[Neuro-Behcet disease].
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Behcet Syndrome↗
Biomedical subjects
Y Wakayama
Publications and source records attributed to Y Wakayama.
Immunocytochemical studies of aquaporin 4 in the skeletal muscle of mdx mouse.
Immunostainability of anti aquaporin 4 antiserum was investigated in the muscles of dystrophin deficient mdx mice. Western blot analysis showed that the rabbit antiserum against aquaporin 4 reacted with a 28 kDa protein in extracts of normal mouse quadriceps femoris muscles but did not react with the protein in extracts of quadriceps femoris muscles of mdx mice. Immunoperoxidase staining of the muscles from normal and mdx mice revealed the positive immunoreaction at the myofiber surface of normal mice and the negative, or the faint and discontinuous immunostaining at the surface of mdx myofibers. Immunogold electron microscopy disclosed the localization of aquaporin 4 molecules at the myofiber plasma membranes of normal mice and the localization was consistent with that of orthogonal array particles in the protoplasmic face of normal muscle plasma membrane seen in freeze fracture replicas. This study demonstrated that the density of aquaporin 4 molecules was decreased in the muscle plasma membranes of mdx mice, resulting in the faulty function of mdx myofibers.
Animals↗
Ultrastructural localization of alpha-, beta- and gamma-sarcoglycan and their mutual relation, and their relation to dystrophin, beta-dystroglycan and beta-spectrin in normal skeletal myofiber.
Ultrastructural localization of alpha-, beta- and gamma-sarcoglycan and their mutual relation, and their relation to dystrophin, beta-dystroglycan and beta-spectrin were investigated in normal skeletal myofibers. Single-immunogold labeling electron microscopy showed that the signals of rabbit and sheep polyclonal antibodies against the synthetic peptide of the cytoplasmic domain of alpha-, beta or gamma-sarcoglycan were present along the inside surface of muscle plasma membrane and at the sarcoplasmic side of plasma membrane invaginations and vesicular structures in subsarcolemmal areas. These localizations were similar to that of dystrophin, beta-dystroglycan and beta-spectrin. Double-immunogold labeling disclosed the close association of alpha-, beta- and gamma-sarcoglycan each other and alpha-, beta-, gamma-sarcoglycan with dystrophin or beta-dystroglycan, and this was confirmed by statistical analysis. Monoclonal antibody against the extracellular domain of alpha-sarcoglycan was used with above-mentioned polyclonal anti-beta- and -gamma-sarcoglycan antibodies for triple-immunogold labeling, in which signals of alpha-sarcoglycan localized at the outer surface of muscle plasmalemma and those of beta- and gamma-sarcoglycans were present at the inside surface of plasma membrane. The triple immunolabeling showed an occasional closely associated presence of the three signals for alpha-, beta-and gamma-sarcoglycans, and a more frequent association for two signals out of alpha-, beta- and gamma-sarcoglycans. This study demonstrated that alpha-, beta- and gamma-sarcoglycan are closely located to one another and to dystrophin and beta-dystroglycan at the muscle plasma membrane.
Animals↗
Expression of selectin families and their ligand sialyl Lewis X in the muscles of inflammatory myopathies: an immunohistochemical study.
OBJECT: Adhesion molecules are suggested to play important roles in the pathogenesis of inflammatory diseases. We examined the expression of adhesion molecules in the muscles of human inflammatory myopathies. METHODS: We immunohistochemically studied the expression and distribution of two molecules in the selectin family (E- and P-selectin) and their common ligand sialyl Lewis X in 18 inflammatory myopathies, 13 disease controls, and 16 normal controls. RESULTS: In inflammatory myopathies, E- and P-selectin were upregulated on the surface of blood vessels, especially on the endothelial cells of the venules. Sialyl Lewis X was upregulated in the blood vessels, infiltrating leukocytes, and the surface of some atrophic myofibers. Some control muscles also showed weakly positive staining with these molecules, however, expression of these molecules was most striking in the muscles of inflammatory myopathies. CONCLUSION: The results suggested that these molecules are upregulated in inflammatory myopathies and might play a role in the pathogenesis of inflammatory myopathies.
