Abstract：
:We examined 19 muscle biopsies from 14 patients with autosomal recessive distal muscular dystrophy (DisMD) histochemically and immunohistochemically to characterize the histologic features of this disease and to determine whether dystrophin and dystrophin-associated proteins (DAPs) are normally present in the muscles of patients with this disorder. The common histologic features in DisMD were active muscle fiber necrosis and regeneration with variation in fiber size, predominantly in the gastrocnemius muscle. There were occasional disorganizations of the intermyofibrillar network such as moth-eaten appearance, lobulated, whorled and targetoid fibers. In one half of the patients, small angular fibers and scattered rimmed vacuoles were also found. On immunohistochemical examination, dystrophin, DAPs, spectrin and laminin were normally expressed along the surface membrane of muscle fibers, even in the advanced stages of the disease. In contrast, dystrophin was absent and DAPs reduced in the sarcolemma of Duchenne muscular dystrophy (DMD) muscles. The overall histochemical features in DisMD were similar to those seen in DMD, though dystrophin and DAPs were normally expressed even in severely affected gastrocnemius muscle. A defect in an as yet unidentified protein rather than in DAPs and dystrophin is probably responsible for the muscle fiber necrosis in DisMD.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Yamanouchi Y,Ozawa E,Nonaka Idoi
10.1016/0022-510x(94)90096-5subject
Has Abstractpub_date
1994-10-01 00:00:00pages
70-6issue
1eissn
0022-510Xissn
1878-5883pii
0022-510X(94)90096-5journal_volume
126pub_type
杂志文章abstract：:Experimental autoimmune encephalomyelitis (EAE) is an organ-specific autoimmune disease characterised by inflammation and demyelination of the central nervous system and is the best available animal model of multiple sclerosis (MS). Since previous studies have shown that EAE is less severe or is delayed in onset durin...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(03)00212-0
更新日期：2003-12-15 00:00:00
abstract：PURPOSE:To investigate the direct and indirect associations of sleep hygiene with daytime sleepiness, depressive symptoms, and quality of life (QoL), in newly diagnosed, untreated patients with mild obstructive sleep apnea (OSA). METHODS:Data were collected from adults with mild OSA. The Sleep Hygiene Index (SHI), Sle...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.10.017
更新日期：2015-12-15 00:00:00
abstract：:Electron transport chain (ETC) dysfunction has been claimed to contribute to the expression of neurodegenerative disorders. We have investigated the effects of the treatment with rivastigmine, a commonly used cholinesterase inhibitor, on lymphocyte mitochondria of patients with Alzheimer's disease (AD). Increased enzy...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/s0022-510x(02)00319-2
更新日期：2003-01-15 00:00:00
abstract：:The authors have shown in a recent paper that survival with amyotrophic lateral sclerosis (ALS) can be increased by the use of non-invasive methods of assisted ventilation (Bipap). However, the progression of muscle weakness was not affected and the quality of life was not positively enhanced. In ALS, reduced physical...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/s0022-510x(99)00218-x
更新日期：1999-10-31 00:00:00
abstract：:The present study was performed to investigate the effects of Ca(2+)/calmodulin-dependent protein kinase II (CaMKII) antisense oligodeoxynucleotides (ODNs) on the assembly of the CaMKII·GluR6·PSD-95 signaling module, GluR6 serine phosphorylation and c-Jun N-terminal kinase 3 (JNK3) activation. A further aim was to det...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.10.012
更新日期：2012-03-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.10.009
更新日期：2007-01-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2008.11.023
更新日期：2009-03-15 00:00:00
abstract：:Baló's concentric sclerosis (BCS) and tumefactive demyelination (TD) are considered atypical forms of multiple sclerosis (MS). Baló lesions are characterized by concentric rings corresponding to alternating bands of demyelination and relatively preserved myelin (Hu and Lucchinetti, 2009). Tumefactive lesions are pseud...
journal_title：Journal of the neurological sciences
pub_type： 信件
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更新日期：2015-01-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.09.007
更新日期：2013-12-15 00:00:00
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journal_title：Journal of the neurological sciences
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(88)90107-4
更新日期：1988-09-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/s0022-510x(99)00282-8
更新日期：2000-01-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(77)90005-3
更新日期：1977-01-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/s0022-510x(98)00049-5
更新日期：1998-04-15 00:00:00
abstract：:Post-stroke epilepsy (PSE) is defined as "recurrent seizures following stroke with confirmed diagnosis of epilepsy". Our objective was to describe the epidemiological, clinical and therapeutic PSE aspects at the Yalgado Ouedraogo Teaching Hospital, the main reference centre for neurological conditions in Burkina Faso....
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.06.038
更新日期：2016-09-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2007.08.030
更新日期：2008-03-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：
更新日期：1996-07-01 00:00:00
abstract：:Nitrous oxide is routinely administered to children and adults with Charcot-Marie-Tooth disease (CMT) as an anaesthetic for procedures such as nerve conduction studies and maintenance for general anaesthesia. However it is listed as a 'moderate to significant' risk of potential toxicity and worsening neuropathy in peo...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2007.12.004
更新日期：2008-05-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/s0022-510x(99)00250-6
更新日期：1999-12-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2005.05.005
更新日期：2005-10-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(76)90250-1
更新日期：1976-11-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.01.024
更新日期：2012-05-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(84)90154-0
更新日期：1984-03-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2019.01.023
更新日期：2019-03-15 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(96)05323-3
更新日期：1997-05-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章,多中心研究,随机对照试验
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更新日期：2006-12-21 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(93)90318-s
更新日期：1993-06-01 00:00:00
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journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(92)90204-x
更新日期：1992-01-01 00:00:00
abstract：:The cause of multiple sclerosis is unknown although it is recognised to involve an inflammatory process associated with demyelinating plaques and more widespread neurodegeneration. It appears to have become progressively more common in females which is further discussed in this issue, and genetic factors, as identifie...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2009.09.009
更新日期：2009-11-15 00:00:00
abstract：:Recently it has been reported that the C57BL mice can be used as a model of benign monoclonal gammopathy (MG). Since experimental models have until now failed to reproduce specific lesions of human dysglobulinemic polyneuropathies, we decided to investigate the peripheral nerve of these mice. The sciatic nerve and the...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(87)90188-2
更新日期：1987-10-01 00:00:00
