Abstract：
BACKGROUND:Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized by the involvement of cerebral small arteries. Although cerebral large artery disease has been reported in CADASIL patients, the prevalence and location of relevant cerebral arterial disease have not been elucidated. In this study, we aim to characterize infarctions associated with cerebral large artery disease in CADASIL patients. METHODS:We retrospectively reviewed 49 consecutive symptomatic patients with genetically confirmed CADASIL, who visited the Asan Medical Center between December 2002 and December 2013. Infarctions located within the territory of a relevant, large cerebral artery were identified with the use of magnetic resonance imaging and magnetic resonance angiography. Patients with or without territorial patterns associated with large artery disease were compared. RESULTS:Out of a total of 49 patients, 23 patients had cerebral infarction. Among these, seven had infarction associated with cerebral large artery disease. The corresponding vascular lesions were located in the intracranial arteries in all seven patients. There were no differences between patients with or without territorial infarction in terms of vascular risk factors, microbleeds, white matter changes, or mutations involving cysteine. A literature review illustrates that symptomatic intracranial diseases are present in CADASIL patients at least in East Asia. CONCLUSION:Infarction in association with intracranial arterial disease may be a manifestation of CADASIL. Further studies are needed to elucidate the pathologic characteristics and to see whether this occurs exclusively in East Asia.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
Kang HG,Kim JSdoi
10.1016/j.jns.2015.11.029subject
Has Abstractpub_date
2015-12-15 00:00:00pages
347-50issue
1-2eissn
0022-510Xissn
1878-5883pii
S0022-510X(15)30039-3journal_volume
359pub_type
杂志文章abstract：PURPOSE:Increased concentrations of the nervous-system-specific proteins neuron-specific enolase (NSE) and S-100 protein (S-100) have been measured with lesions in the CNS. Elevated levels of serum NSE (s-NSE) have been found in status epilepticus, but also after single epileptic seizures. Because larger studies addres...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(00)00478-0
更新日期：2001-01-15 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：:Protection of guinea pigs from experimental allergic encephalomyelitis (EAE) was attempted using bacterial lipopolysaccharide (LPS) from 4 sources. The ability of these LPS to induce DNA synthesis in guinea pig lymph node (LN) cells in vitro was also investigated. It was found that there existed a good correlation bet...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(79)90174-6
更新日期：1979-08-01 00:00:00
abstract：:The purpose of this paper is to systematically review the literature on the use of near-infrared spectroscopy (NIRS) for assessing spasticity. MEDLINE, CINAHL, and EMBASE were searched for human and/or animal studies written in the English language published until November 2018. that used NIRS to examine the hemodynam...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2020.116729
更新日期：2020-05-15 00:00:00
abstract：:Sympathetic skin response (SSR) is a simple, reproducible test of function of a polysynaptic reflex having diverse afferents, a common efferent pathway through the spinal cord, pre and post-ganglionic sympathetic fibers and with sweat glands as effectors. The reflex is co-ordinated in the posterior hypothalamus or upp...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/0022-510x(94)00265-p
更新日期：1995-04-01 00:00:00
abstract：:The in vivo diagnosis of Alzheimer's disease (AD) may be facilitated by cerebro-spinal fluid (CSF) biomarkers in combination with imaging and clinical assessments. By determining the concentration of beta amyloid fragments, total tau (t-tau) and phospho-tau (p-tau), it is possible to detect the conversion of mild cogn...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.02.343
更新日期：2009-08-15 00:00:00
abstract：INTRODUCTION:Functional movement disorders (FMD) are not associated with the kind of structural or biochemical alterations seen in other movement disorders and therefore would be unlikely to be associated with lower urinary tract (LUT) dysfunction. However, LUT symptoms have been observed in patients with FMD. OBJECTI...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.12.048
更新日期：2016-02-15 00:00:00
abstract：:Epidemiological and clinical studies have shown that environmental factors such as infections, smoking and vitamin D are associated with the risk of developing multiple sclerosis (MS). Some of these factors also play a role in the MS disease course. We are currently beginning to understand how environmental factors ma...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2012.10.021
更新日期：2013-01-15 00:00:00
abstract：OBJECTIVES:To examine whether quantitative analysis of the brainstem areas on magnetic resonance imaging (MRI) scans is useful for diagnosis as well as evaluation of disease activity in chronic progressive neuro-Behçet's disease (CPNB). METHODS:MRI scans in patients with acute neuro-Behçet's disease (ANB) (n = 10), CP...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.11.020
更新日期：2014-02-15 00:00:00
abstract：BACKGROUND:Current American Stroke Association guidelines recommend initiating intravenous thrombolysis (IVT) for acute ischemic stroke (AIS) within 60min of patient arrival, given the benefits of IVT for AIS are time dependent. This study aimed to identify the delaying factors in door-to-needle time (DTN) in the emerg...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.03.003
更新日期：2017-05-15 00:00:00
abstract：BACKGROUND:Parkinson's disease (PD) and multiple system atrophy (MSA) are neurodegenerative diseases that can be difficult to diagnose and distinguish from each other. STUDY AIMS AND METHODS: Patients with PD and MSA and controls were studied with magnetic resonance imaging (MRI) using tissue segmentation and outlining...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2005.07.013
更新日期：2005-12-15 00:00:00
abstract：BACKGROUND:Although adoption of new clinical criteria for dementia with Lewy bodies (DLB) leads to an increase in the proportion of patients diagnosed with probable DLB, the sensitivity of clinical diagnostic criteria of DLB is low, and there are no generally accepted clinical or imaging biomarkers to distinguish DLB f...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2015.07.025
更新日期：2015-10-15 00:00:00
