Abstract:
:Six patients with amyotrophic lateral sclerosis were given from 800 to 4000 micrograms of thyrotropin-releasing hormone (TRH) intrathecally for a period of 2-6 months. The progressive course of this disease, manifested by increasing atrophy, paralysis and disability score, was not altered. This supports the hypothesis that the decrease in TRH content in the anterior horn region is secondary to the cellular destruction. TRH appears to play no significant role in the pathogenesis of amyotrophic lateral sclerosis.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Stober T,Schimrigk K,Dietzsch S,Thielen Tdoi
10.1007/BF00314033subject
Has Abstractpub_date
1985-01-01 00:00:00pages
13-4issue
1eissn
0340-5354issn
1432-1459journal_volume
232pub_type
杂志文章abstract::In idiopathic rapid eye movement sleep behaviour disorder (RBD), an association with visuoperceptive disorders has been described. However, such an association has not been clearly established in RBD secondary to Parkinson's disease (PD). We compared visuoperceptive function in four groups of non-demented patients (pa...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-5328-7
更新日期:2010-03-01 00:00:00
abstract::We present a clinical, neuro-radiological and genetic study on a family with members suffering from an autosomal dominantly inherited syndrome characterised by epilepsy, cerebral calcifications and cysts, bone abnormalities; progressive neuro-cognitive deterioration and paranasal sinusitis. This syndrome shares severa...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7393-9
更新日期:2014-10-01 00:00:00
abstract::The etiology of spontaneous cervical artery dissection (sCAD) is unknown. An underlying connective tissue disorder has been suggested. As a collagen disease is conceivable several genes encoding fibrillar collagens have been condsidered as candidate genes for sCAD. We analysed the COL3A1 gene in patients with spontane...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0745-x
更新日期:2002-07-01 00:00:00
abstract:OBJECTIVE:Five different calliper methods for assessing the degree of carotid artery stenosis and visual estimation ("eyeballing") of postmortem carotid arteriograms were compared with the planimetric gold standard of the area reduction at the site of the stenosis. METHODS:During autopsy 53 carotid specimens were remo...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0703-5
更新日期:2005-05-01 00:00:00
abstract::Adolescent-onset spastic ataxia is a proposed novel phenotype in compound heterozygous carriers of an intronic mutation (c.1909 + 22G > A) in the POLR3A gene. Here, we present ten new cases of POLR3A-related spastic ataxia and discuss the genetic, clinical and imaging findings. Patients belonged to six pedigrees with ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09574-9
更新日期:2020-02-01 00:00:00
abstract:OBJECTIVE:To determine the response to treatment and the long-term outcome of patients with the antisynthetase syndrome associated with anti-Jo-1-antibodies. PATIENTS AND METHODS:A total of 12 patients with histologically proven myositis and anti-Jo-1-autoantibodies were evaluated over a mean follow-up period of 66.4 ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0449-5
更新日期:2004-07-01 00:00:00
abstract::Neuroinflammation (microglial activation) and subclinical nigrostriatal dysfunction have been reported in subjects at risk of Parkinsonism. Eight non-manifesting carriers (NMCs) of LRRK2 G2019S mutation had 11C-PK11195 and 18F-DOPA PET to assess microglial activation and striatal dopamine system integrity, respectivel...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-09830-3
更新日期:2020-08-01 00:00:00
abstract::A myasthenia gravis (MG) patient who seems to have recovered can later have recurrence of myasthenic signs. Clearly clinical remission does not always correspond to the normalization of all the factors involved in the pathogenesis of the disease. In ten patients who had apparently recovered from MG, electromyographic ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313771
更新日期:1982-01-01 00:00:00
abstract::Progressive multifocal leukoencephalopathy (PML) is a subacute demyelinating disease of the brain caused by the JC virus that occurs mainly in immunocompromised patients. The prognosis is very poor. As the lesion looks like non- specific leukoencephalopathy, making a diagnosis at the early stage is very difficult. We ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7500-y
更新日期:2014-12-01 00:00:00
abstract:BACKGROUND AND OBJECTIVE:The number of neurological second opinions (SO) and tertiary referrals (TR) is increasing. The main purpose of this study was to assess whether a day-care admission made a meaningful contribution to standard neurological outpatient care, for a wide range of second opinions and tertiary referral...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0019-3
更新日期:2008-11-01 00:00:00
abstract:OBJECTIVE:To explore the value of olfactory identification deficits as a predictor of cerebral β-amyloid status and other markers of brain health in cognitively normal adults aged ~ 70 years. METHODS:Cross-sectional observational cohort study. 389 largely healthy and cognitively normal older adults were recruited from...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10004-4
更新日期:2020-11-01 00:00:00
abstract::We evaluated the health economic burden of patients with Gilles de la Tourette's syndrome (GTS) in Germany over a 3-month observation period. Direct and indirect costs were evaluated in 200 outpatients with GTS (mean age 35 +/- 11.5 years) in Germany. Patients were recruited from three outpatient departments that spec...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-010-5458-y
更新日期:2010-07-01 00:00:00
abstract::The course of multiple sclerosis (MS) can be monitored by measuring changes in brain volume, but consensus is still lacking on the best strategy to be adopted. We compared the reproducibility and sensitivity of volume measurements from different brain portions for detecting changes on magnetic resonance imaging (MRI) ...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s004150070054
更新日期:2000-12-01 00:00:00
abstract::Acute mountain sickness (AMS) can occur during climbs to high altitudes and may seriously disturb the behavioral and intellectual capacities of susceptible subjects. During a Himalayan expedition 32 mountaineers were examined with electroencephalography (EEG) and transcranial doppler sonography (TCD) to assess relativ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0376-8
更新日期:2007-03-01 00:00:00
