Abstract：
:Two siblings are presented with late onset, rapidly progressive truncal ataxia, paralysis of down-gaze and loss of up-gaze saccades in association with other oculomotor dysfunctions as well as dementia. Electron microscopic muscle studies revealed abnormal distribution and form of the mitochondria, probably being the ultrastructural basis of the pathologic changes. A neurological syndrome as that described here has not been reported before.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
al-Din AS,al-Zuhair AG,al-Salem MK,al-Nassar KE,Rudwan MA,Khafaja S,Hamawi Tdoi
10.1016/0022-510x(89)90202-5subject
Has Abstractpub_date
1989-11-01 00:00:00pages
323-31issue
2-3eissn
0022-510Xissn
1878-5883pii
0022-510X(89)90202-5journal_volume
93pub_type
杂志文章abstract：BACKGROUND:Treatment options for neuromyelitis optica (NMO) are currently based on small retrospective case series and open label studies, ranging from 10 to 103 patients. OBJECTIVE:To compare the efficacy and tolerability of azathioprine, cyclophosphamide, mycophenolate, and rituximab in patients with neuromyelitis o...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.02.012
更新日期：2015-04-15 00:00:00
abstract：:The thyroid hormone thyroxine (T4), administered post-natally to neonatal rats, has been shown to accelerate development of auditory function, as expressed by auditory nerve-brainstem evoked responses. This study investigated whether this earlier development was also reflected in other sensory modalities. Rat pups wer...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(94)00229-h
更新日期：1995-02-01 00:00:00
abstract：:The gut microbiome is composed of an enormous number of microorganisms, generally regarded as commensal bacteria. Resident gut bacteria are an important contributor to health and significant evidence suggests that the presence of healthy and diverse gut microbiota is important for normal cognitive and emotional proces...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2019.03.027
更新日期：2019-05-15 00:00:00
abstract：:We report the case of a woman with primary progressive aphasia (PPA) presenting with conduction aphasia. Neurological findings showed bilateral finger tremor and signe de poignet figé in her right hand. Memory, orientation, and activities of daily living were well preserved. Linguistic examination showed severe impair...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2007.12.017
更新日期：2008-06-15 00:00:00
abstract：BACKGROUND:Patients with complaints of carpal tunnel syndrome (CTS) with signs and symptoms not exclusively confined to the median nerve territory, but otherwise fulfilling the clinical criteria may erroneously be withheld from therapy. METHODS:One hundred and twenty one patients who fulfilled the clinical criteria fo...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.06.044
更新日期：2014-09-15 00:00:00
abstract：:Findings in a sural nerve biopsy from a patient with rheumatoid neuropathy are reported. Inflammatory changes in different stages were observed in epineurial arterioles. Arterioles with fibrinoid necrosis contained IgG, IgM, rheumatoid factor and complement. The same substances were found at the inner site of the peri...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(77)90128-9
更新日期：1977-09-01 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：:We report here the results of a simple and reproducible technique which can be used in semi-routine analysis of peripheral nerve biopsy specimens, so as to have a quantitative analysis of the major lipid classes, i.e. cholesterol, cerebrosides, ethanolamine phospholipids, phosphatidyl-choline, phosphatidyl-serine + ph...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(79)90039-x
更新日期：1979-04-01 00:00:00
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...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(88)90206-7
更新日期：1988-12-01 00:00:00
abstract：:Mucopolysaccharidosis (MPS) type I (alpha-iduronidase deficiency) is characterized by storage and massive urinary excretion of dermatan sulfate and heparan sulfate; it may be distinguished into three different subtypes based on age at onset and severity of the clinical symptoms. We report on progressive white matter i...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(02)00014-x
更新日期：2002-03-30 00:00:00
abstract：:Alzheimer's disease (AD) is a complex disorder and the most common form of neurodegenerative dementia. Several genetic, environmental, and physiological factors, including inflammations and metabolic influences, are involved in the progression of AD. Inflammations are composed of complicated networks of many chemokine...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2017.03.031
更新日期：2017-05-15 00:00:00
abstract：:Immunologically pure IgG was isolated from serum of multiple sclerosis (MS) patients and normal controls. The F(ab)2 moiety of IgG was then isolated by salting out between 12.5 and 19.0% Na2SO4 of the pepsin digest of IgG. The ability of the 131I-labelled F(ab)2 to bind to subcellular brain fractions (nuclei, synaptos...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(83)90063-1
更新日期：1983-08-01 00:00:00
abstract：:Thirty patients with acute, unilateral optic neuritis (ON), where re-examination after a mean observation period of 5 years did not reveal any aetiology, were investigated with regard to laboratory abnormalities frequently observed in multiple sclerosis. Eleven patients had oligoclonal IgG in CSF. In 5 of these a meas...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(76)90237-9
