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Biomedical subjects

S Shoji

Publications and source records attributed to S Shoji.

At least 199 records · Page 11Linked to original sources

[Marked clinical improvement by plasmapheresis in a patient with stiff-man syndrome: a case with a negative anti-GAD antibody].

We described a 56-year-old man with stiff-man syndrome, who was markedly improved after plasmapheresis therapy. He had a 12-year history of progressive painful stiffness of his back and limbs, muscle cramps and difficulty in walking. He had been taking oral diazepam and prednisolone. On examination the abdominal and paraspinal muscles and limbs were continuously contracting, confirmed by surface and needle electromyography. Antibodies against glutamic acid decarboxylase (GAD) and pancreatic islet cells in the serum were negative, but antinuclear antibody and anti-smooth-muscle antibody were present. The patient underwent a course of 4 double filtration plasma exchanges of 3,000 ml each in an 8-day period. Plasmapheresis resulted in marked clinical improvement. The disappearance of muscular cramps and a reduction of stiffness occurred within 24 hours after the first plasmapheresis, and he was able to walk unassisted. The patient's subjective improvement continued over 4 months after the plasma exchange. This case provides additional evidence of the autoimmune mechanism of stiff-man syndrome. Plasmapheresis is one choice in the management for stiff-man syndrome.

Antibodies, Antinuclear↗

[A case of HLA-DR2, DQw1 negative post-traumatic narcolepsy].

We reported a case of a 24-year-old man who had frequent sleep attacks beginning 4 years after a head trauma. He showed frequent episodes of excessive daytime sleepiness and cataplexy which were triggered by emotional excitement. He also complained of sleep paralysis and hypnagogic hallucination. An overnight polysomnography revealed the sleep onset REM stage as typically observed in narcoleptic patients. The HLA typing was negative for DR2 and DQw1. He was diagnosed as having HLA-DR2 and DQw1 negative-post-traumatic narcolepsy. Peroral pemoline suppressed excessive daytime sleepiness, sleep paralysis and hypnagogic hallucination with dramatic relief of cataplectic attacks by the addition of imipramine. It has been reported that more than 90% of narcoleptic patients are HLA-DR2 and-DQw1 positive. About 10% of the narcolepsy patients were regarded as symptomatic due to brain tumors, cerebrovascular disorders, head trauma, multiple sclerosis, encephalitis and so on, which mainly affect the brainstem or diencephalon. Thus far, narcolepsy is considered to develop depending on both the genetic background including HLA types, and exogenous factors. According to reported cases with narcolepsy, sporadic cases were HLA-DR2 positive even more frequently than familial cases. To date, however, there have been only three previous reports of a symptomatic narcolepsy patient without association of HLA-DR2 and DQw1. In conclusion, the present report suggests that typical symptomatic narcolepsy could be HLA-DR2 or DQw1 negative.

Adult↗

APP717 missense mutation affects the ratio of amyloid beta protein species (A beta 1-42/43 and a beta 1-40) in familial Alzheimer's disease brain.

We have biochemically purified A beta from brains of two unrelated familial Alzheimer's disease (FAD) pedigrees with the APP717 mutation (Val-->Ile) and from two sporadic Alzheimer's disease (AD) brains and characterized them by means of mass spectrometry and enzyme-linked immunosorbent assay. We observed two types of amyloid beta protein (A beta), the short-tail form (A beta 1-40) and the long-tail form (A beta 1-42/43), in sporadic AD and FAD brains, and found that the ratio of the long-tail form of A beta (A beta 1-42/43) to total A beta was increased in FAD brains. These in vivo results were confirmed in vitro using cultured cells transfected with three kinds of APP cDNAs bearing the APP717 mutations (Val-->Ile, Gly, or Phe). Taken together with the hypothesis that A beta 1-42/43 functions as a "seed" that increases the kinetics of amyloid fibril formation (Jarrett, J. T., and Lansbury, P. T., Jr. (1993) Cell 73, 1055-1058), we conclude that the APP717 missense mutation does not create new A beta species but promotes the increased accumulation of A beta 1-42/43 in the brain, which results in the enhancement of amyloid fibril formation from soluble A beta. These findings provide a causal relationship between this FAD genotype and the pathological phenotype of A beta deposition and senile plaque formation.

Adult↗

Biochemical evidence for the long-tail form (A beta 1-42/43) of amyloid beta protein as a seed molecule in cerebral deposits of Alzheimer's disease.

