Abstract：
:An unusual fatal congenital myopathy in a Chinese female infant is described. Muscle biopsy showed type I fibre smallness with central nuclei and focal decrease in oxidative enzyme activities affecting mainly larger type II fibres. Longitudinal sections from glutaldehyde-fixed araldite-embedded material stained with toluidine blue revealed multiple small foci of myofibrillar degeneration (multicores) along the muscle fibres. Electron-microscopic examination confirmed the presence of multicore lesions affecting mainly the larger fibres. In addition, there were definite degenerative changes involving the smaller fibres with central nuclei. The degenerative process started around the pericentronuclear zones with diffuse extension along the whole length of the muscle fibres resulting in severe atrophy. These degenerative changes were similar to those described in pericentronuclear myopathy. It is therefore suggested that the patient might have either had 2 co-existing myopathies viz. multicore disease and pericentronuclear myopathy or a single entity with combinations of features which had not hitherto been described.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Lee YS,Yip WCdoi
10.1016/0022-510x(81)90173-8subject
Has Abstractpub_date
1981-05-01 00:00:00pages
277-90issue
2eissn
0022-510Xissn
1878-5883pii
0022-510X(81)90173-8journal_volume
50pub_type
杂志文章abstract：:Bradykinesia is a disabling symptom of Parkinson's disease (PD) which presents with slowness of movement. Visual assessment using clinical rating scales is currently the gold standard to assess bradykinesia. Such assessments require multiple separate movements, are subjective, and rely on the ability of the rater to d...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.07.056
更新日期：2016-09-15 00:00:00
abstract：:The nosological relationship between Bickerstaff s brainstem encephalitis (BBE) and Guillain-Barre syndrome (GBS) has yet to be clarified. We report results of the autopsy of a patient for whom overlapping BBE and GBS was diagnosed clinically. This and similar cases support the original authors' hypothesis that BBE is...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(96)00251-1
更新日期：1997-01-01 00:00:00
abstract：:Encephalitogenic activity of myelin basic protein (MBP) isolated in a form retaining binding to all myelin lipids was tested in Lewis rats. Immunization with this new stable lipid-bound and native-like preparation (LB-MBP), induced experimental autoimmune encephalomyelitis (EAE) as intensively as the classical lipid f...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(93)90196-6
更新日期：1993-10-01 00:00:00
abstract：:Sensory ataxia in inorganic germanium intoxication is rare. A 63-year-old housewife had taken inorganic germanium preparations at a dosage of 36 mg a day for about 6 years (total dose about 80 g). She subsequently developed difficulty in writing and gait disturbance with peripheral neuropathy and renal involvement. Ge...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(95)00032-w
更新日期：1995-06-01 00:00:00
abstract：:Room tilt illusion, a misperception that the entire room is tilted, is a rare but nonspecific neurological symptom. We report a patient with Parkinson's disease who developed typical room tilt illusion. Single photon emission computed tomography demonstrated hypoperfusion of the posterior area of the right intrapariet...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.08.054
更新日期：2009-12-15 00:00:00
abstract：BACKGROUND:Entrapment neuropathy of the radial nerve at the spiral groove region is relatively common. However, its localization may be technically challenging. OBJECTIVE:To evaluate the use of ultrasound (US), in relation to electrophysiological testing, for this purpose. METHODS:We studied 32 normal controls to obt...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2008.03.014
更新日期：2008-08-15 00:00:00
abstract：:The activities of the two polyamine biosynthetic decarboxylases (PBD), L-ornithine decarboxylase (ODC) and S-adenosyl-L-methionine decarboxylase (SAMD), have been measured in quadriceps femoris of rats killed at different times after the induction of calciphylaxis- or serotonin(5-HT)-induced myopathy. Decreases in bot...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(89)90004-x
更新日期：1989-01-01 00:00:00
abstract：:A sensitive enzyme immunoassay (EIA) micromethod is described which can measure levels of a 14 kDa human brain lectin (HBL) in the cerebrospinal fluid (CSF) of patients submitted to CSF examination. The assay is based on the use of a polyclonal antibody to HBL and the simultaneous application of biotinylated and unlab...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(91)90110-s
更新日期：1991-09-01 00:00:00
abstract：:Outcome after aSAH depends on several factors, including the severity of the initial event, perioperative medical management, surgical variables, and the incidence of complications. Cerebral vasospasm (CV) is ure to consistently respond to treatment, emphasizing the need for further research into the underlying mechan...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2007.04.046
更新日期：2007-10-15 00:00:00
abstract：:Brainstem gliomas constitute 10% of brain tumors in children and less than 2% in adults. Since therapeutic options are limited and brainstem gliomas are associated with a high morbidity and mortality, we sought to analyze the prognostic factors associated with a better outcome. We reviewed the records of 86 patients w...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.03.009
更新日期：2009-06-15 00:00:00
abstract：BACKGROUND:Differential diagnosis between idiopathic Parkinson's disease (PD) and multiple system atrophy (MSA) is often difficult in early disease stages. Since MSA is misdiagnosed as PD in more than 20% of the early stages, there is need for methods refining the differentiation of the two disease entities. In PD post...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(00)00279-3
更新日期：2000-04-01 00:00:00
abstract：BACKGROUND:We recently reported that left versus right hemisphere cerebral infarctions patients more frequently have worse outcomes. However our clinical experience led us to suspect that the incidence of malignant middle cerebral artery infarctions (MMCA) was higher in the right compared to the left hemispheric stroke...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2015.04.036
