Abstract:
:Two groups of "mdx" mice, totalling 36 animals of both sexes aged between 8 and 30 weeks, have been studied. In the first group of 10 males and 10 females, 8 males (at 8, 12, 20 and 30 weeks) and 4 females (at 12 and 30 weeks) showed severe limb muscle degeneration and inflammation with prominent regeneration and central nucleation of myofibres; fibrosis and fatty infiltration were not a feature. Five males (at 8, 20 and 30 weeks) and 2 females (at 30 weeks) also showed myocardial necrosis and inflammation. In the second group of 8 males and 8 females only 1 mouse, female at 25 weeks, showed similar changes including the myocardial lesion. Three females showed only focal myopathic changes. All the remaining animals in both groups were normal. These findings in terms of the severity and persistence of the myopathy and the myocardial lesion, not hitherto noted in mdx, suggest that it may be of value as an animal model of Duchenne muscular dystrophy (DMD) and/or other human muscle diseases. The variability probably reflects a failure to preserve an homozygous strain.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Bridges LRdoi
10.1016/0022-510x(86)90003-1subject
Has Abstractpub_date
1986-02-01 00:00:00pages
147-57issue
2-3eissn
0022-510Xissn
1878-5883pii
0022-510X(86)90003-1journal_volume
72pub_type
杂志文章abstract:OBJECTIVE:Stiff person syndrome (SPS) is usually characterized by truncal muscle rigidity and episodic painful spasms, but it sometimes appears with ocular symptoms called "stiff eyes". We recorded saccade movements in an SPS patient manifesting with "stiff eyes" conditions with slow saccade velocity and evaluated the ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2019.116506
更新日期:2020-01-15 00:00:00
abstract::Genetic, biochemical and morphological investigations were conducted on skeletal muscle mitochondria from 6 cases of ocular myopathy: 4 cases with Kearns-Sayre syndrome (KSS) and 2 with chronic progressive external ophthalmoplegia. All of these 6 cases showed mitochondrial DNA (mtDNA) deletions in addition to normal s...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(91)90042-6
更新日期:1991-02-01 00:00:00
abstract:OBJECTIVE:To report the first histopathologic description of optic nerve demyelination from tacrolimus (FK 506) toxicity in the absence of toxic levels of tacrolimus in a patient presenting with asymmetric bilateral visual loss after 5 years of tacrolimus therapy. PATIENTS:We report a patient status post cardiac and r...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2010.10.014
更新日期:2011-02-15 00:00:00
abstract:BACKGROUND:Cognitive impairment (CI) is frequently observed in Parkinson's disease (PD) and negatively influences the patient's and carer's quality of life. As a first step, assessment of CI is often accomplished by using screening instruments (level I diagnosis). Three commonly used instruments are the Mini-Mental Sta...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.116735
更新日期:2020-05-15 00:00:00
abstract::We describe a 54-year-old diabetic woman who developed ischemic optic neuropathy followed by acute retinal necrosis and multiple areas of focal venous beading. Vitreous fluid contained amplifiable VZV DNA but not HSV-1, CMV or toxoplasma DNA. The clinical presentation was remarkable for jaw claudication and intermitte...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.12.021
更新日期:2013-02-15 00:00:00
abstract::129 patients aged 18-84 in early rehabilitation period of the first hemispheric ischemic stroke and 48 patients with cerebrovascular disorders without stroke in anamnesis were observed. Cognitive functions (MMSE, FAB, Clock Drawing Test, the 5-Word test, attention assessment, Semantic Verbal Fluency test) and emotiona...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2012.11.013
更新日期:2013-02-15 00:00:00
abstract::The 3243A>G mutation of mtDNA usually is associated with MELAS syndrome. Here we report a patient with the 3243A>G mutation presenting only recurrent muscle fatigue and elevated levels of serum creatine kinase (CK). The mother of the proband was referred to us for type 2 diabetes mellitus, muscle pain and sensorineura...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.01.010
更新日期:2014-03-15 00:00:00
abstract:OBJECTIVE:HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) can progress slowly or rapidly even though a set of symptoms such as spastic paraparesis with pathological reflexes and sweating loss of the lower extremities are commonly observed in patients. Although most of the patients are thought to be ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.10.030
更新日期:2016-12-15 00:00:00
abstract::The cerebrospinal fluid tap test (CSFTT) is widely used to diagnose idiopathic normal pressure hydrocephalus (iNPH) and predict the therapeutic effectiveness of shunting. However, the ability to walk cannot be quantified for patients who are unable to walk. Therefore, we examined whether the iNPH diagnostic aid is pos...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.10.009
更新日期:2016-12-15 00:00:00
abstract::On December 31, 1988 there were 201 registered multiple sclerosis patients in Kuwait, an overall prevalence rate (PR) of 10.2 per 100,000; among them were 186 Arabs, of whom 72 were Palestinians and 51 Kuwaitis. Comparison of these two subgroups, who had a similar age distribution revealed that the disease was 2 1/2 t...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(90)90024-h
更新日期:1990-12-01 00:00:00
abstract::Zika virus infection represents a new neuropathological agent with association to a wide spectrum of neurological complications: a) Congenital Zika Syndrome by affecting the neural stem cells of the human fetal brain; b) Guillain-Barré Syndrome by an autoimmune response against peripheral myelin and/or axonal componen...
