Abstract：
:The phosphomannosyl receptor system is responsible for both the receptor-mediated endocytosis and the intracellular transport of lysosomal enzymes. In the present study this receptor system was examined in affected muscles of patients with various neuromuscular diseases. The total activity of beta-N-acetyl-glucosaminidase, a marker enzyme of lysosomal hydrolases, was significantly elevated in the patients with myopathies (polymyositis and muscular dystrophies) but only slightly increased in those with neurogenic muscle atrophies (amyotrophic lateral sclerosis, polyneuropathy or other neurogenic muscle disease). The increase was most prominent in the group of polymyositis. The content of phosphomannosyl receptors was increased in the patients with myogenic muscle disease but not in those with neurogenic disease. The receptor binding of lysosomal enzymes was saturable and inhibited with mannose 6-phosphate showing the typical characteristics of phosphomannosyl receptors. The characteristics of the receptors were very similar both to control and to diseased muscle samples. When surveying all the material, the content of phosphomannosyl receptors correlated highly significantly with the muscular activity of beta-N-acetylglucosaminidase, muscle atrophy index, and serum creatine kinase activity.
journal_name
Acta Neurol Scandjournal_title
Acta neurologica Scandinavicaauthors
Salminen A,Marjomäki V,Tolonen U,Myllylä VVdoi
10.1111/j.1600-0404.1988.tb05941.xsubject
Has Abstractpub_date
1988-06-01 00:00:00pages
461-7issue
6eissn
0001-6314issn
1600-0404journal_volume
77pub_type
杂志文章abstract：:Treatment with intraventricular pentosan polysulphate (PPS) might be beneficial in patients with Creutzfeldt-Jakob disease. We report a 68-year-old woman with sporadic Creutzfeldt-Jakob disease who received continuous intraventricular PPS infusion (1-120 microg/kg/day) for 17 months starting 10 months after the onset ...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2009.01272.x
更新日期：2010-02-01 00:00:00
abstract：OBJECTIVES:The aim of this pilot study was to evaluate a psychological therapy program used in the treatment of multiple sclerosis (MS) and including cognitive/behavioral strategies, relaxation training and physical exercise. MATERIAL AND METHODS:The participants were 29 patients with MS recruited from an outpatient u...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1034/j.1600-0404.2003.00077.x
更新日期：2003-06-01 00:00:00
abstract：:16 boys with Duchenne muscular dystrophy (DMD) were treated with sodium selenite and vitamin E for one year. One group of patients (10 boys) was examined using a battery of tests to assess muscular strength and function and cardiopulmonary function, and by CT scanning of 2 muscle groups over the 4 years prior to treat...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1988.tb03681.x
更新日期：1988-11-01 00:00:00
abstract：BACKGROUND:No systematic study has been made to separate age-related clinical deterioration and dysfunctional brain areas from those associated with Parkinson's disease (PD). METHODS:This study included 73 de novo patients with PD and 43 age-matched controls. All subjects underwent [(18)F]-fluorodeoxy glucose (FDG) po...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2010.01446.x
更新日期：2011-09-01 00:00:00
abstract：:Multiple sclerosis (MS) is associated with autoimmune disorders (AIDs) in individual patients, and limited data suggest a possible familial association of MS and AIDs; however, no systematic study has been conducted on the occurrence of AIDs in the families of MS patients. Using a standardized interview focused on AID...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1034/j.1600-0404.2000.101001036.x
更新日期：2000-01-01 00:00:00
abstract：OBJECTIVES:Subjective feelings of memory decline are fairly common among the elderly. The causes of this are heterogeneous, and may be related to both affective and cognitive disorders. We attempted to explore the associations between subjective and cognitive measures. MATERIALS AND METHODS:Healthy subjects were studi...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2009.01159.x
更新日期：2010-03-01 00:00:00
abstract：OBJECTIVE:The aim was to investigate risk factors separately and in sum in relation to long-term mortality after arterial cerebral infarction in young adults. MATERIALS AND METHODS:Mortality in relation to the number of risk factors (smoking, hypertension, hypercholesterolemia, diabetes mellitus, myocardial infarction...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2012.01681.x
更新日期：2013-02-01 00:00:00
abstract：:The influence on the cystometrogram of repeated filling and varying filling rates was studied in two groups of neurological patients, one with normal and one with uninhibited neurogenic bladder. A technique which permits an even bladder filling and a continuous recording of the intravesical pressure was used. The effe...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：
更新日期：1981-09-01 00:00:00
abstract：:In Sweden, the annual incidence of surgery for hydrocephalus was 3.4 per 100,000 adults between the years 1996 and 1998. The most common indication for surgery was normal pressure hydrocephalus (NPH; 47%), followed by high-pressure hydrocephalus (27%). Seventy-three percent of the patients had communicating hydrocepha...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2005.00451.x
更新日期：2005-08-01 00:00:00
abstract：OBJECTIVES:The aim of this open label pilot study was to evaluate the efficacy and tolerability of levetiracetam (LEV) as 'de novo' monotherapy in children and adolescents with late onset childhood occipital epilepsy-Gastaut type (COE-G). MATERIAL AND METHODS:Twelve patients suffering from COE-G were enrolled in this ...
