[Physiopathology and clinical presentation of Crow-Fukase Syndrome].
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Humans↗
Biomedical subjects
K Saida
Publications and source records attributed to K Saida.
Antibodies to gangliosides and galactocerebroside in patients with Guillain-Barré syndrome with preceding Campylobacter jejuni and other identified infections.
The relationship between preceding infections and antibodies to glycolipids was investigated in 205 Japanese patients with Guillain-Barré syndrome (GBS). Serological evidence of recent Campylobacter jejuni (C. jejuni) infection was found in 45% of the patients, compared with 1% in healthy controls. In contrast, recent infection of cytomegalovirus (CMV), Mycoplasma pneumoniae (M. pneumoniae) and Epstein-Barr virus (EBV) was detected in only 5%, 2% and none of the patients, respectively. C. jejuni-associated GBS was more frequent in early spring than in other seasons. All stool specimens positive for C. jejuni isolation were obtained within 10 days after the onset of GBS symptoms. Of 13 C. jejuni isolates from GBS patients, 10 (77%) belonged to Penner serotype 19 (heat-stable, HS-19). Elevated titers of anti-GM1 antibody were found in 8 (80%) of 10 GBS patients whose C. jejuni isolates belonged to HS-19 and in none of those infected with non-HS-19 C. jejuni (P = 0.04), and in 49% of 92 patients with C. jejuni infection and 25% of patients without infection of C. jejuni, CMV, EBV, or M. pneumoniae (P = 0.0007). The frequencies of elevated antibody titers to GD1a, GD1b and GQ1b were also significantly higher in GBS patients associated with C. jejuni than those not associated with C. jejuni, CMV, EBV, and M. pneumoniae. GBS in Japan seems to be associated more frequently with C. jejuni and less frequently with CMV than in Europe and North America.
Adolescent↗
Coagulation and vascular abnormalities in Crow-Fukase syndrome.
Coagulation and vascular abnormalities were studied in 4 patients with Crow-Fukase syndrome (CFS or POEMS) to understand the pathophysiology. Fibrinogen, fibrinopeptide A, and thrombin-antithrombin complexes (TAT) increased in sera during active phase of CFS. In nerves of 2 untreated cases, the endothelium of small vessels was immunohistochemically stained with antithrombin III antibody, which indicates the existence of TAT. HLA-DR+ inflammatory cell infiltrate surrounded these vessels. Blood-nerve barrier opening was suggested by strong immunoglobulin staining in the endoneurium. More than 50% of endoneurial blood vessels had narrowed or closed lumina with thick basement membranes. Endothelial cell abnormality and chronic intravascular coagulation may play an important role in the pathogenesis of CFS, in addition to a still unknown demyelinating factor. Refractory cases responded to combined treatment of prednisolone, human leukocyte interferon, and antithrombin drug.
Antifibrinolytic Agents↗
Gene locus for autosomal recessive distal myopathy with rimmed vacuoles maps to chromosome 9.
Distal myopathy with rimmed vacuoles is an autosomal recessive muscular disorder, characterized clinically by weakness of the distal muscles in the lower limbs in early adulthood. Recently, the gene locus for familial vacuolar myopathy with autosomal recessive inheritance (hereditary inclusion body myopathy) was mapped to chromosome 9 by genome-wide linkage analysis of nine Persian-Jewish families. Since both disease conditions share similar clinical, genetic, and histopathological features, we analyzed seven families with distal myopathy with rimmed vacuoles using ten microsatellite markers within the region of the hereditary inclusion body myopathy locus. Significantly high cumulative pairwise lod scores were obtained with three markers: D9S248 (Z(max) = 5.90 at theta = 0), D9S43 (Z(max) = 5.25 at theta = 0), and D9S50 (Z(max) = 4.23 at theta = 0). Detection of obligate recombination events as well as multipoint linkage analysis revealed that the most likely location of the distal myopathy with rimmed vacuoles gene is in a 23.3-cM interval defined by D9S319 and D9S276 on chromosome 9. The results raise the possibility that distal myopathy with rimmed vacuoles and hereditary inclusion body myopathy in Persian Jews are allelic diseases.
Adult↗
Central nervous system lesions in rats infected with Friend murine leukemia virus-related PVC441: ultrastructural and immunohistochemical studies.