journal_title:Journal of the neurological sciences
pub_type: 社论
doi:10.1016/j.jns.2017.10.046
更新日期:2017-12-15 00:00:00
abstract:BACKGROUND:The COVID-19 pandemics required several changes in stroke management and it may have influenced some clinical or functional characteristics. We aimed to evaluate the effects of the COVID-19 pandemics on stroke management during the first month of Italy lockdown. In addition, we described the emergency struct...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.116889
更新日期:2020-07-15 00:00:00
abstract::The ability to accurately navigate within an environment (known or new) is not fully understood but involves a number of highly complicated cognitive process related to both central and peripheral nervous system structures and neuronal networks. We describe a patient who developed loss of his spatial navigational skil...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2011.06.026
更新日期:2011-09-15 00:00:00
abstract::Gulf War veterans meeting criteria for Haley Syndrome 2 of Gulf War illness endorse a particular constellation of symptoms that include difficulty with processing information, word-finding, and confusion. To explore the neural basis of their word-finding difficulty, we assessed event-related potentials (ERPs) associat...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2016.12.023
更新日期:2017-02-15 00:00:00
abstract::The glucocorticoid methylprednisolone (Mepd) increased dystrophin and myosin heavy chain levels in differentiated cultures of cloned human myoblasts. Mepd increased the number of myotubes per area by preventing myotube death and detachment during myogenesis in vitro. Myotube death was the result of an endogenous proce...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(91)90019-4
更新日期:1991-01-01 00:00:00
abstract::In view of recent reports on lower brainstem dysfunction in Huntington's chorea, we studied respiration during sleep in 12 patients with Huntington's chorea (HC) and in controls. There were no statistically significant differences between patients and controls with respect to apnea periods, respiratory frequency and t...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(88)90174-8
更新日期:1988-03-01 00:00:00
abstract:BACKGROUND:Neurological disorders are common in sub-Saharan African, but accurate neuroepidemiologic data are lacking from the region. We assessed a neuroepidemiological screening tool in a rural Ugandan cohort with high HIV prevalence. METHODS:Participants were recruited from the Rakai Neurology Study in rural Rakai ...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2020.117273
更新日期:2020-12-24 00:00:00
abstract::The histochemical ATPase fibre type pattern was examined in muscle biopsy samples obtained from patients with recessive myotonia, paramyotonia and from one patient with dominant myotonia. Absence (less than or equal to 5%) of 2B fibres was a genuine finding in the minority of the cases. In additional cases of recessiv...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(86)90059-6
更新日期:1986-03-01 00:00:00
abstract:OBJECTIVE:Neurodegenerative diseases are often characterized by the presence of intracellular or extracellular protein aggregates in the central nervous system. Mutations of TARDBP gene have been shown to cause Amyotrophic Lateral Sclerosis and have been reported to present with clinical heterogeneity including parkins...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2018.04.043
更新日期:2018-07-15 00:00:00
abstract::Anhedonia is one of the non-motor symptoms observed in the Parkinson's disease (PD). However, there is no clear relationship between anhedonia and its correlation with other symptoms of PD. The aim of this study is to evaluate the characteristics of anhedonia and its correlation with clinical aspects of PD in a relati...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,多中心研究
doi:10.1016/j.jns.2016.10.051
更新日期:2017-01-15 00:00:00
abstract::Hypnic headache (HH) is a rare benign disorder described initially by Raskin in 1988. It is characterized by recurrent nocturnal episodes of headache that periodically awaken the sleeping patient and usually occur in the elderly. This review is aimed at describing the clinical features of the HH cases in childhood tha...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2015.06.048
