Abstract：
:Ten patients with myotonic dystrophy (MyD) and excessive daytime sleepiness (EDS) were studied. Daytime sleepiness was assessed by means of a subjective alertness rating scale, multiple sleep latency tests and auditory event-related potentials. In addition, the diurnal pattern of daytime sleepiness and ultradian rhythm characteristics of nocturnal sleep were assessed. The multiple sleep latency tests and the P300 component of the auditory event-related potentials gave evidence of EDS, whereas the subjective alertness ratings did not. However, the alertness ratings showed a significantly reduced circadian periodicity as compared with controls. Consistent with this, no time-of-day effect was observed for the multiple sleep latency test outcomes. The ultradian rhythm characteristics of nocturnal sleep indicated a prolonged mean cycle duration and decreased stability of NREM/REM cycle. Moreover, the temporal structure of REM sleep showed a pattern similar to that of subjects who develop free-running rhythms when living in temporal isolation. These findings suggest that EDS in MyD reflects a complex and wide-spread malfunction of the circadian and ultradian timing system.
journal_name
J Neurol Scijournal_title
Journal of the neurological sciencesauthors
van Hilten JJ,Kerkhof GA,van Dijk JG,Dunnewold R,Wintzen ARdoi
10.1016/0022-510x(93)90051-ysubject
Has Abstractpub_date
1993-01-01 00:00:00pages
68-75issue
1eissn
0022-510Xissn
1878-5883pii
0022-510X(93)90051-Yjournal_volume
114pub_type
杂志文章abstract：:This is the first report of a patient presenting with a combination of Wallenberg and posterior spinal artery syndromes. The patient developed right hemiplegia and sensory disturbances on the right side of the face and over the whole body. MRI showed infarcts of the cerebellum, medulla oblongata, and upper cervical co...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(00)00318-x
更新日期：2000-06-15 00:00:00
abstract：:On September 28, 1994, the investigators of the Asymptomatic Carotid Atherosclerosis Study (ACAS) reported the interim results of a randomized controlled clinical trial of carotid endarterectomy in patients who have asymptomatic carotid stenosis of greater than 60% reduction in diameter. In addition to aspirin and agg...
journal_title：Journal of the neurological sciences
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：10.1016/0022-510x(95)00010-y
更新日期：1995-03-01 00:00:00
abstract：:Antiserum to the myelin lipid galactocerebroside (GalC) causes rapidly progressive focal demyelination when injected into guinea pig optic nerves. The capacity of anti-GalC to induce central nervous system demyelination is complement-dependent, and demyelinating activity is present in the immunoglobulin fraction of an...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(84)90177-1
更新日期：1984-06-01 00:00:00
abstract：BACKGROUND:Endocrine dysfunction is known to occur in various infectious diseases of the brain. The neuroendocrine dysfunction is not well studied in patients of Tuberculous meningitis (TBM). In this study, we aimed at knowing pattern of endocrine dysfunction in newly diagnosed patients of tuberculous meningitis, struc...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.06.015
更新日期：2017-08-15 00:00:00
abstract：:Parkinson's disease is the most common movement disorder in the broad spectrum of neurodegenerative diseases, associated frequently with gradual decline of the higher mental faculties. From the morphological point of view it is characterized by the degeneration of a substantial number of dopaminergic neurons of the su...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2006.05.006
更新日期：2006-10-25 00:00:00
abstract：:Based on the assumption that brain ischemia and hypoxia are central causes of brain damage, the maintenance of an adequate tissue oxygenation is a primary objective in the field of neurocritical care. Thus, monitoring brain tissue oxymetry, allowing the possibility to discriminate between normal and critically impaire...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2007.04.026
更新日期：2007-10-15 00:00:00
abstract：:HS1-associated protein X-1 (HAX-1) and presenilin-associated rhomboid-like protein (PALR) were reported to play an important role in the activation of HtrA2/Omi, which is also designated PARK13, in the mitochondria. To elucidate the role of HAX-1 and PARL in patients with Parkinson's disease (PD) and dementia with Lew...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2020.116928
