Abstract：
:The progression of clinical disease and serum creatine kinase (CK) levels in canine X-linked muscular dystrophy (CXMD) was studied in 7 dogs from birth to 12-14 months and in 18 dogs at varying intervals from birth to 8 weeks. One affected male was studied from age 3.5 to 6 years, and all pups were descendants of this dog. A lethal neonatal form was recognized in some pups. In the more typical form, clinical signs of stunting, weakness and gait abnormalities were evident by 6-9 weeks and were progressive, leading to marked muscle atrophy, fibrosis and contractures by 6 months. Serum CK levels were markedly elevated, such that affected pups could be identified by 1 week. CK values increased until 6-8 weeks, then plateaued at approx. 100 times normal. Affected females and beagle-cross dogs were less severely affected than large breed-cross dogs. In the 2 adult dogs with cardiac insufficiency CK levels had decreased to 5-15 times normal. These studies show that CXMD and Duchenne muscular dystrophy have striking phenotypic as well as genotypic similarities. In addition, these studies of CXMD suggest that in females and in smaller dogs the same genetic defect results in a less severe clinical disease.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Valentine BA,Cooper BJ,de Lahunta A,O'Quinn R,Blue JTdoi
10.1016/0022-510x(88)90206-7subject
Has Abstractpub_date
1988-12-01 00:00:00pages
69-81issue
1-3eissn
0022-510Xissn
1878-5883pii
0022-510X(88)90206-7journal_volume
88pub_type
杂志文章abstract：:Coronal images of position emission tomography (PET) by 18F-labelled deoxyglucose reconstructed from interpolated scan in Alzheimer's disease clearly indicated suppression of glucose metabolism in the parietal lobe and lateral part of the temporal lobes, compared to normal controls. The medial part of the temporal lob...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(91)90249-7
更新日期：1991-12-01 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 innervation of laryngeal muscle fibers was appraised in adult humans. Sixteen intrinsic laryngeal muscles were dissected during the autopsy of 4 adults (41-71 years old). Longitudinal serial frozen sections, 60 microm thick, of the whole muscles were double-stained for cholinesterase activity and axonal visualizat...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(97)05395-1
更新日期：1997-07-01 00:00:00
abstract：:To assess the role of CSF oligoclonal bands (OB) in determining the clinical outcome in patients with relapsing-remitting multiple sclerosis (RRMS) treated with IFN-beta, we carried out a retrospective, multicentre, observational study recruiting 209 RRMS patients from six MS centres from northern, central and souther...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jns.2006.01.004
更新日期：2006-05-15 00:00:00
abstract：:Increased activity of the MM isoenzyme of creatine kinase (CK) was found in plasma from Bar Harbor dystrophic mice of the 129/ReJ dy/dy strain when compared to the findings in non-dystrophic controls. Total plasma CK activity was only slightly increased in dystrophic animals but plasma from both normal and dystrophic ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(81)90055-1
更新日期：1981-07-01 00:00:00
abstract：:Motor neurone disease (MND) is a useful paradigm for many progressive disabling neurological disorders and serves as a particularly opposite model for the study of patients' responses to progressive and irreversible disability. We studied the progression of disability and the patients' perception of their health in a ...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/0022-510x(95)00062-7
更新日期：1995-05-01 00:00:00
abstract：:Electron microscopical (EM) studies were carried out on the retinas of 2-3-(baby), 12-, 14- and 21-28-day-old (adult) mice infected with avirulent (A774) Semliki Forest virus (SFV). Virions (mature virus), spherules and advanced stages of virus replication, cytopathic vacuoles type II (CPV II), were seen in the retina...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(88)90038-x
更新日期：1988-05-01 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：OBJECTIVE:We estimated the extent and pattern of cardiovascular autonomic dysfunction in dementia with Lewy bodies (DLB) as compared with that in Parkinson's disease (PD). METHODS:We performed meta-iodobenzylguanidine ((123)I-MIBG) scintigraphy of the heart and hemodynamic autonomic function testing using the Valsalva...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,收录出版
