Abstract:
:In a 5-year follow-up of a case of adult metachromatic leukodystrophy, already diagnosed in the preclinical stage, the development of the symptoms of this disease could be studied in detail: initially, lack of drive, emotional lability and depressive mood. At the same time, pain in the arms and beginning gait disturbance. Later, impairment of memory and concentration, disorientation, inadequate behavior and progression of gait disturbance. Finally spastic atactic gait with small steps and dyspractic components, coordination disturbances with writing dysfunction, fast dysarthric speech, hyperkinetic activity, compulsory emotional outbursts and progressive dementia. Only minor neurological signs such as reflex abnormalities. In the EEG, slight slowing of frequencies compared to earlier tracings. Increasing diminution of nerve conduction velocity in the lower limbs. Only minor increase of CSF protein (51 mg%). In spite of normal vision, evoked visual potentials abnormal, response of optical and electrical blink reflexes delayed. Imperfect filling of gallbladder. No significant quantitative changes of the biochemical parameters compared with the findings made 5 years earlier (excretion of urinary sulfatides, diminished activity of arylfulfatase A in urine and leukocytes).
journal_name
Eur Neuroljournal_title
European neurologyauthors
Pilz H,Duensing I,Heipertz R,Seidel D,Lowitzsch K,Hopf HC,Goebel HHdoi
10.1159/000114818subject
Has Abstractpub_date
1977-01-01 00:00:00pages
301-7issue
6eissn
0014-3022issn
1421-9913journal_volume
15pub_type
杂志文章abstract::The association of anaemia and gastro-intestinal abnormalities with disorders of the brain, spinal cord, and peripheral nerves has been recognized since the mid 19th century. In early reports interpretable as subacute combined degeneration, anaemia was overlooked, and conversely in Addison's and other early reports of...
journal_title:European neurology
pub_type: 历史文章,杂志文章
doi:10.1159/000131715
更新日期:2008-01-01 00:00:00
abstract::An HIV-positive patient presented the classical syndrome of pure alexia. Neuroradiologic investigation by computed tomography showed a ring-like lesion in the left posterior white matter. The clinical manifestations as well as the radiologic findings resolved after antiprotozoal treatment. ...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116783
更新日期:1992-01-01 00:00:00
abstract::Nerve conduction of single impulses and the ability to transmit frequent trains of stimuli were studied in sensory median and in sural nerves of controls and patients with diabetes mellitus. Decrease of relative amplitudes and increase of relative latencies were more marked in the diabetics as compared with normal con...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115123
更新日期:1980-01-01 00:00:00
abstract::MPTP, 1-methyl-4-phenyl-pyridinium ion (MPP+) and the structurally related compounds including N-methyltetrahydroisoquinilinium ion inhibited the tyrosine hydroxylase (TH) system in tissue slices of rat striatum at 10(-5) M. Mouse striatum is more susceptible to MPTP than rat striatum. A similar degree of inhibition b...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116350
更新日期:1987-01-01 00:00:00
abstract::The gradual intraneuronal accumulation of an insoluble fibrous material which partly consists of abnormally phosphorylated tau protein (neurofibrillary change) represents an important neuropathological hallmark of Alzheimer's disease. Neurofibrillary tangles and neuropil threads formed from this material develop in on...
journal_title:European neurology
pub_type: 杂志文章,评审
doi:10.1159/000116984
更新日期:1993-01-01 00:00:00
abstract:BACKGROUND:The purpose of this study was to observe the incidence of falls in Parkinson's disease (PD) patients with different cognitive levels and to investigate the effect of the cholinesterase inhibitor Rivastigmine on cognitive dysfunction and falling in PD patients. SUBJECTS AND METHODS:Data from 176 PD patients ...
journal_title:European neurology
pub_type: 杂志文章,随机对照试验
doi:10.1159/000438824
更新日期:2015-01-01 00:00:00
abstract::Dementia is one of the most common organic mental syndromes, usually caused by Alzheimer's disease (AD) or vascular dementia (VD) or both. Regarding AD we review the state or the art of the cholinergic approach and discuss some future options regarding preventive and nonsymptomatic strategies. Therapy for VD will cons...