更新日期：2020-08-15 00:00:00
abstract：BACKGROUND:Contactin-associated protein 2 (Caspr2) antibody is a neuronal surface antibody (NSAb) capable of causing disorders involving central and peripheral nervous systems (PNS). Thymoma can be found in patients with Caspr2 antibodies and is most frequently associated with PNS symptoms. Myasthenia gravis can be fou...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.03.043
更新日期：2014-06-15 00:00:00
abstract：:Myositis was proven histopathologically in 4 patients (age range 36-66 years) who suffered from early or late stages of Borrelia burgdorferi infection. Muscle weakness was present in 3 patients, 1 complaining of additional myalgias. One man came to medical attention because of skin discoloration and swelling of one le...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(89)90089-0
更新日期：1989-06-01 00:00:00
abstract：:Cortical excitability to magnetic stimulation was investigated interictally in 10 patients with migraine with aura, 10 with migraine without aura and in 10 healthy volunteers. Thresholds, latencies and amplitudes of the magnetic-evoked potentials (MEPs) were measured from threshold to 100% stimulus intensity in 10% st...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(96)00044-5
更新日期：1996-07-01 00:00:00
abstract：:The study is designed to assess the oxidative stress intensity in erythrocytes obtained from patients in different clinical phenotypes of neuroinflammation, defined as clinically isolated syndrome (CIS) and relapsing-remitting multiple sclerosis (RRMS). Advanced oxidation protein products (AOPP), malondialdehyde (MDA)...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.11.006
更新日期：2014-02-15 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: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：BACKGROUND:The treatment of hypertension is an essential component of stroke prevention; however, the clinical management of patients with cerebrovascular disease is complicated by orthostatic hypotension (OH). The primary objectives were to: determine the prevalence of OH in a stroke outpatient clinic; describe catego...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2011.10.031
更新日期：2012-03-15 00:00:00
abstract：:A Finnish family is described with 9 cases of presenile dementia in 3 generations. The mean age at onset was 52 years (range 46--62 years). Progressive dementia, upper motor neuron signs, muscular rigidity, and twitching, irregular tremors were consistent features in the 6 clinically investigated patients and were ass...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(79)90171-0
更新日期：1979-08-01 00:00:00
abstract：:Cases of adult-onset idiopathic motor neuron disease (MND) identified from January 1970 through December 1986 were studied in a defined area of California. The patients were followed prospectively throughout the illness in 99% of cases. Among 708 cases aged 25-74 years at onset, the most common type (86%) was typical,...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(93)90245-t
更新日期：1993-08-01 00:00:00
abstract：BACKGROUND AND OBJECTIVE:Multiple sclerosis (MS) has been consistently associated with the HLA-DR2 haplotype and particularly with the HLA-DRB1*15 allele. Epistatic interactions between both parental alleles in the DRB1 loci have been shown to modify the MS susceptibility risk. This study investigated the frequencies o...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2010.07.026
更新日期：2010-11-15 00:00:00
abstract：:Even though the mesocortical dopamine system is known to play an important role in affect control and reward related behaviour, only little is known about the impact of Parkinson's disease on emotional communication. The ability to perceive and express emotions via speech plays an essential role in every day social li...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章,评审
doi：10.1016/j.jns.2009.08.038
更新日期：2010-02-15 00:00:00
abstract：:Somatosensory evoked potentials (SEPs) following median nerve stimulation were abnormal in 7 patients with sensory impairment due to vitamin B12 deficiency. Extensor plantar reflexes indicated a central sensory pathway lesion in 4 cases and absent tendon jerks suggested peripheral neuropathy in 4, but median nerve SEP...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(87)90006-2