abstract::This review summarizes the most relevant articles from the field of neuro-ophthalmology published in the Journal of Neurology from January 2012 to July 2013. With the advent of video-oculography, several articles describe new applications for eye movement recordings as a diagnostic tool for a wide range of disorders. ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-013-7105-x
更新日期:2014-07-01 00:00:00
abstract::Acid maltase deficiency is described in non-identical adult twins. The onset of the disease can be traced into late infancy; the clinical picture is one of severe muscular dystrophy; respiratory insuficiency was the cause of death in one case. The autopsy showed the central nervous system, heart and liver to be spared...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313272
更新日期:1976-08-06 00:00:00
abstract::Thirty-seven Japanese autopsy cases with diffuse Lewy body disease (DLBD) were reviewed from a clinicopathological viewpoint. Based on the neuropathological finding of whether or not many concomitant senile plaques (SPs) and/or neurofibrillary tangles (NFTs) are present. DLBD is divided into two forms: a common form a...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/BF00314594
更新日期:1990-06-01 00:00:00
abstract::We investigated the visual event-related potentials (ERPs) in two subtypes of multisystem atrophy (MSA) in 15 MSA-C patients, 12 MSA-P patients, and 21 normal control (NC) subjects. We used a visual oddball task to elicit ERPs. No significant changes were seen in N1 or N2 latency, in either MSA-C or MSA-P, compared wi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0764-7
更新日期:2002-08-01 00:00:00
abstract:BACKGROUND:D-dimer levels are used in several clinical settings, such as in predicting venous thrombosis, cardioembolic stroke and cancer status. In the present study, we investigated the associations between plasma D-dimer levels at admission, clinical characteristics and mortality at discharge in cryptogenic stroke p...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-018-8742-x
更新日期:2018-03-01 00:00:00
abstract::Peduncular hallucinosis (PH) is a syndrome of hallucinations and brainstem symptoms which has only been described in single case reports. A detailed analysis of five patients was undertaken to investigate the clincial characteristics, hallucinations and behavioural abnormalities of PH in greater detail. Frequent clini...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-0060-0254-4
更新日期:2006-12-01 00:00:00
abstract::Additional autoimmune diseases in people with multiple sclerosis (MS) and their relatives have been studied many times. Studies have employed different designs, and yielded conflicting results. We performed a systematic review, and calculated overall risk of additional autoimmune diseases in people with MS and their f...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00415-012-6790-1
更新日期:2013-05-01 00:00:00
abstract::Absence of AMP-deaminase was demonstrated by histochemical and biochemical methods in a muscle biopsy of a 25-year-old woman with facial and limb girdle myopathy. Venous ammonia failed to rise after ischaemic exercise. This patient further contributes to the variety of clinical pictures associated with AMP-deaminase d...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313744
更新日期:1981-01-01 00:00:00
abstract::We report the effects of intravenous gamma-globulin (IVGG) in severe Guillain-Barré syndrome (GBS) in a small open study. Seven patients were given IVGG (0.4 g/kg/day) for 5 consecutive days. At the start of treatment all had progressing limb weakness and none could stand unaided. Within 24 h all but one patient start...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00838447
更新日期:1993-01-01 00:00:00
abstract::We recently reported findings that loss of cortical acetylcholinesterase (AChE) activity is greater in parkinsonian dementia than in Alzheimer's disease (AD). In this study we determined cognitive correlates of in vivo cortical AChE activity in patients with parkinsonian dementia (PDem, n = 11), Parkinson's disease wi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0971-0
更新日期:2006-02-01 00:00:00
abstract::The phase III, multicenter, randomized, placebo-controlled PreCISe trial assessed glatiramer acetate (GA) effects in patients with clinically isolated syndromes (CIS) suggestive of multiple sclerosis (MS). To assess the neuroprotective effect of GA in a subset of patients in the PreCISe trial, we used proton magnetic ...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1007/s00415-013-6903-5
更新日期:2013-07-01 00:00:00
abstract::It is now 10 years since the first report of mutations in the presenilin genes that were deterministic for familial autosomal dominant Alzheimer's disease. The most common of these mutations occurs in the presenilin-1 gene (PSEN1) located on chromosome 14. In the ensuing decade, more than 100 PSEN1 mutations have been...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-005-0019-5
更新日期:2006-02-01 00:00:00
abstract::A consecutive series of 327 patients (188 males, 139 females; mean age 68.4, SEM 1.33) were hospitalized within 12 h of the onset of their first-ever hemispheric infarct. Three groups of patients were identified: diabetics (n = 70), non-diabetic hyperglycaemics (n = 93) and normoglycaemics (n = 164). Case-fatality rat...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00812155
更新日期:1992-08-01 00:00:00
abstract::Four cases of mild botulinus type B intoxication are reported with the clinical neurophysiological studies. Electromyographically tetanic stimulation of the N. ulnaris initially led to tetanic potentiation of the muscle action potential. After an interval without any symptoms tetanic reduction became apparent between ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00312957
更新日期:1978-02-14 00:00:00
abstract::We should like to emphasize the following points: 1. Apathy is defined here as a quantified and observable behavioral syndrome consisting in a quantitative reduction of voluntary (or goal-directed) behaviors; 2. Therefore, apathy occurs when the systems that generate and control voluntary actions are altered; 3. These...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-006-7012-5
更新日期:2006-12-01 00:00:00
abstract::Functional changes in the organisation of neuronal circuitries are generally used to explain parkinsonian motor symptoms and levodopa-induced dyskinesias. Based on information from histology and neurophysiological microrecordings of specific basal ganglia nuclei, the most widely accepted scheme is based on a central l...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00007765
更新日期:2000-04-01 00:00:00