journal_title：Acta neurologica Scandinavica
pub_type： 临床试验,杂志文章,多中心研究
doi：10.1111/j.1600-0404.2009.01264.x
更新日期：2009-11-01 00:00:00
abstract：:The clinical indications for temporal artery biopsy were explored using clinical decision analysis. The analysis indicated that biopsy is most useful in patients with a high likelihood of steroid side effects. It is nearly always useful when the pre-biopsy likelihood of temporal arteritis is low, but loses its value w...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1988.tb03627.x
更新日期：1988-08-01 00:00:00
abstract：:Outcome was studied prospectively in 28 consecutive patients with occlusion of the middle cerebral artery (MCA). They comprise a subgroup of 101 consecutive patients with TIA or stroke less than or equal to 75 years of age, admitted within 72 h after the stroke. Cerebral angiography and CT-scan were performed within 1...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1991.tb04692.x
更新日期：1991-04-01 00:00:00
abstract：:In this study levels of neuron-specific enolase (NSE), S-100 protein (S-100) and myelin basic protein (MBP) in cerebrospinal fluid (CSF) of children and adults with distinct neurological disorders were examined. A previous study from our department demonstrated age related reference values for these brain-specific pro...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1995.tb01696.x
更新日期：1995-09-01 00:00:00
abstract：OBJECTIVE:To compare the sensitivity and specificity of isoelectric focusing (IEF) with immunofixation, agarose gel electrophoresis (AGE) and the IgG index in detecting intrathecally synthesized IgG in multiple sclerosis (MS) and in other nervous system disorders. MATERIALS AND METHODS:Cerebrospinal fluid (CSF) and se...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1034/j.1600-0404.2000.102005322.x
更新日期：2000-11-01 00:00:00
abstract：:Both carotid endarterectomy (CEA) and carotid artery stenting (CAS) are common treatments for carotid artery stenosis. Several randomized controlled trials (RCTs) have compared CEA to CAS in the treatment of carotid artery stenosis. These studies have suggested that CAS is more strongly associated with periprocedural ...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章,评审
doi：10.1111/ane.13062
更新日期：2019-04-01 00:00:00
abstract：:A case of necrotizing brain stem encephalitis due to listeria monocytogenes is described in a 48-year-old man who had brain stem encephalitis of complicated course and with selective destruction of the vasomotoric and respiratory centers. He developed that very rare Ondine's curse syndrome, being only able to breathe ...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1988.tb05949.x
更新日期：1988-06-01 00:00:00
abstract：OBJECTIVE:To clarify the optimum trace count for jitter calculation. MATERIALS AND METHODS:Single-fiber electromyography was performed during voluntary contraction (VC-SFEMG) of extensor digitorum muscle in six healthy and five myasthenic subjects. Twenty single-fiber action potential (SFAP) pairs were recorded from e...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1034/j.1600-0404.2003.00120.x
更新日期：2003-10-01 00:00:00
abstract：OBJECTIVES:To determine headache characteristics in the obstructive sleep apnea syndrome (OSAS) and in insomnia. PATIENTS AND METHODS:Fifty-six OSAS patients and 50 insomnia patients were assessed in the same time period. Patients in both groups underwent an accurate interview to investigate headache occurrence and it...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2005.00372.x
更新日期：2005-05-01 00:00:00
abstract：OBJECTIVES:Health-related quality of life (HRQL) has become an important outcome criterion of medical interventions, but was hardly studied in patients with facial spasms. MATERIALS AND METHODS:Patients with blepharospasm or hemifacial spasm treated with botulinum toxin were included. A healthy control group sociodemo...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2004.00357.x
更新日期：2005-01-01 00:00:00
abstract：:Magnetic resonance imaging (MRI) has enabled ante mortem diagnosis of Hallervorden Spatz disease (HSD). Childhood-onset cases are the most common type and usually present with progressive dystonia and dementia. The duration of illness is 15 to 20 years, leading to death. Presentation in adulthood and infancy have also...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1034/j.1600-0404.2001.103003201.x
更新日期：2001-03-01 00:00:00
abstract：:Neuropsychiatric symptoms such as delusions and misidentifications have been reported in dementia ranging from 10% to 73% in Alzheimer's disease (AD) patients and up to 40% in multi-infarct dementia (MID) patients. The aim of this study was to investigate in 61 AD and 31 MID patients both the frequency and the content...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1993.tb04177.x
更新日期：1993-07-01 00:00:00
abstract：:In a double-blind cross-over trial with placebo, FPL 57787, a chromone derivate, was tested on 15 patients as a prophylactic against migraine. There was found to be no effect. ...
