Abstract:
BACKGROUND:The role of electromyography (EMG) recorded from the external anal sphincter (EAS) in the diagnosis of atypical parkinsonian syndromes is a matter for continuous debate. Most studies addressing this issue are retrospective. METHODS:In this study, we prospectively investigated six patients with Parkinson's Disease (IPD), 14 patients with multiple system atrophy (MSA) and eight with progressive supranuclear palsy (PSP) using EMG of the EAS, motor-evoked potential (MEP) to the EAS and EMG of m. gastrocnemius and nerve conduction velocity measured at the sural nerve. Patients were followed up for 2 years to secure correct diagnosis. RESULTS:The mean duration of motor unit potentials (MUPs) recorded from the EAS was significantly longer in patients with MSA and PSP compared with MUPs recorded from patients with PD (P < 0.005 for both). There were no signs of diffuse loss of motor neurons or peripheral neuropathy. MEP revealed signs of supranuclear affection in patients with MSA, whereas in patients with PSP the mechanism is a focal loss of motor neurons in Onuf's nucleus. CONCLUSION:Abnormal EMG of the EAS is strongly suggestive of atypical parkinsonism and the pathophysiology may be different in patients with MSA and PSP.
journal_name
Acta Neurol Scandjournal_title
Acta neurologica Scandinavicaauthors
Winge K,Jennum P,Lokkegaard A,Werdelin Ldoi
10.1111/j.1600-0404.2009.01169.xsubject
Has Abstractpub_date
2010-03-01 00:00:00pages
198-203issue
3eissn
0001-6314issn
1600-0404pii
ANE1169journal_volume
121pub_type
杂志文章abstract:OBJECTIVES:To describe clinical, electrophysiological and genetic data of five unrelated Sicilian pedigrees harbouring a heterozygous Ser78Leu mutation in the myelin protein zero (MPZ) extracellular domain. MATERIALS AND METHODS:Clinical, electrophysiological and genetic findings of 16 patients were reported. Polymorp...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2008.01021.x
更新日期:2008-11-01 00:00:00
abstract::Three patients are described with muscular dystrophy and contractures. Although this disorder bears similarities to Emery-Dreifuss disease and variants previously described, absence of cardiomyopathy is a distinguishing feature. Electrodiagnostic testing and muscle biopsy are consistent with a myopathy. An autosomal d...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1986.tb03302.x
更新日期:1986-04-01 00:00:00
abstract::Beta-2-microglobulin (beta 2m) levels were studied in the cerebrospinal fluid (CSF) and plasma of 52 patients with clinically definite multiple sclerosis (MS) and of 14 with aseptic meningo-encephalitis (AM). Reference values for beta 2m were defined in 72 subjects of different age groups with tension headache (TH). P...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1989.tb03934.x
更新日期:1989-12-01 00:00:00
abstract::We investigated the types of syndrome, location, size and presumed causes of 72 patients with acute capsular lacunar infarction. Clinical syndromes were classified into pure motor hemiparesis (PM), sensorimotor stroke (SM) and ataxic hemiparesis (AH). Lesion sizes of AH were significantly smaller than those of PM (p <...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1993.tb04233.x
更新日期:1993-10-01 00:00:00
abstract::Three patients at Stage II of subacute sclerosing panencephalitis (SSPE) were treated with semipurified alpha-interferon (IFN) using different combinations of intrathecal and intravenous routes: 1 x 10(6) IU of alpha-IFN were given every other day up to a total of 15 x 10(6) IU. Transient improvement of neurological s...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1988.tb03639.x
更新日期:1988-09-01 00:00:00
abstract::Among industrial solvents in present use trichloroethylene belongs to those which have been the concern of most neurological and occupational investigations since the beginning of this century. Reports on a broad spectrum of neurotoxicity are however mostly based on case-studies and accidental circumstances. The objec...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1993.tb04101.x
更新日期:1993-03-01 00:00:00
abstract:BACKGROUND:Understanding how antiepileptic (AED) monotherapy influences normative test scores is of importance in the clinic for correct interpretation of neuropsychological profiles. Previous studies have primarily reported minor influence on neuropsychological raw scores, and the clinical relevance of these findings ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章,随机对照试验