Cell Adhesion Molecules↗
[Quality of life in elderly patients with cerebral vascular disease and Parkinson's disease].
We developed a questionnaire for the study of background factors and quality of life (QOL) in elderly patients with cerebral vascular disease (CVD) and Parkinson's disease (PD). The questionnaire covered the background factors and four sections such as physical, functional, psychological and social health sections. Each section had 15 questions and disease-specific questions for CVD or PD were included in the physical health section. We analyzed 107 patients with CVD (76 elderly patients, aged 65 or more, 31 non-elderly patients under 65) and 136 patients with PD (91 elderly, 45 non-elderly). In the background section, of a total of 243 patients with CVD and PD, the elderly patients needed the assistance of their spouse and their sons wives more frequently than non-elderly patients. With regard to rehabilitation, non-elderly CVD patients had rehabilitation more frequently than the elderly CVD patients, while a higher percentage of elderly patients with PD had rehabilitation training more frequently than the non-elderly PD patients. In the QOL section, there was no difference between elderly and non-elderly CVD patients, while elderly PD patients were statistically more significantly disabled physically and weak-minded psychologically. The physical disabilities of the elderly PD patients in this statistical investigation included slow motion, stooped posture, frozen gait, difficulty in turning and standing up, constipation and dysuria. The psychological problems of elder PD patients included forgetfulness and a feeling of aging. These patients had significantly fewer consultations by family and relatives than the non-elderly PD patients. The overall tendency of QOL in patients with CVD and PD was similar to that of PD patients.
Aged↗
[A case of non-herpetic acute encephalitis presenting high intensity lesion at unilateral temparal cortex on MR FLAIR image].
We reported a case of non-herpetic acute encephalitis with unilateral temporal cortex lesion revealed by MR imaging and SPECT study. The patient was an eighteen years old woman who developed tonic-clonic seizure after common cold symptom. She was healthy before this episode. Neurological abnormality was only a single convulsion at onset and there was no other abnormal physical and neurological signs except for low grade fever. Electroencephalogram showed spike and slow wave complex of 2 Hz focused on a right posteriotemporal point (T 6) and an MR FLAIR (fluid-attenuated inversion recovery) image revealed a high signal intensity area at right temporal cortex. There was a decrease of cerebral blood flow in the same portion on SPECT study. This lesion was obscure on T1 and T2 MR images. Cerebrospinal fluid showed pleocytosis with normal glucose level and protein concentration. Bacterial and fungal cultures of CSF were negative and a detection of tubercule bacillus by PCR hybridization method was also negative. Although CSF findings suggested viral infection of CNS, virological study could not demonstrate infections of herpes simplex virus type 1, type 2, varicella-zoster virus, cytomegalovirus, measles virus, mumps virus, Japanese encephalitis virus, and influenza virus type A and B. After infusion of acyclovir and antibiotics, the patient was discharged from our hospital without sequelae of encephalitis. EEG was normal at this point and a high intensity area of MR FLAIR image disappeared two months later. SPECT findings were normalized six months later. The encephalitis presenting unilateral temporal cortex lesion without the infection of herpes simplex virus is thought to be very rare. Our case was distinguished from non-herpetic acute limbic encephalitis by an extent of the lesion and clinical manifestations. MR FLAIR image was useful for the detection of the lesion in this case.
Acute Disease↗
Benign adult-onset focal myositis confined to the calf muscles.
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Female↗
The ubiquitin-proteasome pathway in distal myopathy with rimmed vacuoles.
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Cysteine Endopeptidases↗
[A management for severe acquired stuttering in a case of pure akinesia syndrome].
