisms.

Adult↗

Associated movement as a sequel to thoracotomy: aberrant regeneration to the latissimus dorsi muscle.

We describe two patients with unusual associated movements as a sequel to thoracotomy. The two patients developed involuntary twitchings of the latissimus dorsi, which were induced by contractions of the adjacent muscles, the serratus anterior muscle in patient 1 and the external intercostal muscle in patient 2, after lobectomy for lung cancer. Electrophysiological and radiological studies showed that aberrant regeneration from the adjacent nerves to the denervated latissimus dorsi could be responsible for the associated movements.

Electromyography↗

Development of non-invasive fetal DNA diagnosis from maternal blood.

Several attempts have been made to detect and retrieve fetal nucleated cells including nucleated erythrocytes (NRBCs), leukocytes, and trophoblasts in maternal blood. We have recently developed a new method for non-invasive fetal DNA diagnosis from maternal blood. Peripheral blood granulocytes including NRBCs were isolated by a discontinuous density gradient method using Percoll (Pharmasia). NRBCs were found and retrieved at a single cell level using a micromanipulator under a microscope. To determine whether the origin of the NRBCs was maternal or fetal, the NRBCs were analysed by polymerase chain reaction (PCR) amplification to determine the presence of a Y-chromosome-specific repeat sequence in mothers carrying male fetuses. We were successful in predicting fetal sex accurately in 10 out of 11 samples taken from maternal blood. This new technique opens up fetal DNA diagnosis from maternal blood during the first trimester of pregnancy to the whole population because there is no risk to the fetus or the mother.

Cell Nucleus↗

Increased CSF C4d in demyelinating neuropathy indicates the radicular involvement.

Plasma and cerebrospinal fluid (CSF) levels of C4d and the circulating immune complex (CIC) to C1q were measured in 12 patients with chronic inflammatory demyelinating polyneuropathy and Guillain-Barré syndrome. CSF C4d values more than 2 SD from the mean of 8 cervical spongylosis cases were demonstrated in the patients with proximal demyelination. The CSF C4d probably originated from both intrathecal synthesis and the systemic circulation. CSF levels of C4d may serve as a sensitive indicator for the radicular involvement in demyelinating polyneuropathy.

Adult↗

[Clinical course of a HIV-seropositive patient with thrombocytopenia and hypergammaglobulinemia who was initially screened by annual complete physical examination].

A 57-year-old man was admitted because of hypergammaglobulinemia which was initially pointed out by annual complete physical examination. No significant abnormal findings were observed except polyclonal hypergammaglobulinemia at that time (IgG; 2,662 mg/dl, IgA; 422 mg/dl). Seven months later, he has progressed to show thrombocytopenia. The laboratory data showing the reduction of peripheral CD 4-positive T cells (CD 4; 0.2 x 10(9)/l, CD 4/8 ratio; 0.19) and positive serum HIV antibody revealed that his hypergammaglobulinemia and thrombocytopenia were resulted from HIV infection. The peripheral platelet count decreased to 28 x 10(9)/l at minimal point, however, it recovered to 160 x 10(9)/l within 7 months without treatment. His good performance status has been still maintained over 4 years. His whole clinical course suggests that there is no significant correlation between peripheral platelet counts, serum gammaglobulin levels or the amounts of PAIgG in this case. This case proposes that we should also consider a possibility of HIV infection for the patients showing hypergammaglobulinemia.

HIV Seropositivity↗

Changes of motor evoked potentials in global and focal ischemic models of cats.

In order to evaluate the significance of motor evoked potentials (MEPs) in central nervous system monitoring, the authors conducted two sets of experiments using feline ischemic models. Twenty-three adult mongrel cats were divided into two groups: global (n = 9) and focal ischemic (n = 14) groups. In the case of global ischemia, which was induced by hypovolemic hypotension due to blood letting, deterioration of the D wave began when the mean arterial blood pressure (MABP) approached 45 mmHg, and regional cerebral blood flow (rCBF) dropped to 40% of control. Complete disappearance of the D wave was observed below 30 mmHg in MABP and 20% of control in rCBF. In the case of focal ischemia, which was induced by transorbital occlusion of the middle cerebral artery, the percentages of rCBF at which the D wave disappeared ranged from 9 to 20%. Changes in the amplitude of the D wave--an increase and following decrease--preceded the prolongation of its latency. In contrast with the D wave I waves were too easily affected by ischemia. Moreover, rCBF at the point of disappearance of the I wave varied greatly. In conclusion, the D wave is stable in mild ischemia and is a reliable indicator of critically profound ischemia (%rCBF < 40%). Monitoring the D wave of MEPs seems to be a useful method for avoiding the deterioration of motor function by ischemic insult.