doi：10.1016/j.jns.2007.01.002
更新日期：2007-03-15 00:00:00
abstract：:Affective disorders and personality changes have long been considered pre-motor aspects of Parkinson's disease (PD). Many authors have used the term "premorbid personality" to define distinctive features of PD patients' personality characterized by reduced exploration of new environmental stimuli or potential reward s...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2017.01.013
更新日期：2017-03-15 00:00:00
abstract：:Respiratory failure accounts for the majority of deaths in amyotrophic lateral sclerosis (ALS) but only rarely is ALS diagnosed on the basis of respiratory insufficiency. We report four ALS patients presenting with acute respiratory failure. In three patients we have performed EMG needle examination of both hemidiaphr...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(96)00089-5
更新日期：1996-08-01 00:00:00
abstract：:The interictal hypometabolism in patients with focal epilepsy is usually regarded as stationary. In this study we investigated to which extent the hypometabolism may depend on the activity of the epileptic focus. In focal penicillin-induced epilepsy in rats the epileptic focus is hypermetabolic. This focus is accompan...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/0022-510x(94)90325-5
更新日期：1994-07-01 00:00:00
abstract：:We recorded saccadic, pursuit and fixation eye movements in patients (n = 5) with moderately advanced Huntington's disease (HD), offspring of HD patients (n = 22) and control subjects (n = 15), using the scleral sensor coil technique. Saccadic slowing was seen in all patients, no controls and (marginally) in a few at-...
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
abstract：:Mast cells (MC) have been implicated in the pathogenesis of experimental allergic encephalomyelitis (EAE). In order to further evaluate their role, several morphological and functional studies were performed. Semiquantitative counts of histological sections showed a significant reduction in MC numbers in EAE brains. I...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(94)90300-x
更新日期：1994-04-01 00:00:00
abstract：INTRODUCTION:The underlying pathophysiology of neurological complications in patients with hemolytic-uremic syndrome (HUS) remains unclear. It was recently attributed to a direct cytotoxic effect of Shiga toxin 2 (Stx2) in the thalamus. Conventional MRI of patients with Stx2-caused HUS revealed - despite severe neurolo...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.06.045
更新日期：2015-09-15 00:00:00
abstract：:A procedure for placing a crush lesion in the sciatic and tibial nerve of the rat based on anatomical landmarks is described. These crush lesions are used to study the process of regeneration of peripheral nervous tissue and the beneficial effects of melanocortins on speed and quality of nerve regeneration. A new prec...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(86)90109-7
更新日期：1986-07-01 00:00:00
abstract：OBJECTIVE:To determine the sensitivity and specificity of clonal immunoglobulin heavy chain gene rearrangement (IGHR) analysis in the distinction of benign and malignant lymphoproliferative diseases. METHODS:A retrospective analysis was conducted of patients in whom a malignant lymphoproliferative process was suspecte...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.05.044
更新日期：2006-09-25 00:00:00
abstract：:A 66-year-old man presented with right cerebellar infarction and ischemic lesions in the left dorsal thalamus and right upper parietal lobe. Angiography showed occlusion of the right internal carotid artery proximal to an ipsilateral proatlantal artery type I, 70% stenosis of the left internal carotid artery, and apla...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/0022-510x(93)90178-2
更新日期：1993-07-01 00:00:00
abstract：:There is significant evidence that the pathogenesis of several neurodegenerative diseases, including Parkinson's disease, Alzheimer's disease, Friedreich's ataxia (FRDA), multiple sclerosis and amyotrophic lateral sclerosis, may involve the generation of reactive oxygen species (ROS) and/or reactive nitrogen species (...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2005.03.012
更新日期：2005-06-15 00:00:00