journal_title:European neurology
pub_type: 杂志文章,评审
doi:10.1159/000113372
更新日期:1997-01-01 00:00:00
abstract::13 patients with spinal dural arteriovenous fistulas (DAVF) diagnosed in our Institution have been the object of this study. The necessity to include the disease in the differential diagnosis with other pathologies affecting the lower thoracic cord, particularly in middle-aged and old males, is emphasized. Involvement...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000117015
更新日期:1994-01-01 00:00:00
abstract::This study assessed gender differences in diabetic patients with ischemic stroke with respect to vascular risk factors, clinical features and early outcome. Ischemic stroke was diagnosed in 261 diabetic women and 300 diabetic men of 2,446 ischemic stroke patients included in a prospective stroke registry over 17 years...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000094249
更新日期:2006-01-01 00:00:00
abstract::Machado-Joseph disease (MJD) is a dominantly inherited cerebellar ataxia associated with spasticity, ophthalmoplegia, dystonia and peripheral neuropathy. Presented here are 5 MJD cases. A morphometric analysis of the histopathology of their sural nerves was carried out to know the relationship between axon size and my...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000066169
更新日期:2002-01-01 00:00:00
abstract::The importance of patent foramen ovale (PFO) has long been discussed in the literature in relation to different coexisting medical conditions. Many researchers focused on the occurrence of PFO in ischemic stroke patients, especially in the subgroup of young patients with a recurrent event. Unfortunately, before establ...
journal_title:European neurology
pub_type: 杂志文章,评审
doi:10.1159/000342900
更新日期:2013-01-01 00:00:00
abstract::Our knowledge about pathophysiology of intracerebral hemorrhage (ICH) mainly originates from preclinical models of ICH. In this study, cerebral ultrastructure surrounding hematoma and its correlation with clinical severity were investigated in ICH patients. Thirty patients with basal ganglia hemorrhage and 6 control s...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000434631
更新日期:2015-01-01 00:00:00
abstract:BACKGROUND:One of the hallmarks of the α-synucleinopathies is the degeneration of the autonomic nervous system. SUMMARY:This review discusses the diagnosis and management of cardiovascular autonomic failure within the context of the α-synucleinopathies. In addition, it outlines the utility of various laboratory assess...
journal_title:European neurology
pub_type: 杂志文章,评审
doi:10.1159/000368828
更新日期:2015-01-01 00:00:00
abstract:AIMS:This study was designed to investigate if folate treatment is able to reverse the phenytoin-induced deficiency of salivary immunoglobulin A (IgA). METHODS AND MATERIAL:Twenty-five epileptic patients who had been under phenytoin therapy for at least the last 6 months were randomly selected and subjected to folic a...
journal_title:European neurology
pub_type: 杂志文章,随机对照试验
doi:10.1159/000121419
更新日期:2008-01-01 00:00:00
abstract::Three patients with cerebral tumor involving diencephalic midline structures (2 malignant gliomas, 1 craniopharyngioma) and no metabolic abnormalities developed disorientation in time and place or coma with triphasic waves (TW) seen on electroencephalograms. Serial EEG recordings showed persistence or disappearance of...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116614
更新日期:1990-01-01 00:00:00
abstract::The frontal lobe is the largest lobe of the brain, and it is thus commonly involved in stroke. Moreover, almost one in five strokes is limited to the prerolandic areas. This high frequency of anatomical involvement is in sharp contrast with the apparent rarity of clinical frontal dysfunction in stroke. It is remarkabl...