Animals↗

Purification and properties of peditoxin and the structure of its prosthetic group, pedoxin, from the sea urchin Toxopneustes pileolus (Lamarck).

A protein toxin, termed peditoxin, containing an active prosthetic group was isolated and purified from the globiferous pedicellariae of the sea urchin Toxopneustes pileolus (Lamarck). The prosthetic group, called pedoxin, is a small molecular mass substance (206 daltons) with an empirical formula of C10H10N2O3 and is comprised of a heterocyclic lactone structure formed from pyridoxal and glycine. Administered subcutaneously or intramuscularly to mice in sublethal doses, pedoxin markedly reduced basal body temperature and led to sedation and anesthetic coma accompanied by muscular relaxation. At higher doses, it leads to convulsion and death (LD50 200 mg/kg). The apoprotein, a cytochrome b-like heme protein called pedin (molecular mass, 10,000 daltons) is itself not toxic, but the purified holoprotein is extremely toxic, causing anaphylaxis-like shock and death in experimental animals at low doses (LD50 70 micrograms/kg). Small amounts of the prosthetic group added to holoprotein preparations caused the toxicity to be greatly enhanced, suggesting that holoprotein preparations also contain apoprotein capable of being activated by the low molecular weight toxin. The structure of pedoxin was determined to be 3-hydroxy-2-methyl-5-methoxy-4-pyridineformyl-glycyliden ester and was confirmed by total synthesis.

Amino Acid Sequence↗

Comparison of three-dimensional phase-contrast magnetic resonance angiography with three-dimensional time-of-flight magnetic resonance angiography in cerebral aneurysms.

Magnetic resonance angiography (MRA) can be divided into two types: namely, the phase-contrast (PC) method and the time-of-flight (TOF) method. The purpose of this study is to determine which method is better suited to demonstrate cerebral aneurysms. In a total of 27 cerebral aneurysms confirmed by cerebral angiography, both three-dimensional (3-D) PC and 3-D TOF methods were performed, and the corresponding findings were compared with conventional cerebral angiographic findings. Aneurysm visualization was grouped into three categories: "good" when clearly distinguishable, "fair" if distinguishable when reconstructed, and "poor" if completely undistinguishable. All MRAs were independently interpreted by two neurosurgeons in our institute. Sensitivity was 92.6% when MRA was performed with the TOF method, whereas it was 70.4% when performed with the PC method. In conclusion, the TOF method was more useful for demonstrating the aneurysms than the PC method due to its higher spatial resolution and shorter imaging time.

Adult↗

[Excitability of cervical lower motoneuron in juvenile muscular atrophy of the distal upper limb].

We investigated excitability of cervical lower motoneurons in 18 patients with juvenile muscular atrophy of the distal upper limb (Hirayama's disease), by F wave analysis and electrophysiological estimation of the number of motor units in the abductor pollicis brevis muscles of the affected side. In all the 18 patients, F wave persistencies and numbers of motor units were decreased, whereas amplitudes of single motor unit potentials were increased, compared to those of age-matched normal controls. Patients in a progressive phase showed markedly decreased F wave persistencies, overwhelming the degree of decreases in numbers of motor units. In half of them, F waves were significantly increased in number during neck flexion. On the other hand, patients treated with neck brace and patients having stabilized symptoms with longer duration showed F wave persistence reduction corresponded to the degree of the motor unit loss, and no changes in F wave persistencies in a posture of neck flexion. These results suggest that the disorder is characterized by decreased excitabilities of survived cervical anterior horn cells, as well as denervation and reinnervation, presumably resulting from local compression or circulatory failure by anterior shift of the posterior dural wall during neck flexion.