abstract：:Guillain-Barré syndrome (GBS) is considered a monophasic disorder yet recurrences occur in up to 6% of patients. We retrospectively studied an Italian-Japanese population of 236 GBS and 73 Miller Fisher syndrome (MFS) patients and searched for factors which may be associated with recurrence. A recurrent patient was de...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.02.053
更新日期：2016-06-15 00:00:00
abstract：:Hemorheologic changes from silent to acute and chronic cerebral infarction have seldom been reported. We evaluated hemorheologic profiles of the whole blood viscosity, plasma viscosity and fibrinogen level in stroke at-risk patients with silent cerebral infarction, patients with acute or chronic cerebral lacunar infar...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/s0022-510x(96)05309-9
更新日期：1997-03-20 00:00:00
abstract：OBJECTIVE:This study used reaction time (RT) as an objective marker of cognitive fatigue and fatigability in patients with multiple sclerosis (MS). METHOD:RT was measured in fifteen healthy controls and in thirty MS patients with cognitive fatigue identified with the Fatigue Scale for Motor and Cognitive Function (FSM...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.03.024
更新日期：2014-05-15 00:00:00
abstract：:We review post-infectious and post-vaccination neurological syndromes involving peripheral and central nervous system (CNS) and report an illustrative case of simultaneous occurrence of Guillain-Barré syndrome (GBS), confirmed by nerve conduction velocities, plus MRI-demonstrated transverse myelitis (TM) and acute enc...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2018.09.028
更新日期：2018-12-15 00:00:00
abstract：:Medial temporal lobe epilepsy is commonly associated with hippocampal atrophy on MRI and hippocampal sclerosis on histopathological examination of surgically-resected specimens. Likewise, it is well-established that prolonged seizures and status epilepticus can lead to hippocampal edema as noted on MRI. In this paper,...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.08.016
更新日期：2013-11-15 00:00:00
abstract：BACKGROUND:Mild cognitive impairment (MCI) is a well-known precursor of Alzheimer's disease (AD) but often also precedes dementia with Lewy bodies (DLB). The early differentiation of DLB from AD is important to delay disease progression. Olfactory dysfunction is a well-known early sign of both AD and Lewy body disorder...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.06.013
更新日期：2015-08-15 00:00:00
abstract：:The analysis of the firing intervals of motor units has been suggested as a diagnostic tool in patients with neuromuscular disorders. Part of the increase in number of turns seen in patients with myopathy could be secondary to the decrease in motor unit firing intervals at threshold force of the motor units, as noted ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(87)90077-3
更新日期：1987-03-01 00:00:00
abstract：BACKGROUND:Tumefactive demyelinating lesions (TDLs) are associated with a variety of demyelinating diseases in the central nervous system (CNS). However, there are no current guidelines describing how to classify and treat patients with this rare phenotype. Thus, the present study aimed to determine the long-term evolu...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.08.034
更新日期：2015-11-15 00:00:00
abstract：PURPOSES:The control of spasticity is often a significant problem in the management of patients with stroke. The aim of this study was to evaluate the effect of acupuncture treatment on the spastic states of stroke patients. SETTING:An outpatient Acupuncture Department in the First Teaching Hospital of Tianjin Univers...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章,随机对照试验
doi：10.1016/j.jns.2008.09.018
更新日期：2009-01-15 00:00:00
abstract：:Parkinson's disease (PD) is a common disorder of the central nervous system in the elderly. The pathogenesis of PD is a complex process, with genetics as an important contributing factor. This factor may stem from mitochondrial gene variations and mutations as well as from nuclear gene variations and mutations. More r...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2014.07.067
更新日期：2014-11-15 00:00:00
abstract：BACKGROUND:Tourette syndrome (TS) is a neurodevelopmental condition characterised by multiple tics and co-morbid behavioural problems. Previous research found that up to 76% of patients with TS experience affective symptoms, with 13% fulfilling diagnostic criteria for depression. OBJECTIVES:We aimed to assess the seve...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jns.2016.03.030
更新日期：2016-05-15 00:00:00