更新日期:2015-09-15 00:00:00
abstract::Despite apparent relationships between ischemic stroke and the acute phase response (APR), considerable variation in the APR exists between individuals. We therefore performed post-hoc analysis of individual APR profiles in 31 patients with ischemic stroke in relation to volume of brain infarction. Patients with ische...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2006.09.011
更新日期:2006-12-21 00:00:00
abstract::In recent years it has become clear that multiple sclerosis (MS) patients benefit from physical exercise as performed in aerobic training but little is known about the effect on functional domains and physiological factors mediating these effects. We studied immunological, endocrine and neurotrophic factors as well as...
journal_title:Journal of the neurological sciences
pub_type: 临床试验,杂志文章
doi:10.1016/j.jns.2004.06.009
更新日期:2004-10-15 00:00:00
abstract::Increasing evidence shows that exposure to an enriched environment (EE) after cerebral ischemia/reperfusion injury has neuroprotective benefits in animal models, including enhancing functional recovery after ischemic stroke. However, the mechanism underlying this effect remains unclear. To clarify this critical issue,...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2014.10.022
更新日期:2014-12-15 00:00:00
abstract::We investigated whether the reduced intramembranous particles (IMP) in the muscle plasma membrane in mdx mice reflects a preferential depletion of a particular size of the IMP. The experiments were performed using the freeze-fracture method to analyze the frequency distribution of the size of IMP, the density of ortho...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/s0022-510x(96)05350-6
更新日期:1997-05-29 00:00:00
abstract::Quantitative analysis of motor unit potentials (MUPs) with spike triggering and averaging was performed from the mildly affected brachial biceps muscle of 18 patients with amyotrophic lateral sclerosis (ALS) and of 18 age-matched controls. The total MUP duration was only 6% higher in ALS patients than in controls (P l...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/0022-510x(90)90242-f
更新日期:1990-02-01 00:00:00
abstract::Myotonic Dystrophy Type 1 (DM1) in combination with demyelinating neuropathy is very rare in literature. In this study, DM1 and demyelinating neuropathy were demonstrated clinically and electromyographically in a 43-year-old female patient from Turkey. In the patient an expanded CTG repeat in the Myotonic Dystrophy Pr...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2009.09.028
更新日期:2010-01-15 00:00:00
abstract::In this paper we review the findings of magnetic resonance imaging (MRI) in optic neuritis and visual dysfunction due to other optic neuropathies. With advances in MRI technology, it has become possible to visualise optic nerve pathology. STIR and RARE sequences, contrast-enhanced sequences, and phased array surface c...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/s0022-510x(99)00272-5
更新日期:2000-01-15 00:00:00
abstract:BACKGROUND:There is little information about intracranial atherosclerotic disease (ICAD) and extracranial atherosclerotic disease (ECAD) in patients with acute spontaneous intracerebral hemorrhage (ICH). The purpose of the present study was to elucidate the prevalence of and the factors that correlate with ICAD and ECA...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章
doi:10.1016/j.jns.2013.06.031
更新日期:2013-09-15 00:00:00
abstract::The aim of this study was to review the epidemiological and clinical characteristics of neuromyelitis optica (NMO) and the immunopathological mechanisms involved in the neuronal damage. NMO is an inflammatory demyelinating autoimmune disease of the central nervous system that most commonly affects the optic nerves and...
journal_title:Journal of the neurological sciences
pub_type: 杂志文章,评审
doi:10.1016/j.jns.2015.05.034
更新日期:2015-08-15 00:00:00