更新日期：1987-12-01 00:00:00
abstract：:Glucose-6-phosphate dehydrogenase (G6PDH), 6-phosphogluconate dehydrogenase (6PGDH) and glyceraldehyde-3-phosphate dehydrogenase (GAPDH) activities were assayed in superficial pectoral muscles of hereditary dystrophic chickens, 1 week, 2 weeks, 4 weeks and 4 months after hatching. In control chickens, activities of G6...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/0022-510x(85)90049-8
更新日期：1985-04-01 00:00:00
abstract：OBJECTIVES:There is insufficient data on the efficacy of intravenous immunoglobulins (IVIg) as maintenance treatment in myasthenia gravis (MG). We therefore examined response to maintenance IVIg therapy in a cohort of MG patients. METHODS:We reviewed all MG patient files treated with IVIg in our neuro-immunology clini...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2013.10.043
更新日期：2014-03-15 00:00:00
abstract：OBJECTIVE:To investigate whether there is a difference in the neuro-autonomic and hemodynamic parameters in patients with postural tachycardia syndrome (POTS) according to onset time of orthostatic tachycardia during the Head-up tilt (HUT) test. METHODS:We retrospectively reviewed neuro-autonomic and hemodynamic data ...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2018.04.033
更新日期：2018-07-15 00:00:00
abstract：:Adult onset cerebral X-ALD (AOCALD) is a rare disease, but should be considered an important differential diagnosis in adults presenting with leukencephalopathy. We here report the case of a 40-year-old man with a history of progressive cognitive decline who presented with a first-time seizure. Initial workup included...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2009.06.012
更新日期：2009-10-15 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：:Bilateral striatal necrosis (BSN) has many causes and is characterized by unique clinical and neuroradiological features. Herein, we report a clinical and genetic analysis of three BSN cases from two independent Japanese families harboring a mitochondrial DNA (mtDNA) 14459G>A mutation located in a coding region of the...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2017.05.015
更新日期：2017-07-15 00:00:00
abstract：OBJECTIVE:To describe the clinical characteristics, delay to diagnosis and post-intervention outcomes of patients with low-flow spinal arteriovenous fistulas (SAVFs). METHODS:In this retrospective observational study, we reviewed all patients with low-flow SAVFs angiographically diagnosed at our institution between 20...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2020.116863
更新日期：2020-06-15 00:00:00
abstract：:Intravascular large B-cell lymphoma (IVLBCL) is a rare lymphoproliferative disorder characterized by massive intravascular growth of lymphoma cells with a predilection for the central nervous system (CNS). Diagnosis is generally delayed by variable clinical presentation and nonspecific laboratory findings. Brain biops...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2012.10.012
更新日期：2013-02-15 00:00:00
abstract：:Traumatic brain injury (TBI) is a considerable cause of mild cognitive impairment and dementia. Intranasal administration of nerve growth factor (NGF) has previously been found to improve cognitive function after TBI, but the mechanism remains unclear. This study aimed to investigate the effects of intranasal NGF on t...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/j.jns.2014.06.037
更新日期：2014-10-15 00:00:00
abstract：:To compare muscle fiber loss in young and old mdx mice, we have blocked regeneration in one leg with a high dose (18 Gy) of X-rays administered at two ages; 16 days, just prior to the onset of the myopathy, and 15 weeks, when the myopathy is considered to be quiescent. Mice were examined 4 days after irradiation to lo...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(99)00061-1
更新日期：1999-04-01 00:00:00
abstract：:Systemic administration of opiates or direct injection of opioid peptides into the periaqueductal gray (PAG) produces a profound antinociception which is thought to be associated with inhibition of neuronal activity in the PAG. This inhibitory effect has been postulated to result from opiate inhibition of GABAergic ne...
journal_title：Journal of the neurological sciences
pub_type： 杂志文章
doi：10.1016/s0022-510x(98)00327-x
更新日期：1999-03-01 00:00:00