abstract：:We studied the production within the CNS of anti-HIV antibodies, of non-specific IgG, and the presence of HIV antigens in the serum and CSF of 28 HIV infected patients belonging to group IV in the Center for Disease Control classification. CSF and serum were diluted under optimal conditions to equalize their IgG conte...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(90)90162-g
更新日期：1990-11-01 00:00:00
abstract：:The author describe 2 personal observations of peripheral neuropathy with cryoglobulinemia and the 28 cases previously recorded are reviewed. The characteristics of the usually sensorimotor neuropathy are not specific. Nevertheless, the association with purpura, Raynaud's syndrome and leg ulcers and the inconstant agg...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(78)90005-9
更新日期：1978-02-01 00:00:00
abstract：:We evaluated 14 previously healthy dogs with acute progressive flaccid quadriparesis and hyporeflexia. Electrophysiological and pathological features indicated varying degrees of motor-sensory polyneuropathy with some demyelination. Perivenular cellular infiltrations were present in roots of one autopsied case. Recove...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(82)90147-2
更新日期：1982-11-01 00:00:00
abstract：:Eclampsia accounts for a third of maternal mortality in developing countries. The neurological manifestations of eclampsia consist of seizures and alteration of sensorium or coma on a background of pre-eclampsia. Occasionally there can be focal neurological deficits too. Recent studies with CT scan and MRI have demons...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/s0022-510x(97)00274-8
更新日期：1998-02-18 00:00:00
abstract：:Demonstration of the direct involvement of cranial blood vessels by varicella zoster virus (VZV) is facilitated by immunohistochemistry (IHC), in situ hybridization (ISH) and polymerase chain reaction (PCR) techniques. The extent to which an inflammatory vasculitis serves as the pathogenic mechanism for VZV encephalom...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00171-3
更新日期：1998-08-14 00:00:00
abstract：:Inherited muscular dystrophy of the chicken is an abnormality affecting the normal development and function of fast-twitch skeletal muscles. Several different strains of dystrophic chickens have been developed by selection for high lipid content in the pectoralis muscle and early onset of the disorder or by outcrossin...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(80)90073-8
更新日期：1980-05-01 00:00:00
abstract：:A study was made of Wistar rat soleus muscle following intraperitoneal administration of denervated muscle extract over 1 and 2 days. Light microscopy revealed the appearance on fiber surfaces of basophilic satellite structures whose histochemical behaviour differed from that of the parent fiber. Small fibers showing ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(94)00212-7
更新日期：1995-01-01 00:00:00
abstract：:Identification of the Negri bodies in the brain of an 8-year-old boy who died 8 days after a paralytic illness and 20 days after a dog bite, and who had received 9 injections of Semple's anti-rabies vaccine, provided evidence that he died of acute rabies encephalitis and not of post-vaccinal allergic encephalomyelitis...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(86)90093-6
更新日期：1986-09-01 00:00:00
abstract：:Hereditary spongiform dystrophy in young children is characterised by macrocephaly with spasticity, convulsions and ultimately a decerebrate state and diffuse electroencephalographic changes. Histological examination of the brain remains essential for its diagnosis. A review of the ultrastructural studies reported by ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(76)90101-5
更新日期：1976-06-01 00:00:00
abstract：:McLeod syndrome is a rare X-linked disorder involving neurological defects and acanthocytosis. We examined the XK gene in three patients with neuroacanthocytosis, one of whom had cardiomyopathy, and his symptoms were very similar to those of McLeod syndrome. We found two new transversions (C to G at codon 204 and G to...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(97)00067-1
更新日期：1997-09-10 00:00:00
abstract：OBJECTIVE:Treatment of restless legs syndrome (RLS) is primarily based on drugs. Since many patients report improvement of symptoms due to cooling their legs, we examined the efficacy of cryotherapy in RLS. PATIENTS AND METHODS:35 patients (28 women, 60.9±12.5years) with idiopathic RLS and symptoms starting not later ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,随机对照试验
doi：10.1016/j.jns.2016.09.006
更新日期：2016-11-15 00:00:00
abstract：OBJECTIVE:The Circle of Willis (CoW) is often underdeveloped or incomplete, leading to suboptimal blood supply to the brain. As hypoperfusion is thought to play a role in the aetiology of white matter hyperintensities (WMH), the objective of this study was to assess whether incomplete CoW variants were associated with ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2020.117268
更新日期：2021-01-15 00:00:00
abstract：:The Hyper-IgE syndrome (HIES) is a rare primary immunodeficiency which recently has been associated with heterozygous dominant-negative mutations in the signal transducer and activator of transcription 3 (STAT3). Although HIES is characterized by recurrent staphylococcal infections, the microbial invasion of the centr...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.07.045
更新日期：2011-10-15 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：: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：:Preclinical evidence strongly indicate that adenosine A(2A) receptor antagonists represent a promising class of drugs for the treatment of motor deficits associated to Parkinson's disease. The effects of adenosine A(2A) receptor antagonists were here assessed in a rat model of parkinsonian tremor induced by cholinomim...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.05.038
更新日期：2006-10-25 00:00:00
abstract：:An X-linked myopathy was recently associated with mutations in the four-and-a-half-LIM domains 1 (FHL1) gene. We identified a family with late onset, slowly progressive weakness of scapuloperoneal muscles in three brothers and their mother. A novel missense mutation in the LIM2 domain of FHL1 (W122C) co-segregated wit...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.06.017
更新日期：2010-09-15 00:00:00
abstract：BACKGROUND:The timed-up-and-go test (TUG) is a popular test for mobility, but it only measures the total time to complete tasks. Recent advances in smartphone technology enables measuring respective times of 6 components in the test (Stand, Go, Turn1, Come, Turns and Sit). We examined availability of these data for ass...
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