Newborn F344 rats were injected intraperitoneally with PVC441 virus, a neuropathogenic variant of Friend murine leukemia virus, and developed paraparesis of hind limbs 35-40 days after infection. Immunohistochemical study using monoclonal anti-PVC441 antibody revealed that in the central nervous system endothelial cells but not neuronal or glial cells were infected with PVC441 virus. The major pathological changes were myelin vacuolation and oligodendrocyte degeneration in the white matter at the white-gray border zone. Anterior and lateral funiculi and intercalated myelin of anterior horns were dominantly affected in the spinal cord from the sacral to cervical level. The midbrain was also vacuolated. An ultrastructural study demonstrated that many viral particles were present outside the endothelial cells but only sparsely inside endothelial cells and pericytes. Endothelial cell membranes and tight junctions were also disrupted. Immunohistochemical studies with antibodies against major histo-compatibility complex class Ia, intercellular adhesion molecule-I, glial fibrillary acidic protein, neurofilament protein, CD3 and OX42 revealed the presence of abundant microglia but not of lymphocytes or polymorphonuclear cells in the lesions. Axonal degeneration and astrogliosis were mild in degree. These pathological changes explain the observed spastic paraparesis in the rats, and represent a good model of spongiform diseases of the human central nervous system of retroviral origin, such as human T cell leukemia virus-associated myelopathy and AIDS.
Animals↗
[A case of cortical reflex myoclonus manifesting tremor].
A 58-year-old woman developed slowly progressive tremulous myoclonus provoked mainly by action and posture. She had neither seizure nor dementia. No one in her family had similar symptoms. The presence of giant somatosensory evoked potentials (SEP) with enhanced long loop reflex and premovement cortical spikes demonstrated by the jerk-locked averaging method suggest that the involuntary movement is cortical reflex myoclonus. Magnetoencephalogram revealed that the generator sources of giant SEPs were the post-central somatosensory cortex and probably also the pre-central motor cortex. Symptoms improved after treatment with zonisamide, clonazepam and valpolate. This kind of involuntary movement might be called cortical myoclonic tremor.
Anticonvulsants↗
Post-infectious encephalitis with anti-galactocerebroside antibody subsequent to Mycoplasma pneumoniae infection.
Galactocerebroside (Gc) is a major component of myelin in both the peripheral and central nervous systems. Although it is regarded as an important glycolipid hapten of myelin in rabbit experimental allergic neuritis (EAN), its role in human demyelinating diseases is not known. We studied three post-infectious encephalitis (PIE) patients related to Mycoplasma pneumoniae infection. All three of three patients with encephalitis and M. pneumoniae infection were positive for Gc antibodies (100%), while 25% of 32 M. pneumoniae-infected patients without neurological disease were positive, and 3.8% of 52 healthy controls. This indicates anti-Gc antibody is induced by M. pneumoniae infection. One of the PIE patients, who had extraordinary high titer antibody to Gc, showed an extensive, diffuse white matter demyelination and poor recovery. Since circulating anti-Gc antibody induces central nervous system demyelination in animals with elevated antibody titers and disruption of the blood-brain barrier, anti-Gc antibody may have an important function in the increased demyelination in PIE patients after M. pneumoniae infection.
Antibodies↗
Sequence and neuronal expression of mouse endothelin-1 cDNA.
We have isolated and sequenced a cDNA that encodes mouse endothelin-1 (ET-1). The putative protein contains 202 amino acids corresponds to the prepro-form of ET-1. Twenty-one amino acids sequence of the putative mature ET-1 was identical with that of rat, porcine, bovine, and human. In situ hybridization histochemistry indicate that ET-1 mRNA was expressed in several hypothalamic nuclei including the suprachiasmatic nucleus (SCN) in rodent brain.
Amino Acid Sequence↗
Cytokines and myelin antibodies in Crow-Fukase syndrome.
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Antibodies↗
The immunopathology of Guillain-Barré syndrome.
Identification of new antigens in different patterns of Guillain-Barré syndrome has led to new pathophysiological concepts of Guillain-Barré syndrome and the related Miller-Fisher syndrome. Patients with Guillain-Barré syndrome occurring after Campylobacter jejuni infection have been found to develop more frequently axonal and motor forms of the syndrome. Anti-GM1 antibodies decreased Na+ current in the presence of complement. In acute axonal Guillain-Barré syndrome, macrophages were found in the periaxonal space without damaging myelin sheath. Important epitopes may be localized on the axolemma, but further studies are needed to confirm these observations.
Animals↗
[Vasoactive intestinal contractor (VIC)/mouse ET-2 and VIC receptor: biological activity, gene expression, and specific receptor].
Using molecular biology techniques with the Endothelin (ET) cDNA as a probe, we discovered a novel peptide, vasoactive intestinal contractor (VIC). VIC differed from ET (= ET-1) in 3 amino acid residues. Synthetic VIC had in vivo pressor and in vitro vasoconstrictor activity such as that of ET. Northern blot analysis, however, indicated the VIC gene to be expressed in the intestine. Furthermore, VIC evoked stronger contractile response in ileum than ET. VIC may thus possibly be reasonably classified as a gut peptide. From the cDNA sequence analysis, the mature VIC is predicted to be produced via an intermediate from the deduced prepro VIC.
Amino Acid Sequence↗
Endothelin induced collagen remodeling in experimental pulmonary hypertension.