We reported a 73-year-old man with pure akinesia syndrome who showed severe acquired stuttering and paradoxical kinesia on speech. He was evaluated in another hospital for bradykinesia and frozen gait at age of 67 when his cranial MRI disclosed ischemic changes in bilateral basal ganglia and periventricular deep white matter. The treatment with L-dopa and L-threo DOPS was not effective. His symptoms were slowly progressive and got worse gradually. At age of 72, he began to have difficulty in speech due to severe acquired stuttering, and one year later, he visited our hospital. The diagnosis of pure akinesia syndrome was made because of akinesia, micrographia, marked frozen gait with paradoxical kinesia and disturbance of postural reflex without tremor and rigidity. His speech showed severe acquired stuttering with marked blocking and repetition of initial phonemes at the beginning of speech. But intelligible speech recurred with pointing the characters by his finger or with writing an initial letter of word, although his speech was small and monotonous. Surface EMG findings of muscles participating speech in acquired stuttering showed the similar tonic discharge to those of muscles of lower extremity in frozen gait. These results implied that freezing phenomenon and festination of muscles participating speech in our patient may result in acquired stuttering.
Aged↗
Immunogold and freeze etch electron microscopic studies of merosin localization in basal lamina of human skeletal muscle fibers.
Merosin is a basement-membrane-associated protein found in striated muscle, peripheral nerve and placenta, the deficiency of which causes the muscle wasting condition in C57BL/6J-dy/dy, so-called dy/dy mouse. Moreover, merosin is the binding protein of 156 kDa alpha-dystroglycan which binds dystrophin by way of 43 kDa beta-dystroglycan. Therefore, merosin is an important component of the basal lamina of normal skeletal myofibers. We investigated the ultrastructural localization of merosin antibody in normal human skeletal myofibers by using immunogold electron microscopy and freeze etch electron microscopy. The ultrastructure of the basal lamina showed the presence of the lamina lucida, lamina densa and lamina reticularis. The lamina lucida appeared electron translucent with the exception of fuzzy fibrils. The immunogold electron microscopy disclosed that the merosin was present at the innermost layer (lamina lucida) of the basal lamina of normal human skeletal myofibers. With freeze etch replica electron microscopy, short cross-bridge fine fibrils were noted in the lamina lucida, connecting the basal lamina to the outer leaflet of the muscle plasma membrane. They measured 3-13 nm in diameter, 20-90 nm in length and were distributed with a spacing of 30-40 nm. The immunogold particles showing the presence of the merosin epitope were associated with these connecting structures.
Basement Membrane↗
Ultrastructural localization of alpha 1-syntrophin and neuronal nitric oxide synthase in normal skeletal myofiber, and their relation to each other and to dystrophin.
We investigated the ultrastructural localization of alpha 1-syntrophin and neuronal nitric oxide synthase (nNOS) in normal human skeletal myofibers and analyzed their relation to each other and to dystrophin using single and double immunogold-labeling electron microscopy. Single immunolabeling showed antibodies to alpha 1-syntrophin and nNOS on the inner surface of the muscle plasma membrane, the sarcoplasmic side of plasma membrane invaginations, and the sarcoplasm near mitochondria of subsarcolemmal areas. The epitopes of alpha 1-syntrophin and nNOS tended to be present in clusters. Double immunolabeling revealed that epitope combinations of alpha 1-syntrophin-dystrophin, alpha 1-syntrophin-nNOS, and nNOS-dystrophin occurred more frequently in doublet form than did other epitope combinations, such as alpha 1-syntrophin-beta- spectrin and nNOS-beta-spectrin. These increased frequencies were noted both at the muscle plasma membrane undercoat and near mitochondria of subsarcolemmal areas. A significantly higher percentage of doublets comprised antibodies against alpha 1-syntrophin and dystrophin (28.5 +/- 1.5%, group mean +/- SE) than those against alpha 1-syntrophin and beta-spectrin (9.2 +/- 0.8%, P < 0.01). Furthermore, nNOS formed doublets significantly more frequently with dystrophin (25.2 +/- 3.3%) and alpha 1-syntrophin (26.0 +/- 4.1%) than with beta-spectrin (13.9 +/- 2.3%; P < 0.05). These data support the association of dystrophin, alpha 1-syntrophin, and nNOS at the inner surface of the muscle plasma membrane and near mitochondria of subsarcolemmal areas of normal human skeletal myofibers.