Adult↗

[Enhanced regeneration of terminal axons after hyperbaric oxygen therapy in a patient resembling progressive postpoliomyelitis muscular atrophy].

We found an electromyographical proof of reconstruction of the motor nerve terminals following hyperbaric oxygen therapy. A 38-year-old man who had been partially recovered for thirty four months from acute onset paraplegia following a gastrointestinal infection developed progressive muscular atrophy and weakness of the lower limbs, and was first admitted to our hospital. Cerebrospinal fluid examination was normal and nerve conduction studies showed small compound muscle action potentials without an evidence of segmental demyelination. There were ample fibrillation potentials on electromyography. Single fiber electromyography (SFEMG) showed increased fiber density, abnormal jitter and blockings without neurogenic jitter, which were similar to findings in post-poliomyelitis syndrome. He was treated by hyperbaric oxygen consisting of two hour exposures to pressures of two atmospheres breathing 100% oxygen. These exposures continued for a month daily, and thereafter once a week for one year. Clinical improvement of the weakness and a decrease in amount of fibrillation potentials occurred on and after a month after treatment. We found significant changes on SFEMG a year later. There were increased fiber densities and decreased mean values of consecutive differences. These changes indicate diminished degeneration and enhanced regeneration of the terminal axons. We think that hyperbaric oxygen has a beneficial effect on oxygen metabolism of remaining motoneurons which may not be able to maintain excessive metabolic demands of all their sprouting axons.

Adult↗

Hyperaemia prior to acute cerebral swelling in severe head injuries: the role of transcranial Doppler monitoring.

Acute cerebrovascular congestion after a closed head injury is significantly related to intracranial hypertension. As an indirect method of cerebral blood flow measurement; transcranial doppler sonography (TCD) provides a rapid and noninvasive assessment of cerebral haemodynamics, including hyperaemic conditions. TCD examinations was serially performed in 35 patients with severe head injury with intact cerebral circulation; i.e. the mean flow velocity (MFV) patterns of the middle cerebral artery (MCA) did not show signs of cerebral circulatory arrest such as systolic spike, to and fro, or no flow. The results showed that the MFV of the MCAs and ipsilateral extracranial internal carotid arteries (ICAs) in 9 of these patients increased sharply and pulsatility index (PI) decreased during 48-96 hours after the injury. This was soon followed by patterns of high intracranial resistance, consistent with elevated intracranial pressure (ICP) in monitored patients and acute brain swelling on repeated computed tomographic (CT) scans. The correlation between increased MFVs, decreased PIs, and cerebral haemodynamic changes leading to acute brain swelling is discussed. The number of patients who ended with severe disability, vegetative state, or death was 66% in this group of 9 patients, compared to only 34% for the 35 patients overall with severe head injury. Though the morbidity and mortality rates largely depend on the primary injury, the presence of acute cerebral swelling aggravate the grave course in these patients. And the ability of TCD to monitor the hyperaemic state prior to oedema should lead us to adjust the therapy in order to minimize the secondary insult related to intracranial hypertension.

Adult↗

Cerebral circulation in moyamoya disease: a clinical study using transcranial Doppler sonography.

Transcranial Doppler sonography was performed on eight patients diagnosed as Moyamoya disease. Angiographically, the patients-four adults (mean age 42) and four children (mean age 7.7)-underwent a complete six- or five-vessel angiographic study. The results showed the following: (1) Despite the presence of stenosis, all middle cerebral arteries showed very low-flow velocity compared to their ipsilateral distal internal carotid arteries. In adult cases, the difference was very significant (p < 0.02). (2) Relatively high-flow velocity was observed in the posterior cerebral arteries of children, and in the ophthalmic arteries of adult cases. (3) In several occasions, very low-flow velocity values were still detected despite the fact that with angiography, the respective arterial segments were hardly opacified. The relation and discrepancy between these results and the angiographic findings, and the potential application of transcranial doppler in assessing and grading the severity of moyamoya disease are discussed.

Adolescent↗