doi:10.1111/j.1600-0404.2008.01109.x
更新日期:2009-03-01 00:00:00
abstract:OBJECTIVE:White matter (WM) integrity of Spinocerebellar ataxia 2 (SCA2) is poorly understood, more so in the early stages of SCA2. In this study, we evaluated the microstructural integrity of the WM tracts with an emphasis on the nature of in vivo pathological involvement in early SCA2. MATERIALS AND METHODS:We evalu...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13359
更新日期:2020-10-08 00:00:00
abstract::Supernatants of peripheral blood lymphocytes (PBL), containing lymphokines, isolated from MS patients and healthy subjects were tested for demyelinating activity in organotypic culture of the rat cerebellum. PBL were isolated on Ficoll-paque gradient and cultures in RPMI 1640 medium for 72 h with or without phytohemag...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1986.tb07853.x
更新日期:1986-09-01 00:00:00
abstract::The prophylactic effect of a 5-HT uptake inhibitor, femoxetine, was compared with that of propranolol in a double-blind crossover study of 6-months duration. 29 patients commenced the experiment. 3 subjects withdrew because of side effects and 2 because the program was inconvenient for them. In the 24 patients who con...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1983.tb04836.x
更新日期:1983-10-01 00:00:00
abstract:BACKGROUND:Thirty patients with glioblastoma multiforme (GBM) were treated by boron neutron capture therapy (BNCT) at the Studsvik facility in Sweden, in a clinical trial exploring a procedure in which 900 mg p-boronophenylalanine (BPA) per kilo body weight was infused in 6 h. OBJECTIVE:The present study was designed ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2007.00837.x
更新日期:2007-09-01 00:00:00
abstract:OBJECTIVE:Satisfactory anabolic reactions, including the activation of the hypothalamic-pituitary-adrenal (HPA) axis, are essential following severe traumatic brain injury (TBI) and aneurysmal subarachnoid hemorrhage (SAH). Many factors may influence this activation. This study aimed to investigate whether individuals ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13212
更新日期:2020-04-01 00:00:00
abstract:OBJECTIVE:To clarify the relationship between mitochondrial DNA (mtDNA) sequence variations and phenotypes in patients with A3243G mutation. MATERIALS AND METHODS:We studied whole mtDNA sequences in two families with A3243G mutation and characteristic clinical features. Two brothers in Family 1 had shown thiamine defi...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2002.01172.x
更新日期:2002-08-01 00:00:00
abstract::To clarify the central nervous and the cardiovascular mechanisms mediating the Cushing response (CR) during intracranial hypertension, the effects of continuous and prolonged expansion of an epidural supratentorial balloon was studied in six dogs. Regional cerebral blood flow, cardiac output, extracerebral tissue bloo...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1985.tb00872.x
更新日期:1985-09-01 00:00:00
abstract:OBJECTIVES:To study macrosomatognosia and microsomatognosia occurring as migraine aura symptoms. MATERIAL AND METHODS:Topological distribution of these body schema disturbances were assessed in a collection of 562 pictures submitted as entries to the 4 national Migraine Art competitions. RESULTS:The said symptoms wer...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2000.9s334.x
更新日期:2000-06-01 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:BACKGROUND:Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is an autoimmune disorder of the central nervous system (CNS). Interleukin (IL)-6 and IL-17A may play important roles in the pathogenesis of this disease. High-mobility group box protein 1 (HMGB1), a small but highly conserved ubiquitous protein, is rec...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12850
更新日期:2018-02-01 00:00:00
abstract::A two-phase epidemiological study of essential tremor was carried out by investigating a rural population aged over 40 years in 2 municipalities in southwestern Finland. In the first phase, the subjects answered a questionnaire as to whether they had experienced tremor during the month preceding the inquiry. In the se...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1982.tb03129.x
更新日期:1982-07-01 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::We described a patient with progressive non-syndromic hearing loss (NSHL) harboring the A3243G mutation in the mitochondrial DNA (mtDNA). Muscle biopsy showed scattered ragged-red, cytochrome c oxidase negative fibers, whereas the biochemical analysis of the mitochondrial respiratory chain complexes was normal. Restri...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2004.00254.x