Amino Acid Sequence↗
A comparative freeze-fracture study of plasma membrane of dystrophic skeletal muscles in dy/dy mice with merosin (laminin 2) deficiency and mdx mice with dystrophin deficiency.
The intramembranous particle (IMP), orthogonal array (OA) and orthogonal array subunit particle (OASP) densities of skeletal muscle plasma membranes of merosin deficient dy/dy mice and their control mice at 7, 14 and 28 days after birth were analysed by freeze-fracture electron microscopy. Similar studies were performed on dystrophin-deficient mdx mice with mild muscle weakness at 28 days after birth for the comparison with those of dy/dy mice with severe muscle weakness at the same age. In the pre-clinical stage of dy/dy mice at 14 days after birth, the membranes showed a significantly decreased density of OAs (P<0.01 by Wilcoxon rank-sum test) as compared with control mice, while those in the clinical stage of dy/dy mice at 28 days after birth showed normal IMP density but a marked depletion of OA density (P<0.01). Moreover, at 28 days after birth, the reduction of OAs in the plasma membranes of dy/dy mice was more marked than that of mdx mice (P<0.05 by Wilcoxon rank-sum test). These results provided us with the information that the OA density was affected more severely with merosin deficiency than with dystrophin deficiency, and again supported our previously proposed concept that the clinical severity in muscular dystrophies correlated with the OA density.
Animals↗
[Multiple solitary lesions in deep white matter in the early stage of Creutzfeldt-Jakob disease. A case report].
A 56-year-old housewife was admitted to our hospital because of involuntary movement on her left arm. Her neurological examination on admission showed mild weakness of her left arm and cerebellar ataxia. She developed periodic synchronous discharge on electroencephalogram and her cerebrospinal fluid revealed elevated level of neuron-specific enolase. Thereafter she developed dementia, followed by apallic state and diagnosed as having Creutzfeldt-Jakob disease (CJD). Interestingly, her MRI on admission revealed multiple solitary lesions in deep cerebral white matter, which were detected as high signal intensity by T2 weighed image. A few months later, these lesions tended to extend, and finally fused around the lateral ventricle in parallel with remarkable cortical atrophy. We excluded other diseases such as cerebrovascular disorders. Finally, we concluded the white matter change seen from early stage in this case may be the lesion associated with CJD, and the CJD case with early white matter changes has been seldomely described.
Atrophy↗
[Cerebrospinal fluid neuron-specific enolase as a useful indicator for the early diagnosis of Creutzfeldt-Jakob disease].
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Aged↗
Urinary basic fetoprotein in the diagnosis and follow-up of patients with urothelial carcinoma.
BACKGROUND: Urothelial carcinoma is one of the most common malignant neoplasms of the urinary tract and is characterized by a high local recurrence rate. However, no specific and reliable tumor marker has been identified for the diagnosis and follow-up of patients with urothelial carcinoma. METHODS: Urinary basic fetoprotein (U-BFP) levels were measured by enzyme immunoassay in 119 patients with urothelial carcinoma, 64 patients with other urologic malignancies, 144 patients with benign diseases, and 166 healthy individuals to evaluate it clinical efficacy as a marker of urothelial carcinoma. The histologic logic localization of BFP was also studied. A cutoff value was set at 14 ng/mL based on the maximum diagnostic efficiency. RESULTS: The specificity and sensitivity of U-BEP for urothelial carcinoma was 91.7% and 58%, respectively. THe U-BFP positive rate increased significantly with the histologic grade. The U-BFP positive rate was higher in both Tis and T2-4 than in Ta, 1 disease. The U-BFP level exceeded the cutoff value before curative therapy in 32 patients; U-BFP was normalized in 97% of these patients within 2 weeks after therapy. There was a total of 11 recurrences and the U-BFP level returned to abnormally high levels in all instances. Histologic localization of U-BFP was demonstrated in the cytoplasm of the tumor cells in 80% of the patients. CONCLUSION: Based on these results, U-BFP is a potential tumor maker of urothelial carcinoma. In particular, the test could be used to monitor patients whose U-BFP value is positive before therapy.