更新日期：2015-01-01 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：:We study a patient with extrapontine myelinolysis (EPM), without central pontine myelinolysis (CPM), who exhibited changes in abnormalities observed with MRI. MRI in the acute stage of the bilateral basal ganglia showed a high signal intensity on T2-weighted images, and a low signal on T1-weighted images. The low sign...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00275-5
更新日期：1998-12-11 00:00:00
abstract：BACKGROUND:The role of subthreshold depression (subD) in Parkinson's Disease (PD) is not clear. The present study aimed to compare the quality of life (QoL) in PD patients with subD vs patients with no depressive disorder (nonD). Factors related to subD were identified. MATERIAL AND METHODS:PD patients and controls re...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2020.117109
更新日期：2020-11-15 00:00:00
abstract：OBJECTIVES:Several studies have established the relevance of S-100 in blood as a marker of brain damage after traumatic brain injury. However, a more specific marker is required and glial fibrillary acidic protein (GFAP) is considered to be a good candidate. METHODS:In order to assess the increase of GFAP in serum (s-...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2005.09.007
更新日期：2006-01-15 00:00:00
abstract：BACKGROUND:Tele-rehabilitation after stroke holds promise for under-resourced settings, especially sub-Saharan Africa (SSA), with its immense stroke burden and severely limited physical therapy services. OBJECTIVE:To preliminarily assess the feasibility and outcomes of mobile technology-assisted physical therapy exerc...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2018.01.039
更新日期：2018-04-15 00:00:00
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：:We present an uncommon case of a 38-year-old man presented with bilateral subacute weakness of intrinsic hand muscles, manifesting as bilateral claw-hand, without sensory deficits and absent tendon reflexes in upper arms. Nerve conduction studies showed findings consistent with demyelinating GBS. During the fourth day...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.07.009
更新日期：2013-11-15 00:00:00
abstract：:Though the core symptoms of Parkinson's disease (PD) are motor-related, a majority of patients also have neuropsychiatric symptoms concerning mood, behavior and cognition. Apathy and depression are considered among the most frequent ones, and have a negative impact on global functioning and quality of life. Recent neu...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2011.07.006
更新日期：2011-11-15 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：:Over two decades, a 42-year old woman experienced the gradual onset of choreic involuntary movements, dystonia, and tics. Decreased caudate nucleus metabolism on 2-deoxyglucose PET scan and a heterozygous 49-CAG repeat expansion within the HTT gene established the diagnosis of HD, although no other family history was ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.08.034
更新日期：2012-12-15 00:00:00
abstract：:The aim of the study was to investigate glucose derangement and its short- and long-term prognostic significance in nondiabetic ischemic stroke patients. The study involved 262 consecutive patients, mean age: 70.1+/-12.4 years, with a supratentorial ischemic stroke. The following data were collected: patients characte...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(01)00566-4
更新日期：2001-08-15 00:00:00
abstract：:Shapiro syndrome is a rare entity, comprising a triad of recurrent hypothermia, hyperhidrosis and congenital agenesis of the corpus callosum. Fewer than 50 cases have been described, almost invariably in patients presenting in childhood or early adulthood. We present a case of an 80 year old woman presenting with recu...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.03.014
更新日期：2013-06-15 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：BACKGROUND:Allelic heterogeneity and phenotype variability-especially in age at onset, penetrance and progression-are reported in ALS1 families. For this reason, SOD1 gene mutation data in ALS1 patients are currently being gathered to better understand the genotype-phenotype relationship in this disorder. Here, we repo...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2003.10.018
更新日期：2004-04-15 00:00:00
abstract：:Antibodies to the myelin-associated glycoprotein (MAG) are implicated in the pathogenesis of an acquired demyelinating polyneuropathy. We studied IgM affinity to MAG in 18 patients with anti-MAG antibodies. Binding of sera was tested for anti-MAG immunoreactivity in central nervous system (CNS) by ELISA and in CNS and...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(02)00359-3
更新日期：2003-03-15 00:00:00
abstract：BACKGROUND:Motor impairment in Parkinson's disease (PD) includes slowness (bradykinesia), decreased amplitude (hypokinesia), impaired rhythm and a progressive reduction in speed and amplitude during movement repetition (sequence effect). In the present study we aimed to analyse bradykinesia features in newly-diagnosed ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2016.08.028
更新日期：2016-10-15 00:00:00
abstract：:The parotid glands and part of the submandibular glands were irradiated in 18 amyotrophic lateral sclerosis patients with very advanced disease and severe drooling problems. In a prospective study, a single dosage of 7.0-7.5 Gy was administered bilateral to the larger part of the parotid glands and the posterior parts...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章
doi：10.1016/s0022-510x(01)00631-1
更新日期：2001-10-15 00:00:00
abstract：OBJECTIVE:To investigate the effect of mild cognitive impairment (MCI) on balance. METHODS:87 subjects with subjective memory impairment were enrolled, and subdivided into two groups, MCI and non-MCI, according to diagnostic criteria of amnestic subtype of MCI according to the 1999 MCI international panel (Current Con...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.02.031
更新日期：2011-06-15 00:00:00