We measured the amounts of total A beta, A beta 1-40 and A beta 1-42/43 in brain tissues using a newly developed ELISA assay and found that the amounts of insoluble A beta 1-42/43 and insoluble A beta 1-40 were linearly related to the amount of A beta deposits or total insoluble A beta at their lower and higher concentrations, respectively. In an experiment to characterize the A beta species in brain homogenates with buffered saline, we unexpectedly detected soluble A beta which was derived from the insoluble amyloid deposits in brain tissue, indicating reversible depolymerization of A beta from insoluble amyloid deposits. To confirm this finding, we performed 5 consecutive washes of insoluble precipitates of AD brains with buffered saline. Both species of A beta were found in all 5 supernatant fractions and their amounts were gradually decreased. The ratio of A beta 1-42/43 to A beta 1-40 was increased with the numbers of washes, indicating that A beta 1-40 existed in an exposed manner as compared to A beta 1-42/43. Thus the present finding is the first biochemical evidence that A beta 1-40 was the predominant species involved in the reversible exchanging reaction on seeding A beta 1-42/43 between the soluble and the insoluble forms (amyloid fibrils).

Alzheimer Disease↗

Thiamine disulfide as a potent inhibitor of human immunodeficiency virus (type-1) production.

Thiol and disulfide compounds were tested as an anti-HIV drug against transactivator (Tat)-mediated transactivation of HIV-1. Of all the compounds tested, thiamine disulfide, alpha-lipoic acid, and N-acetycysteine significantly depressed HIV-1 Tat activity. Thiamine disulfide alone in these compounds possessing anti-HIV-Tat activity markedly inhibited production of progeny HIV-1 in acute and chronic HIV-1-infected CEM at nontoxic concentrations of 500-1000 microM. Thiamine disulfide (500 microM) blocked 99.7% of HIV-1 production after 96 hr culture in acute HIV-1 (LAV-1) infection (m.o.i. = 0.002), whereas it inhibited 90-98% of HIV-1 production in chronic-infected cells (CEM/LAV-1, H9/MN, and Molt-4/IIIB). The results suggest that thiamine disulfide may be important for AIDS chemotherapy.

Acetylcysteine↗

Presence of gene expression of vitamin D receptor and 24-hydroxylase in OK cells.

We investigated the gene expression of 1,25-dihydroxy-vitamin D3 (1,25(OH)2D3) receptors (VDR) and 25-hydroxy-vitamin D3-24-hydroxylase (24-hydroxylase) in opossum kidney (OK) cells. Reverse transcription-polymerase chain reaction demonstrated the presence of VDR gene expression in OK cells. Northern hybridization, using rat VDR cDNA, also demonstrated a 4.4 kbp VDR mRNA expression which was affected by serum but not by 1,25(OH)2D3 and/or parathyroid hormone stimulation. Stimulation with 1,25(OH)2D3 induced 24-hydroxylase mRNA expression which peaked at 6 h. This study clearly demonstrates for the first time the presence of VDR and 24-hydroxylase gene expression in OK cells, suggesting that OK cells are a useful model for investigating the genetic action mechanisms of 1,25(OH)2D3.

Animals↗

Dose-dependent association of apolipoprotein E allele epsilon 4 with late-onset, sporadic Alzheimer's disease.

We examined the apolipoprotein E (apo E) genotypes in 47 patients with late-onset sporadic Alzheimer's disease (mean age at onset +/- standard deviation, 72.2 +/- 6.4 years), 8 with late-onset familial Alzheimer's disease (75.5 +/- 5.1 years), 18 with early-onset sporadic Alzheimer's disease (52.8 +/- 4.7 years), and 10 with early-onset familial Alzheimer's disease (52.0 +/- 6.8 years) in Japan and compared them with genotypes in control subjects. In late-onset sporadic Alzheimer's disease, apo E-epsilon 4 frequency increased significantly (epsilon 4 frequency: 0.34 vs 0.095 in controls, p < 0.0001), and the odds ratio, which represents the strength of association between Alzheimer's disease and apo E-epsilon 4, markedly increased with increasing dose of apo E-epsilon 4 gene (3 [95% confidence interval, 2-6] in one dose; 43 [95% confidence interval, 12-154] in two doses). This study also suggested that apo E-epsilon 4 is associated with both late-onset (epsilon 4: 0.31) and early-onset familial Alzheimer's disease (epsilon 4: 0.35). In contrast, we found no association between apo E-epsilon 4 and early-onset sporadic Alzheimer's disease (epsilon 4: 0.08). These results indicate that the risk of developing late-onset sporadic Alzheimer's disease is markedly dependent on the dose of apo E-epsilon 4, while apo E-epsilon 4 does not appear to be a major risk factor for early-onset sporadic Alzheimer's disease.