Adolescent↗
Ultrastructural localization of adhalin in normal murine skeletal myofiber.
The ultrastructural localization of adhalin and its relations to dystrophin, beta-dystroglycan, and beta-spectrin were studied in normal murine skeletal myofibers. The C-terminal peptides of adhalin and beta-dystroglycan were synthesized based on their cDNAs, and the affinity-purified antibodies against these peptides were produced. Single-immunolabeling electron microscopy showed that the adhalin was located just inside the muscle plasma membrane or inside the myofiber a short distance from the plasma membrane. The adhalin signal was also noted at the sarcoplasmic side of plasmalemmal invaginations or at vesicular structures in subsarcolemmal areas. Double-immunogold-labeling electron microscopy disclosed a similar localization of dystrophin, beta-dystroglycan, and beta-spectrin. The close association of adhalin with dystrophin or beta-dystroglycan was demonstrated by formation of doublets by signals of antibodies of adhalin with those of dystrophin or beta-dystroglycan and was confirmed by statistical analyses. This study demonstrated that the location of adhalin is close to that of dystrophin and beta-dystroglycan at the muscle plasma membrane.
Animals↗
Electron microscopic observations of triple immunogold labelling for dystrophin, beta-dystroglycan and adhalin in human skeletal myofibers.
Dystrophin is the Duchenne muscular dystrophy gene product and is a membrane cytoskeletal protein present in the network of the plasma membrane undercoat. Adhalin (50 kDa dystrophin-associated glycoprotein) and beta-dystroglycan (43 kDa dystrophin-associated glycoprotein) are the transmembrane components of the normal muscle plasma membrane, and beta-dystroglycan has been demonstrated to bind dystrophin at the inside surface of normal muscle plasma membrane. This investigation was undertaken to test whether the epitopes of dystrophin, beta-dystroglycan and adhalin are closely associated with each other by using triple immunogold labelling electron microscopy on normal human skeletal myofibers. Although closely associated signals of triplet immunogold particles were observed, there were less numerous than expected. However, closely associated signals of two epitopes of dystrophin and beta-dystroglycan, dystrophin and adhalin, or adhalin and beta-dystroglycan were frequently observed. These ultrastructural findings are consistent with biochemical evidence implying that dystrophin, beta-dystroglycan and adhalin are closely associated with each other at the normal muscle plasma membrane.
Cytoskeletal Proteins↗
Microwave ablation of the adrenal gland: experimental study and clinical application.
OBJECTIVE: To evaluate the heating effect of microwave irradiation on the dog adrenal gland and to assess the clinical feasibility of microwave ablation of adrenal tumours. MATERIALS AND METHODS: The thermal distribution and histology were evaluated after dog adrenal glands were irradiated at different intensities with microwaves. Irradiation at 70 W for 30 s was also performed after laparotomy, on the adrenal tumour of a patient with primary aldosteronism. RESULTS: Microwave irradiation at 70 W for 30 s provided a sufficiently high temperature to produce coagulation within a 5 mm radius of the microwave electrode. The adrenal tumor in the patient with primary aldosteronism was coagulated successfully by this dose of microwave irradiation. CONCLUSION: Microwave ablation of the adrenal gland may be clinically applicable in the treatment of adrenal tumours.
Adrenal Glands↗