更新日期:2004-07-01 00:00:00
abstract:OBJECTIVES:The Flt3 ligand (FL) is a cytokine with a neurotrophic and antiapoptotic activity in the central nervous system that induces the survival of neurons. The aim of the study was to measure levels of FL in amyotrophic lateral sclerosis (ALS) patients. MATERIALS AND METHODS:The study involved 23 ALS patients and...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2006.00704.x
更新日期:2006-09-01 00:00:00
abstract::A representative sample of 117 patients with definite multiple sclerosis (MS) was interviewed on pain syndromes. Chronic syndromes lasting more than one month included dysaestesthesia, low back pain, spasms, tonic seizures, tightening and painful sensations in the extremities. Acute syndromes included neuralgia, L'Her...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1991.tb04937.x
更新日期:1991-09-01 00:00:00
abstract:OBJECTIVES:To examine differences in cerebrovascular reactivity between multi-infarct and Alzheimer types of dementia. PATIENTS AND METHODS:Using transcranial Doppler, measurements of flow velocity in the middle cerebral arteries during apnea, thumb-to-finger opposition and verbal and design discrimination tasks were ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1998.tb05969.x
更新日期:1998-06-01 00:00:00
abstract::A prospective electrophysiological study of phrenic nerve was performed in 59 subjects undergoing open-heart surgery. The nerve was stimulated percutaneously at the neck and the diaphragmatic response was recorded with surface electrodes placed over the 8th intercostal space. The latency, amplitude, duration and area ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1995.tb07000.x
更新日期:1995-04-01 00:00:00
abstract::We describe eight patients from three families presenting with myoclonus, ataxia, infrequent seizures and minimal intellectual impairment. All were Arabs from different parts of the Arabian peninsula. The new consensus on terminology, genetic and clinical definition of Baltic myoclonus, Ramsay Hunt syndrome and Unverr...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1992.tb05126.x
更新日期:1992-11-01 00:00:00
abstract:OBJECTIVES:Mutations in the p150 subunit of the axonal transport protein dynactin (DCTN1) have been reported in patients with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). Given the common features of neurodegeneration in multiple sclerosis (MS), FTD and ALS, sequence variants of the DCTN1 gene...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.2007.00884.x
更新日期:2007-10-01 00:00:00
abstract::Sixteen patients with HTLV-1 associated myelopathy (HAM) were examined for the presence of HTLV-1 provirus genome by Southern blot analysis of genomic DNA from peripheral blood mononuclear (PBM) cells. Random integration of the provirus was detected in 14 of 16 HAM patients. By contrast, the provirus genome could not ...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/j.1600-0404.1990.tb00968.x
更新日期:1990-03-01 00:00:00
abstract:BACKGROUND:Mild malformation of cortical dysplasia (mMCD) with oligodendroglial hyperplasia (MOGHE) is an epilepsy-related pathologic entity highlighted in post-surgical specimens of frontal lobe epilepsy (FLE) patients. AIMS OF THE STUDY:We present two temporal lobe epilepsy (TLE) cases with MOGHE and discuss clinica...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.13142
更新日期:2019-10-01 00:00:00
abstract:OBJECTIVES:Patients with Parkinson's disease (PD) and essential tremor (ET) have a higher risk of cognitive impairment than age-matched controls. Only a few small studies (11-18 subjects per group) have directly compared the cognitive profile of these conditions. Our aim was to compare the cognitive profile of patients...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1111/ane.12752
更新日期:2017-11-01 00:00:00
abstract:OBJECTIVES:To report three cases of premature death in juvenile myoclonic epilepsy (JME), a benign form of idiopathic generalized epilepsy (IGE) in which no case of epilepsy-related death has been reported. MATERIAL AND METHODS:We retrospectively analyzed all medical records of JME patients first referred to two epile...
journal_title:Acta neurologica Scandinavica
pub_type: 杂志文章
doi:10.1034/j.1600-0404.2001.00003.x
更新日期:2001-09-01 00:00:00