journal_title：Acta neurologica Scandinavica
pub_type： 临床试验,杂志文章
doi：10.1111/j.1600-0404.1979.tb02923.x
更新日期：1979-03-01 00:00:00
abstract：:Although intravenous (i.v.) heparin is widely used as the first line treatment for cerebral venous and sinus thrombosis (CVST), the most appropriate therapy for this disease is still controversial. We report 2 cases of CVST who were successfully treated by means of loco-regional thrombolysis with urokinase. In the fir...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1034/j.1600-0404.2001.00058.x
更新日期：2001-01-01 00:00:00
abstract：OBJECTIVES:To investigate the association between tumor necrosis factor-alpha (TNF-alpha) G-308A, tumor necrosis factor-beta (TNF-beta) G+252A and interleukin-4 (IL-4) C-590T polymorphisms and susceptibility to multiple sclerosis (MS) development and clinical course of the disease. MATERIALS AND METHODS:Two hundred an...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2006.00743.x
更新日期：2007-03-01 00:00:00
abstract：:By a rapid PCR-based method to assess the 8344 mtDNA mutation associated with MERRF disease, we have studied DNA from blood samples of 10 individuals belonging to a family spanning four generations in which one patient showed the complete MERRF phenotype, three other members were less severely affected, while the rema...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1993.tb05368.x
更新日期：1993-12-01 00:00:00
abstract：:From December 1976 through March 1982, 188 patients entered an open non-random study carried out on hospitalized patients with a history of transient ischemic attacks or amaurosis fugax. Ninety-two patients received peroral anticoagulants usually combined with heparin treatment during the first days of treatment, and ...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.1985.tb03232.x
更新日期：1985-06-01 00:00:00
abstract：BACKGROUND AND PURPOSE:The role of the natural anticoagulants, antithrombin III (AT III), protein C (PC), and protein S (PS), in patients with mild to moderate ischemic stroke remains uncertain. We aimed to find out whether their levels in peripheral blood correlated with the severity of neurological deficit or can pre...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1034/j.1600-0404.2002.1o112.x
更新日期：2002-02-01 00:00:00
abstract：OBJECTIVES:Orthostatic hypotension (OH) and postural orthostatic tachycardia syndrome (POTS) are well-known causes of orthostatic intolerance (OI). In addition, there are OI patients who are characterized by the symptoms of OI and lack of abnormal findings in head-up tilt (HUT) test. The aim of this study was to determ...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/ane.12516
更新日期：2016-08-01 00:00:00
abstract：OBJECTIVES:Sparse information on dose-response characteristics for initial antiepileptic drug monotherapy in children with idiopathic generalized epilepsy (IGE) is available. The aim of this study is to characterize the therapeutic dose of valproate in children with newly diagnosed IGE. MATERIALS AND METHODS:Effect of...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章
doi：10.1111/j.1600-0404.2009.01308.x
更新日期：2010-03-01 00:00:00
abstract：:For patients with Parkinson's disease (PD), excessive daytime sleepiness (PD-EDS) is a debilitating non-motor symptom and may be affected by mood symptoms, especially depression and anxiety. Few neuroimaging works have attempted to identify the neural features of PD-EDS, but various findings were reported. The purpose...
journal_title：Acta neurologica Scandinavica
pub_type： 杂志文章,评审
doi：10.1111/ane.12704
更新日期：2017-08-01 00:00:00