更新日期：1976-01-01 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：:Psychiatric disorders seem to be more frequent in patients with epilepsy (PWE) than the general population. Although researchers have documented a strong association between epilepsy and psychiatric comorbidities, the nature of this relationship is poorly understood. According to this, psychiatric diseases are often u...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2014.05.043
更新日期：2014-08-15 00:00:00
abstract：:We report a novel heteroplasmic mutation p.Y440C in the mitochondrial DNA-encoded subunit I of the cytochrome c oxidase (COX) gene in a patient with late onset progressive painless weakness. Her muscle biopsy showed scattered COX-negative fibers and several small collections of inflammatory cells. The mutation was det...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.05.003
更新日期：2012-08-15 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：:A 53-year old woman developed slowly progressive dysarthria, mild enlargement of the tongue and dysphagia since 1 year ago. All neurological differential diagnoses that could have explained the bulbar symptoms were excluded. The swallowing sequence was pathologic and immunoglobulins were markedly reduced. A bone marro...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(96)05326-9
更新日期：1997-04-15 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:Neuromyelitis optica (NMO) is a severe autoimmune inflammatory disorder of the central nervous system. NMO and its abortive forms are referred to as NMO spectrum disorders (NMOSD). NMOSD are mostly associated with antibodies to aquaporin-4 (AQP4-IgG). However, recent studies have demonstrated antibodies to m...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.08.3254
更新日期：2017-10-15 00:00:00
abstract：BACKGROUND:The Oxfordshire Community Stroke Project (OCSP) classification is a simple tool to categorize clinical stroke syndromes. We compared the outcomes of stroke patients after intravenous thrombolysis stratified by the baseline National Institutes of Health Stroke Scale (NIHSS) score or by the OCSP classification...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.10.003
更新日期：2013-01-15 00:00:00
abstract：OBJECTIVE:This study investigated the utility of sideline concussion tests, including components of the Sports Concussion Assessment Tool, 3rd Edition (SCAT3) and the King-Devick (K-D), a vision-based test of rapid number naming, in an outpatient, multidisciplinary concussion center treating patients with both sports-r...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.06.038
更新日期：2017-08-15 00:00:00
abstract：:Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by segmental vasoconstriction and dilatation of intracranial arteries, usually revealed by headaches, which spontaneously resolve in few weeks. We report a patient with RCVS, revealed by thunderclap headaches, involving both internal and external ca...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.09.033
更新日期：2012-02-15 00:00:00
abstract：:The excitotoxin kainic acid (10 nmol/microliter) was used to produce bilateral lesions in the nucleus basalis magnocellularis (NBM) of rats which provides extensive cholinergic innervation to the cerebral cortex. The behavioral effects of physostigmine, THA (9-amino-1,2,3,4-tetrahydroacridine hydrochloride) and NIK-24...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(89)90041-5
更新日期：1989-03-01 00:00:00
abstract：BACKGROUND:The impact of deep brain stimulation (DBS) on cognitive and urinary disorders, falls, and eventually hospitalizations and mortality in Parkinson's disease (PD) is still debated. OBJECTIVE:We compared the rates of dementia, mild cognitive impairment (MCI), urinary incontinence, nocturia, falls, hospitalizati...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,多中心研究
doi：10.1016/j.jns.2019.07.029
更新日期：2019-10-15 00:00:00
abstract：:Advanced MRI studies demonstrated several diffuse non-lesional features in multiple sclerosis, including changes detectable in gray matter areas. Standard T2 weighted MRI scans of deep gray matter structures, including the thalamus, caudate, putamen, dentate nuclei often demonstrate hypointensity. T2 hypointensity has...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2008.12.013
更新日期：2009-07-15 00:00:00
abstract：:This study investigated the relation between severity of auditory comprehension impairment in aphasia and the functional integrity of the posterior superior temporal region as evaluated by middle- and long-latency auditory evoked potentials and dipole source analysis. AEPs were studied in 20 stroke patients and in age...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(93)90064-6
更新日期：1993-03-01 00:00:00
abstract：OBJECTIVE:Sweating on the palms of the hands and soles of the feet, so-called emotional sweating, is considered to be mediated by the limbic system, including the amygdala and anterior cingulate cortex. To reveal involvement of the limbic system in emotional sweating, we evaluated emotional sweating on the palms in pat...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.04.007
更新日期：2011-07-15 00:00:00
abstract：:We investigated the localization and extent of beta-amyloid precursor protein (APP) immunoreactivity as a sensitive marker for impairment of fast axonal transport in the spinal cords of patients with HTLV-I-associated myelopathy (HAM)/tropical spastic paraparesis (TSP). The results from this study show that APP, used ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(00)00324-5
更新日期：2000-06-15 00:00:00
abstract：BACKGROUND:Intracranial hemorrhage (ICH) is the most common neurologic complication of reversible cerebral vasoconstriction syndrome (RCVS). In this study, we compared hemorrhagic and non-hemorrhagic RCVS with an aim to evaluate the risk factors and short-term clinical outcomes of hemorrhagic lesions. METHODS:We used ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2021.117312
更新日期：2021-01-07 00:00:00