journal_title:European neurology
pub_type: 杂志文章,评审
doi:10.1159/000117068
更新日期:1994-01-01 00:00:00
abstract::Early traumatic epilepsy, whose frequency is rather high, especially in children, is usually characterized by focal motor seizures (57%) or by generalized seizures (43%). The association of both running and laughing fits in the same patient on the contrary is quite exceptional, as only three cases have been reported i...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114830
更新日期:1977-01-01 00:00:00
abstract::We performed serial neurophysiological tests in 22 patients with confirmed Guillain Barré syndrome (GBS) for 12 months following onset of the disease. Twenty-five age- and sex-matched healthy controls were also tested. Tests included nerve conduction and automated quantitative thermal threshold measurements. The commo...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116841
更新日期:1992-01-01 00:00:00
abstract::Lumbar CSF concentrations of homovanillic acid (HVA) and 5-hydroxyindole-acetic acid (5HIAA) have been determined in ten patients suffering from chronic brain post-traumatic syndromes, at various length of time after the brain trauma. Lower concentrations of HVA (mean 21 +/- 14 mug/ml) compared to controls (mean 46 +/...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114709
更新日期:1975-01-01 00:00:00
abstract::The role of genetics in Parkinson's disease (PD), previously controversial, is now supported by several studies. A major breakthrough has been the discovery of a single gene defect in familial Parkinson's disease. A single base pair change at position 209 from G to A (G209A) in the fourth exon of the alpha-synuclein g...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000008008
更新日期:1999-01-01 00:00:00
abstract::Temporal arteritis (TA) is a vasculitis involving mainly cranial branches of the aorta that can lead to ischemic complications such as amaurosis or ischemic stroke. Increment in the platelet count has been described in the acute period of the disease. We studied retrospectively the platelet count in patients with TA, ...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000052088
更新日期:2001-01-01 00:00:00
abstract:OBJECTIVES:We attempted to investigate changes of the pedunculopontine nucleus (PPN) according to walking ability in chronic stroke patients, using diffusion tensor imaging (DTI). METHODS:55 consecutive chronic stroke patients who were not able to walk due to injury of the corticospinal tract (CST) at stroke onset and...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000324152
更新日期:2011-01-01 00:00:00
abstract::Nocturnal sleep patterns were registered from 6 parkinsonian patients treated with bromocryptine, ("-Br-L-ergocryptine, C.B.-154) a dopamine-like agonist. No significant differences in their sleep patterns were found in comparison with patients treated with L-dopa. Since bromocryptine acts direct on the dopaminergic r...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114973
更新日期:1978-01-01 00:00:00
abstract::Ten patients with left-sided visuospatial neglect due to cerebrovascular accident were monitored using the Behavioural Inattention Test (BIT) at weekly intervals, on a long-term basis. The novel finding was that some patients showed occasional but transitory marked improvement in their visuospatial neglect at times pr...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000117028
更新日期:1994-01-01 00:00:00
abstract::A 21-year-old male developed back pain, fever, and rapidly progressive quadriparesis. Lumbar tap yielded frank pus which was confirmed on magnetic resonance imaging (MRI) to be located mainly in the cervical epidural space. Conservative antibiotic remedy was partially effective for restoration of the neurological defi...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116855
更新日期:1992-01-01 00:00:00
abstract::The central motor pathways were studied with the technique of percutaneous electrical stimulation of the central nervous system in 40 normal volunteers. There were no significant differences between the right and left sides, or between males and females. A linear correlation between the cortical latency (Lcor) or the ...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116396
更新日期:1989-01-01 00:00:00
abstract:AIM:To determine whether corpus callosum atrophy predicts future clinical deterioration in multiple sclerosis. METHODS:In 39 multiple sclerosis patients the area of corpus callosum in the sagittal plane, T2 and T1 lesion volumes, brain parenchymal fraction and brain atrophy were determined at baseline and 1 year after...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000337683
更新日期:2012-01-01 00:00:00
abstract:BACKGROUND:The aim of this study was to assess the plasma levels of factor VIII (FVIII), von Willebrand factor (vWF) and their association in patients with cerebral venous sinus thrombosis (CVST). METHODS:We prospectively included 25 CVST patients admitted to the university hospital and 53 voluntary subjects as a cont...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000331048
更新日期:2011-01-01 00:00:00
abstract::Direct evidence of the anatomical localization of brain function is provided by functional neurological changes during awake surgery combined with data from preoperative functional magnetic resonance imaging and diffusion tensor imaging studies. The goal of the present study was to analyze the etiology and mechanism o...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000258636
更新日期:2010-01-01 00:00:00
abstract::The aim of this work was to quantify the accumulation of iron in the basal ganglia in multiple sclerosis (MS) patients and in a control group, and to investigate the relationship between iron accumulation and other parameters assessed in MS, i.e. lesion load (LL) and brain parenchymal fraction (BPF). Magnetic resonanc...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000279305
更新日期:2010-01-01 00:00:00