To investigate pathophysiological roles of endothelin-1 (ET-1) in collagen remodeling in pulmonary hypertension, we measured: (a) mRNA expression, concentration, localization of ET-1; (b) changes in types and content of collagen in the lung; (c) and confirmed direct effects of ET-1 on type V collagen metabolism in vascular smooth muscle cells. Monocrotaline-treated rats showed pulmonary hypertension with medial hypertrophy and perivascular fibrosis of pulmonary arteries. At the progressive stage of pulmonary hypertension, both ET-1 levels and its mRNA expression in the lung increased. Total collagen in the lung rose markedly with a higher rate of increase in type V collagen. ET-1, which exists in vascular smooth muscle cells, other perivascular cells and endothelium, stimulated type V collagen production. Our results suggest that local production of ET-1 in the lung contributes to progression of pulmonary hypertension through changes in phenotypes and content of collagen.
Animals↗
Regenerative capacity of mdx mouse muscles after repeated applications of myo-necrotic bupivacaine.
We injected bupivacaine (BPVC), which produces muscle fiber necrosis, repeatedly into the soleus muscles of mdx mice, which represent a model of human Duchenne muscular dystrophy, over a 12-month period. Cytological and morphometric analysis revealed that the regenerative capacity of repeatedly BPVC-injected mdx muscles was almost equal to that of the saline-injected mdx muscles. At 9 months of age the endomysial collagen content of mdx muscles was 4.6 times that of control mice muscles, and was 7.2 times that of control mice muscle at 12 months. These results suggest that the regenerative capacity of the mdx muscle is quite large and that myo-necrosis induced by an extrinsic cause, such as BPVC, may not be an important factor in the disease progress. However, endomysial collagen, for which the mechanism of increase may be related to the defect of dystrophin, may play an important role in gradual decline of regeneration.
Age Factors↗
[Guillain-Barré syndrome and infection].
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Autoantibodies↗
Improvement of ischemic myocardial dysfunction by nisoldipine.
We examined the cardioprotective effect of nisoldipine against myocardial dysfunction during ischemia and reperfusion in Langendorff perfused rabbit hearts. Nisoldipine was administered to the hearts before 60 minutes of global ischemia. This agent inhibited the increase of end-diastolic pressure during ischemia and also improved the recovery of left ventricular developed pressure and coronary flow during reperfusion in a concentration-dependent manner. The maximal cardioprotective effect was observed in 10(-8) M nisoldipine. The beneficial effect was associated with an increase of coronary flow during reperfusion. Therefore, both the nisoldipine-increased coronary flow during reperfusion and the inhibition of ischemic contracture by nisoldipine seem to play a crucial role in improving myocardial dysfunction of ischemic-reperfused hearts.
Animals↗
Failure to transfer multiple sclerosis into severe combined immunodeficiency mice by mononuclear cells from CSF of patients.
To confirm the reported transfer of multiple sclerosis (MS) by CSF cells, we injected CSF cells from six MS patients in the exacerbation stage into the cisterna magna of 18 severe combined immunodeficiency mice. No clinical neurologic abnormalities or light- or electron-microscopic pathologic changes were present in any transferred mice, and the reported results could not be reproduced.
Adult↗
[A surgical case of giant mediastinal teratoma].
A 35-year-old female was admitted with a large abnormal shadow in the left lung field on a chest X-ray. She was examined by chest tomogram, chest CT, MRI and bronchofiberscope. The CT and MRI showed a large tumor mass pressing the left lung and calcified deposits in the central part of the tumor. Bronchofiberscopic findings showed a stenosis of the left main bronchus. The clinical diagnosis was mature teratoma arising from anterior medistinum. She underwent a median sternotomy and the tumor was removed. The size of the resected specimen, filled with sebaceous material, hair and teeth, etc., was 14 x 12 x 10 cm (1,045 g). Histopathological examination of the specimen revealed a mediastinal mature teratoma. She has been well for 16 months postoperatively.
Female↗
[Treatment of neuro-immunologic diseases by immunosuppressants].
Immunosuppressive treatments of neuro-immunologic diseases: myasthenia gravis, polymyositis, chronic inflammatory demyelinating polyneuropathy and multiple sclerosis were reviewed. The treatments need to be planned in terms of 2-5 years. Cautions must be taken for adverse effects of short and long terms. Corticosteroids were the most well used and were studied medication of the first choice among immunosuppressants in these diseases except for CIDP in which large amounts of IV-Ig or plasmapheresis are the first choice. Pulse treatment of very high doses of steroids are used in refractory or severe cases. As for immunosuppressants, in polymyositis iv MTX is the choice since its response is quicker than AZ. CsA is used in cases with pneumonitis. In MS, pulse treatments of steroids followed by gradual decreasing doses of steroids are used for 2-3 months in each relapse. Recently, IFN-alpha 2a and IFN-beta significantly reduced the number of relapses and improved MRI findings. Chronic applications of AZ, CY, Cs or MTX have possibilities of reducing relapses, and new drugs like mizoribine and mitoxantrone etc. are in trials.
Demyelinating Diseases↗