Abstract:
:Continuing the previously published clinical development of a case of adult metachromatic leukodystrophy (MLD), we now describe the terminal phase and death (at 46 years of age) of our patient. The final phase was characterized clinically by progression of generalized peripheral neuropathy, advanced extrapyramidal and pyramidal tract symptomatology, dementia and brainstem dysfunction. First biochemical results show a moderate relative increase (3- to 5-fold) of sulfatides in the frontal lobe white matter but not in the cortex. The analysis of fatty acids in total lipid extract shows a decrease of long-chained fatty acids in favor of short-chained fatty acids, this change is more pronounced in white matter in the cortex. The clinical course and biochemical results are discussed in relation to previous cases analyzed by us. Epidemiological aspects especially emphasize routine serach for MLD amongst patients with neuropsychiatric symptomatology showing unusual psychoses, presenile dementias or unspecific disturbance of motor coordination possibly with electroneurographic evidence of peripheral neuropathy.
journal_name
Eur Neuroljournal_title
European neurologyauthors
Seidel D,Heipertz R,Goebel HH,Duensing I,Pilz Hdoi
10.1159/000115164subject
Has Abstractpub_date
1980-01-01 00:00:00pages
288-93issue
5eissn
0014-3022issn
1421-9913journal_volume
19pub_type
杂志文章abstract:OBJECTIVES:Little is known about optic radiation (OR) injury in intracerebral hemorrhage (ICH). We attempted to investigate OR injury in patients with ICH by diffusion tensor imaging (DTI). METHODS:Forty-three consecutive patients with putaminal hemorrhage and 40 normal healthy control subjects were recruited. DTI dat...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000345271
更新日期:2013-01-01 00:00:00
abstract::Serum from patients with multiple sclerosis (MS) and other diseases was added to the medium perfusing isolated hemisected frog spinal cord, and the effect on ventral root responses (VRR) tested. As control, a standardized commercial human serum (Moni-trol I) was used. In 25 out of 40 spinal cord preparations Moni-trol...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114824
更新日期:1977-01-01 00:00:00
abstract:BACKGROUND:Intravenous thrombolysis (IVT) with urokinase is the standard reperfusion therapy for acute cerebral infarction (ACI) in China. Only about 30% patients who use urokinase for IVT can recanalize. Therefore, this study aimed to analyze the influencing factors of recanalization after IVT using urokinase in ACI p...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000507288
更新日期:2020-01-01 00:00:00
abstract:BACKGROUND:Locked-in syndrome (LIS) is a condition characterized by quadriplegia and anarthria. The most common cause is a ventral pontine lesion due to atherosclerotic basilar artery disease. METHODS:Cases with LIS were prospectively identified among the patients with acute ischemic stroke over 3 years, between 2009 ...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000345272
更新日期:2013-01-01 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::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 disturba...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114818
更新日期:1977-01-01 00:00:00
abstract::This study investigates the remote effects of botulinum toxin injections by examining the motor unit architecture of noninjected distant muscles. In 21 dystonia patients treated with botulinum toxin (n = 11, mean cumulative dose = 815 mU; n = 10, mean cumulative dose = 7,207 mU) and 10 control individuals, a blinded s...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000008222
更新日期:2000-01-01 00:00:00
abstract::We examined a 29-year-old woman who had language disturbances and memory impairment after a left thalamic infarction. MRI showed injury that was limited to the tuberothalamic artery territory. Beside reduced voice volume and verbal memory trouble, she presented with aspontaneity, loss of psychic self-activation and af...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116930
更新日期:1993-01-01 00:00:00
abstract::Fluctuations in motor performance and dyskinesias are a common problem in the long-term management of Parkinson's disease. The pharmacokinetics and the pharmacodynamics of levodopa, loss of presynaptic dopamine terminals, alterations in postsynaptic dopamine receptor sensitivity, or changes in the modulatory influence...
journal_title:European neurology
pub_type: 杂志文章,评审
doi:10.1159/000118882
更新日期:1996-01-01 00:00:00
abstract::Acute ischemia of the brain induces a cascade of biochemical and physiological events. The final consequences depend on the fact whether ischemia is of transient or permanent, total or partial nature. Alteration of extracellular potassium concentration, intracellular calcium and potassium concentration, development of...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116058
更新日期:1986-01-01 00:00:00
abstract:BACKGROUND:Transient ischemic attacks (TIAs) bear a presumed high risk of early recurrence of stroke. Data in the literature, however, are inconsistent, as recurrence rates range from 9.5 to 20%, at 90 days. AIMS:The study was designed to determine the risk of stroke after TIA. METHODS:94 consecutive patients referre...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000332999
更新日期:2012-01-01 00:00:00
abstract::Experimental results suggested a modulation or 'programming' interaction of delta sleep-inducing peptide (DSIP) with endogenous opioid-peptidergic systems and exogenous intracerebrally or systemically administered morphine and amphetamine. The induction of cerebral MAO-A activity, a pronounced influence on the circadi...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115716
更新日期:1984-01-01 00:00:00
abstract::Henri Duret (1849-1921) was a surgeon whose training started in the laboratory of Jean-Martin Charcot and Alfred Vulpian at La Salpêtrière in 1874. Using injections of colored gelatine, Duret was the first to describe the distribution of supply arteries in the brainstem and then in the cortex. His descriptions correla...
