Brain and nerve = Shinkei kenkyū no shinpo
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Spatial patterns of spontaneous fluctuations in blood oxygenation level-dependent (BOLD) signals reflect the underlying neural architecture. The study of the brain network based on these self-organized patterns is termed resting-state functional MRI (fMRI). This review article aims at briefly reviewing a basic concept of this technology and discussing its implications for neuropsychological studies. ⋯ Second, I have discussed the reservations and potential pitfalls of 2 major imaging methods: voxel-based lesion-symptom mapping and task fMRI. Problems encountered with voxel-based lesion-symptom mapping can be overcome by using resting-state fMRI and evaluating undamaged brain networks in patients. Regarding task fMRI in patients, I have also emphasized the importance of evaluating the baseline brain activity because the amplitude of activation in BOLD fMRI is hard to interpret as the same baseline cannot be assumed for both patient and normal groups.
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Collagen VI-related muscle disorders include severe Ullrich's disease (Ullrich congenital muscular dystrophy:UCMD) and milder Bethlem myopathy. Mutations in the 3 collagen VI genes, namely, COL6A1, COL6A2, and COL6A3, cause both diseases. UCMD is inherited in an autosomal recessive manner, and de novo dominant mutations are also reported. ⋯ We evaluated the role of nonsense-mediated mRNA decay (NMD) in UCMD associated with a premature termination codon in the COL6A2 gene, which caused the loss of collagen VI. A pharmacological block of NMD caused upregulation of the mutant collagen VI and partially functional extracellular matrix formation. Cyclosporin A has been reported to correct mitochondrial dysfunction and muscle apoptosis in patients with collagen VI myopathies, and a pilot trial of cyclosporin A was carried out.
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Review Case Reports
[Clinical features of tetanus: a review with case reports].
Tetanus is a CNS disorder characterized by muscle spasms that is caused by the exotoxin of an anaerobic bacterium, Clostridium tetani. This disease is killing tens of thousands of neonates in developing countries. Although the incidence and mortality of tetanus has dramatically dropped in developed countries due to effective vaccination, appropriate wound management, and recent advances in intensive care, treatments remain difficult. ⋯ No patients died due to ICU managements, in which the most useful were propofol for spasm control and sedation, and magnesium sulfate for autonomic overactivity. Retrospectively, the Tetanus Severity Score (TSS) for mortality proposed by Thwaites et al (2006) is considered a useful tool also for predicting the clinical outcome at discharge. Although tetanus has been traditionally classified into the generalized, local, and cephalic types, a simpler severity-based classification into "severe", "moderate", and "mild" types may be more practical with regard to disease management.
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Review Comparative Study
[Progress in NIRS monitoring of cerebral blood flow].
Various studies have demonstrated the usefulness of near infrared spectroscopy (NIRS) for detecting cerebral ischemia during a carotid endarterectomy; however, it is difficult to apply NIRS to the diagnosis of ischemic stroke, since commercially available NIRS, which uses continuous-wave light, does not provide quantitative values of baseline hemoglobin (Hb) concentrations. In contrast, time-resolved near-infrared spectroscopy (TRS) permits quantitative measurement of Hb concentrations. We applied TRS to detection of cerebra vasospasm after subarachnoid hemorrhage (SAH). ⋯ Finally, TRS performed on day 1 after SAH revealed significantly higher CoSO2 than that of the controls (p = 0.048), but there was no significant difference in total Hb. TRS detected vasospasm by evaluating the CBO in the cortex and may be more sensitive than TCD, which assesses the blood flow velocity in the M1 portion. TRS may be useful for the diagnosis of ischemic events in stroke patients.
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Botulism is a neuroparalytic disease caused by neurotoxins produced by Clostridium botulinum. Food-borne botulism is a kind of exotoxin-caused food intoxication. Although this disease is rarely reported in Japan now, it is a cause of great concern because of its high mortality rate, and botulism cases should be treated as a public health emergency. ⋯ C. botulinum was also obtained from stool culture on the 17th and 50th disease days. Botulism is a curable disease if treated early. Although it is a rare condition, it should always be considered in the differential diagnosis of patients with rapid onset of cranial nerve and limb muscle palsies.