Brain and nerve 

About: Brain and nerve is an academic journal published by Igaku Shoin. The journal publishes majorly in the area(s): Medicine & Dementia. It has an ISSN identifier of 1344-8129. Over the lifetime, 1533 publications have been published receiving 6944 citations. The journal is also known as: Shinkei kenkyuu no shimpo.

Cognitive impairment in multiple sclerosis

Abstract: Cognitive impairment may occur in up to 70% of all patients with multiple sclerosis (MS). Although MS can affect various sites within the central nervous system, a specific pattern of cognitive deficits tends to be seen, especially in the early stages of the disease. These deficits include problems with attention, information processing speed, and working memory. This constellation of deficits can occur with any disease course, and a minimal correlation has been found between physical disability assessed by EDSS and cognitive impairment. Many studies have shown that cognitive impairment is correlated with brain lesion volume, as well as brain atrophy. There are promising neuroimaging indicators that may be useful for identifying patients at risk for cognitive impairment, such as diffusion tensor imaging, the magnetization transfer ratio, and N-acetyl aspartate levels. Cognitive dysfunction is associated with adverse effects on quality of life, employment status, and social activities. Today, there are three avenues for treatment: disease modifying therapies, symptomatic treatments, and cognitive rehabilitation. Unfortunately, data linking therapeutic interventions are limited. A better understanding of cognitive function and its correlation with disease mechanisms will assist in providing a new comprehensive treatment strategy that begins immediately with the diagnosis of MS.

The diagnosis of stupor and coma

Abstract: Advances in the knowledge and understanding of stupor and coma, aided by the considerable impact of CT scanning on neurological diagnosis, are reflected in this substantially revised edition. The authors have included new references and illustrations, but the third edition retains its approach to that art of diagnosis based on the understanding of pathophysiology and pathobiochemistry.

Pathophysiology of spasticity

Abstract: Spasticity is characterized by abnormal increase in phasic and velocity-dependent response to muscle stretching, such as exaggerated tendon jerks and clonus. Clasp-knife phenomenon is often observed in the affected muscles. Spasticity is caused by either an excess input to the spinal cord or a disordered descending control of the spinal motor system including the myotatic reflex arc. The increased input due to hyperactivity of γ motor neurons has not been demonstrated in human subjects. The hyperactivity of excitatory interneurons and decreased function of the inhibitory system interneurons in the spinal cord as well as the uncoupling of the inhibitory system from the central system are believed to be involved in spasticity; however, the underlying pathology may differ based on the lesion site and the time elapsed since loss of reciprocal inhibition.

The role for oxidative stress in neurodegenerative diseases

Abstract: A growing body of evidence suggests oxidative stress involvement in neurodegenerative diseases; however, it remains to be determined whether oxidative stress is a cause, result, or epiphenomenon of the pathological processes. This review concerns the current issue, focusing on Alzheimer disease (AD), Parkinson disease (PD), and amyotrophic lateral sclerosis (ALS). Several studies have indicated that oxidative stress initially occurs in the disease-specific, site-restricted sources such as amyloid-beta in the cerebral cortex of AD brain, alpha-synuclein in the brain stem of PD brain, and glutamate receptor-coupled Ca2+ channel in the motor system of ALS spinal cord. Subsequent events in the neurons common to these diseases are glutamate-induced neurotoxicity and increased cytosolic Ca2+ levels, resulting in activation of Ca2+ -dependent enzymes including NADPH oxidase, cytosolic phospholipase A2, xanthine oxidase, and neuronal nitric oxide synthase (NOS). These enzymes produce reactive oxygen and nitrogen species (ROS/RNS), which oxidatively modify nucleic acid, lipid, sugar, and protein, leading to nuclear damage, mitochondrial damage, proteasome inhibition, and endoplasmic reticulum (ER) stress. Mitochondrial damage results in both ROS leakage from the electron transport system and Ca2+ release. Nuclear damage induces p53 activation, and proteasome inhibition reduces p53 degradation. The resultant increased p53 levels in the nucleus induce Bax activation and Bcl-2 inhibition, followed by a release of cytochrome c into the cytosol that truncates procaspase-9. ER stress triggers activation of caspase-12 as well as caspase-9 via the tumor necrosis factor (TNF) receptor-associated factor-2 / apoptosis-signaling kinase-1 / c-Jun N-terminal kinase pathway. Oxidative stress also stimulates astrocytes and microglia to yield and secrete cytokines such as TNFa and FasL that cause not only neuronal caspase-8 activation but also glial inflammatory response through induction of nuclear factor-kappaB-mediated, proinflammatory gene products including cytokines, chemokines, growth factors, cell adhesion molecules, and ROS/RNS-producing enzymes. The activated caspases truncate procaspase-3 to exert classical apoptosis. Moreover, oxidative DNA damage leads to the release and nuclear truncation of mitochondrial apoptosis-inducing kinase, which triggers apoptosis-like programmed cell death via cyclophilin A. These observations could indicate crucial implications for oxidative stress in several steps of the pathomechanisms of neurodegenerative diseases.

[Collagen VI-related muscle disorders].

Abstract: 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 Bethlem myopathy is usually inherited in an autosomal dominant manner, but a rare autosomal recessive inheritance has recently been reported Patients with UCMD have generalized muscle weakness, multiple contractures of the proximal joints, and hyperextensibility of the distal joints Bethlem myopathy is characterized by a combination of proximal muscle weakness and contractures of finger, elbow, and ankle joints Because intermediate phenotypes occur, UCMD and Bethlem myopathy should be considered diseases in a continuous spectrum of collagen VI-related muscle disorders Abnormalities of cell adhesion, regeneration, mitochondrial permeability transition pore, and autophagy have been reported in UCMD Respiratory surveillance for nocturnal hypoventilation and proper respirator implementation are crinical management considerations in UCMD Orthopedic assessment in necessary if surgery for Achilles tendon contractures is being considered in patient with Bethlem myopathy 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