journal_title:European neurology
pub_type: 传,历史文章,杂志文章
doi:10.1159/000361046
更新日期:2014-01-01 00:00:00
abstract:AIMS:We conducted this study to investigate the independent association of medial temporal atrophy (MTA) and white matter hyperintensities (WMH) with cognitive impairments of Alzheimer's disease (AD) patients and the interaction between MTA and WMH. METHODS:From 13 centers, a total of 216 AD patients were consecutivel...
journal_title:European neurology
pub_type: 杂志文章,多中心研究
doi:10.1159/000329277
更新日期:2011-01-01 00:00:00
abstract::The biochemical process underlying Parkinson's disease is dopamine cell death of the nigrostriatal system. The age-dependent cell death is now proposed to be elicited by the formation of free hydroxy radicals which are formed from hydrogen peroxide, a product of oxidation of dopamine by monoamine oxidase, especially t...
journal_title:European neurology
pub_type: 杂志文章,评审
doi:10.1159/000118535
更新日期:1993-01-01 00:00:00
abstract::Previous findings regarding the role of TNF-alpha-308 gene polymorphism in multiple sclerosis (MS) are contradictory. The aim of this study was to investigate the possible influence of TNF-alpha-308 polymorphism on MS susceptibility and the MS disease process in a Croatian and Slovenian population. Genotyping was perf...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000099159
更新日期:2007-01-01 00:00:00
abstract::Camptocormia (bent spine syndrome, cyphose hystérique) is an abnormality characterized by severe forward flexion of the thoracolumbar spine which typically increases during walking or standing and completely disappears in supine position. Camptocormia can be due to central nervous system diseases, such as Parkinson's ...
journal_title:European neurology
pub_type: 杂志文章,评审
doi:10.1159/000314897
更新日期:2010-01-01 00:00:00
abstract:INTRODUCTION:We investigated the predictive value of standard neurological evaluation, a commercially available biomarker assay and neuroimaging in the subacute phase for outcome after thrombolytic therapy in ischemic stroke. METHODS:Thirty-four consecutive ischemic stroke patients were evaluated by means of the NIH S...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000215876
更新日期:2009-01-01 00:00:00
abstract::64 patients on hemodialysis were investigated. The mean duration of dialysis was 43 months. In all the patients, serum aluminium levels, systolic blood pressure (averaged over a period of 6 weeks) and the EEG were investigated. Psychological testing to assess the level of intelligence (IQ) was also performed. The seru...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115500
更新日期:1982-01-01 00:00:00
abstract::Changes in the maximal rate of some cerebral enzymatic activities related to 400ene transduction and neurotransmission (lactate dehydrogenase; citrate synthase and malate dehydrogenase; total NADH-cytochrome c reductase and cytochrome oxidase; glutamate dehydrogenase; acetylcholine esterase) were assayed both in the c...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115242
更新日期:1981-01-01 00:00:00
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::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::Trials of pharmacological intervention for acute stroke show several points of similarity with legal trials by jury. The relationships between sponsoring organisation and regulatory authorities and the roles of the many other participants are described. Only through a partnership can the common aim of developing impro...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000068509
更新日期:2003-01-01 00:00:00
abstract:BACKGROUND:The aim of this study was to elucidate the influence of insular infarction on blood pressure (BP) variability and outcomes according to the region of the insular cortex affected. METHODS:A total of 90 patients diagnosed with acute unilateral ischemic stroke were registered. The BP variability was calculated...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000486306
更新日期:2018-01-01 00:00:00
abstract::The preventive effect of Atrovent Dosier-Aerosol on the airway flow resistance (Raw) caused by Tensilon was studied on 10 patients suffering from myasthenia gravis. Atrovent completely prevented the increase of Raw in 8 cases and considerably decreased it in 2 cases. The measure of the reaction observed may be influen...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115206
更新日期:1981-01-01 00:00:00
abstract::T lymphocytes were determined according to their ability to form rosettes with sheep erythrocytes, in cerebrospinal fluid (CSF) from 120 patients, and in peripheral blood from 59 patients. Normal CSF contained 74.9 +/- 9.6% T lymphocytes. Increased T lymphocyte percentage was found in CSF from patients with active mul...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115074
更新日期:1979-01-01 00:00:00
abstract::To examine the possible influence of the blood-CSF barrier function on mathematical formulas for estimating intrathecal IgG production (IgG index, Tourtellotte's formula [TOURT], Schuller and Sagar's formula [Schull], and Reiber and Felgenhauer's formula [IgG(loc)]), we analysed serum and CSF samples from 105 healthy ...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116920
更新日期:1993-01-01 00:00:00
abstract::Oxidative stress and generation of reactive oxygen species are believed to be implicated in Parkinson's disease (PD). Erythrocyte activity of superoxide dismutase (SOD) and catalase, the blood glutathione system, and plasma levels of thiobarbituric-acid-reactive substances (TBARS) were measured in 80 PD patients. Thes...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000103641
更新日期:2007-01-01 00:00:00
abstract::The pathogenesis of primary degenerative dementia is still unknown and current therapeutic approaches can aim only at ameliorating some of the symptoms associated with this brain disease. At present, most approaches, attempt to correct a cholinergic deficit. Although the therapeutic benefits obtained so far with curre...
journal_title:European neurology
pub_type: 杂志文章,评审
doi:10.1159/000116479
更新日期:1989-01-01 00:00:00
abstract::A critical analysis of cases of systemic lupus erythematosus (SLE) and chorea is presented. Choreatic movements occurred early in the course of the disease in most cases; the duration varied from several days to 3 years. No sex predominance was found and no relationship could be demonstrated between chorea and other n...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115743
更新日期:1984-01-01 00:00:00