Aged↗

cAMP-dependent inward rectifier current in neurons of the rat suprachiasmatic nucleus.

Electrophysiological properties of the inward rectification of neurons in the rat suprachiasmatic nucleus (SCN) were examined by using the single-electrode voltage-clamp method, in vitro. Inward rectifier current (IH) was produced by hyperpolarizing step command potentials to membrane potentials negative to approximately -60 mV in nominally zero-Ca2+ Krebs solution containing tetrodotoxin (1 microM), tetraethylammonium (40 mM), Cd2+ (500 microM) and 4-aminopyridine (1 mM). IH developed during the hyperpolarizing step command potential with a duration of up to 5 s showing no inactivation with time. IH was selectively blocked by extracellular Cs+ (1 mM). The activation of the H-channel conductance (GH) ranged between -55 and -120 mV. The GH was 80-150 pS (n = 4) at the half-activation voltage of -84 +/- 7 mV (n = 4). The reversal potential of IH obtained by instantaneous current voltage (I/V) relations was -41 +/- 6 mV (n = 4); it shifted to -51 +/- 8 mV (n = 3) in low-Na+ (20 mM) solution and to -24 +/- 4 mV (n = 4) in high-K+ (20 mM) solution. Forskolin (1-10 microM) produced an inward current and increased the amplitude of IH. Forskolin did not change the half-activation voltage of GH. 8-Bromo-adenosine 3',5'-cyclic monophosphate (8-Br-cAMP, 0.1-1 mM) and dibutyryl-cAMP (0.1-1 mM) enhanced IH. 3-Isobutyl-1-methylxanthine (IBMX, 1 mM) also enhanced IH. The results suggest that the inward rectifier cation current is regulated by the basal activity of adenylate cyclase in neurons of the rat SCN.

1-Methyl-3-isobutylxanthine↗

Endotoxin and pro-inflammatory cytokines stimulate endothelin-1 expression and release by airway epithelial cells.

Endothelin is a potent bronchoconstrictor peptide first identified as a novel vasoconstrictor produced by vascular endothelial cells. Recent reports suggest that airway epithelial cells are also capable of releasing this active peptide. To investigate the regulatory mechanism of endothelin expression, we studied the effects of endotoxin and pro-inflammatory cytokines such as interleukin-1 and tumour necrosis factor on the expression and release of endothelin-1 by airway epithelial cells. Both endotoxin and the cytokines stimulated endothelin-1 release by human bronchial epithelial cells. Northern blot analysis showed increased expression of preproendothelin-1 mRNA by these factors. These results suggested that airway epithelial cells might play a role in the local airway smooth muscle tone through the production of endothelin, which might be upregulated by inflammatory products in the airways.

Aged↗

[Studies on respiratory infections in primary care clinic (V). The pattern of distribution on bacteria, Mycoplasma pneumoniae and virus isolated from patients with respiratory infections, who were seen in six private clinics, and clinical efficacy of ciprofloxacin and roxithromycin].

The pattern of distribution of bacteria, Mycoplasma pneumoniae and virus isolated from the same specimen recovered from the throat swab or the sputum of 479 patients with respiratory infections who were seen in six private clinics in Sendai City of Japan during the period from October to November in 1992 (period I) and from January to February in 1993 (period II) was documented. Of the 479 patients, 234 had acute pharyngitis, 145 had acute bronchitis, 96 had influenza, 21 had acute tonsillitis, 5 had acute pneumonia and 9 had other respiratory infections. One hundred (42.4%) strains of potential pathogen and one strain of M. pneumoniae were recovered from 236 cases in period I, and 66 (27.2%) strains of potential pathogen, one strain of M. pneumonae and 73 strains of Influenza virus (30.0%: 43 of type A Hong-Kong and 30 of type B) from 243 cases in period II. Of the 166 strains, major isolates were Staphylococcus aureus (56 strains), Streptococcus pneumoniae (12 strains), Streptococcus pyogenes (15 strains), Haemophilus influenzae (17 strains), Esherichia coli (4 strains), Klebsiella spp. (35 strains), Pseudomonas aeruginosa (4 strains) and Acinetobacter spp. (23 strains). Only one strain of S. aureus was resistant to methicillin (MIC: 50 micrograms/ml). None of S. pneumoniae was resistant to 1 microgram/ml of ampicillin. Ciprofloxacin was administered to 113 cases and roxythromycin to 220 cases by doctors in charge.(ABSTRACT TRUNCATED AT 250 WORDS)

Ciprofloxacin↗

Clinical evaluation of plasma exchange and high dose intravenous immunoglobulin in a patient with Isaacs' syndrome.

The clinical effects of plasma exchange and high dose intravenous immunoglobulin (IVIg) were evaluated in a 41 year old woman with Isaacs' syndrome. After double filtration plasma exchange, symptoms almost disappeared for two to three weeks and the recorded continuous muscle action potentials were considerably decreased. Symptoms recurred within a few months. On the other hand, IVIg worsened the symptoms of the disorder: during and after IVIg at a dose of 0.2 g/kg/day (total 50 g), widespread myokymia, pseudomyotonia, and muscle cramps gradually increased. Symptoms improved after another course of plasma exchange.

Action Potentials↗

Wernicke's encephalopathy induced by hyperemesis gravidarum, associated with bilateral caudate lesions on computed tomography and magnetic resonance imaging.

The case of an 18-year-old woman with Wernicke's encephalopathy induced by hyperemesis gravidarum is reported. She had severe vomiting and received antiemetic therapy and intravenous administration of glucose and low-dose insulin solution without thiamine. She developed coma, nystagmus, ataxia and polyneuropathy. CT and MR imaging showed bilateral caudate lesions as well as symmetrical periventricular lesions of the thalamus and hypothalamus and periaqueductal gray matter. Caudate lesions are quite rare in Wernicke's encephalopathy.

Abortion, Missed↗

Fever of unknown origin: a review of 80 patients from the Shin'etsu area of Japan from 1986-1992.

In this survey involving 10 hospitals, we analyzed data on 80 Japanese patients from the Shin'etsu area (Nagano-ken and Niigata-ken) who were observed for fever of unknown origin (FUO). Our objectives were to identify the underlying causes and the relevant diagnostic methods. Fourteen of the patients died of the underlying illness. The cause of the FUO was infection in 43 patients, allergic or autoimmune disease in 13, neoplasm in 7, miscellaneous causes in 3, and undetermined in 14. FUO was self-limited in 13 patients and persistent in one patient. Methods successfully used to establish the final diagnosis in 66 patients were: evaluation of the clinical course or response to treatment in 16, serologic tests in 12, bacteriologic studies in 10, biopsy in 9, cytologic examination in 6, conventional radiology in 6, necropsy in 3, endoscopy in 2, and biochemical testing in 2.

Adolescent↗

Primary ocular and central nervous system malignant lymphoma first manifested as uveitis: possible role of single photon emission computed tomography with N-isopropyl-123I-p-iodoamphetamine in the diagnostic procedure.

A case of primary ocular and central nervous system malignant lymphoma was reported which was first manifested as bilateral uveitis ten months before the neurological symptoms appeared. The lesion of lymphoma in the central nervous system was clearly demonstrated as a heavy accumulation of N-isopropyl-123I-p-iodoamphetamine by single photon emission computed tomography. Since most brain tumors reportedly show a low uptake of N-isopropyl-123I-p-iodoamphetamine, single photon emission computed tomography with N-isopropyl-123I-p-iodoamphetamine could be helpful in making the diagnosis, as well as in visualizing primary central nervous system malignant lymphoma.

Adult↗

Patch-clamp analysis of hypoglycemia-induced inhibition of synaptic transmission in the rat dorsolateral septal nucleus.

The role of glucose in neuronal transmission was examined in the rat dorsolateral septal nucleus (DLSN) by using 'slice-patch' techniques. Removal of glucose from the oxygenated Krebs solution produced an outward current associated with an increased K+ conductance. The glucose-depletion depressed the excitatory postsynaptic current (EPSC), the inhibitory postsynaptic current (IPSC) and the late hyperpolarizing current (LHC) produced by stimulation of the fimbria/fornix pathway. It is hypothesized that glucose regulates neurotransmission in the rat DLSN by modulating the release of transmitters from presynaptic nerve terminals.

Animals↗

[Sequential monitoring of various HCV-related markers in chronic hepatitis C with interferon treatment].

Interferon (IFN) which is a powerful antiviral cytokine is capable to cure approximately 40% of patients with chronic hepatitis C. We should like to know in detail whether IFN is operating enough to improve chronic hepatitis C during IFN treatment. Therefore it was evaluated what kind of HCV related markers were important to monitor the clinical course. We discussed HCV-RNA (RT-PCR, DNA probe assay) and some antibodies to HCV including our new IgM antibody to a HCV core peptide, cp14 (amino acid 5-40 of the core protein). HCV-RNA assayed by RT-PCR was considered best to monitor the therapeutic effect of IFN on chronic hepatitis C. However, as it is still not in general, IgM anti-cp14 might be an alternative in monitoring the response to IFN in patients with chronic hepatitis C.

Biomarkers↗