Abstract:
:We investigated the efficacy of botulinum toxin A (BtxA) therapy in patients with atypical parkinsonian disorders (APD) exhibiting different types of disabling focal dystonia unresponsive to oral drug therapy. Eight patients with functionally disabling focal dystonia out of a series of 60 consecutive patients with APDs regularly treated at our outpatient movement disorders clinic were included. Patients were diagnosed according to established criteria and had disabling limb dystonia (n=4) or craniocervical dystonia (n=4) unresponsive to oral pharmacological treatment. Localization and dose of BtxA injections was determined individually based on clinical examination as well as EMG in patients with limb dystonia. BtxA reduced dystonic symptoms in all patients; only one developed a transient local side-effect. BtxA was particularly effective in the long-term treatment (up to 50 months) of blepharospasm associated with progressive supranuclear palsy (PSP). BtxA also alleviated PSP-associated retrocollis and orofacial dystonia with lower lip retraction associated with PSP and multiple system atrophy. BtxA treatment of limb dystonia in corticobasal degeneration (CBD) temporarily improved hand and arm function in early-disease stages while treatment in advanced stages reduced pain, facilitated hygiene and prevented secondary contractures. Limb dystonia was also alleviated by BtxA therapy in one patient with neuronal multisystem degeneration of undetermined cause. The results suggest that BtxA therapy may represent an effective means of alleviating disabling focal dystonia in different APDs. Particularly in early stage APD with disabling limb dystonia local BtxA injections may result in functional improvement.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Müller J,Wenning GK,Wissel J,Seppi K,Poewe Wdoi
10.1007/s004150200009keywords:
subject
Has Abstractpub_date
2002-03-01 00:00:00pages
300-4issue
3eissn
0340-5354issn
1432-1459journal_volume
249pub_type
临床试验,杂志文章abstract:BACKGROUND:Spinal muscular atrophy (SMA) is caused by a homozygous deletion of the survival motor neuron (SMN)1 gene. The nearly identical SMN2 gene plays a disease modifying role. SMA is classified into four different subtypes based on age of onset and clinical course (SMA types 1-4). The natural history of early onse...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0929-0
更新日期:2008-09-01 00:00:00
abstract::Cerebral ischemia of 15 min was produced in rats by increasing intracranial pressure above the systolic blood pressure level by infusing artificial cerebrospinal fluid into the cisterna magna. Pulmonary edema was prevented by blocking the vasomotor response with a ganglioplegic agent, and reducing the fluid load by pe...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00316314
更新日期:1977-12-01 00:00:00
abstract::Although multiple sclerosis (MS) is considered to be an inflammatory demyelinating disease, increasing evidence indicates that it is also an axonal pathology; indeed, studies of experimental allergic encephalitis showed that several neuronal proteins such as synapsins take part in the pathogenesis of the axonal dysfun...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0293-7
更新日期:2004-02-01 00:00:00
abstract::Horizontal eye-movement responses of four patients with Alzheimer-type dementia were recorded using the infrared oculographic technique. Abnormally frequent saccadic intrusions occurred during the fixation and slow, smooth pursuit tasks in all four patients. Saccadic intrusions have previously been reported in the lit...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313742
更新日期:1983-01-01 00:00:00
abstract::Biomarkers beyond clinical assessment are needed in patients who suffer from amyotrophic lateral sclerosis (ALS). Here, single-voxel proton magnetic resonance spectroscopy ((1)H MRS) of the gray matter of the motor cortex and the white matter including the pyramidal tracts was used to investigate concentrations of N-a...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0495-2
更新日期:2007-08-01 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::Parkinson's disease (PD) is the most common neurodegenerative movement disorder. Recent advances in genetics and pathophysiology have led to new insights into the pathogenesis of PD. Ten loci have been linked to hereditary PD. Mutations in alpha-synuclein and ubiquitin carboxy hydrolase L1 (UchL1) cause autosomal domi...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-003-1304-9
更新日期:2003-10-01 00:00:00
abstract::The purpose of this study is to monitor the development of anti-natalizumab antibodies to evaluate their first appearance in multiple sclerosis patients, since their presence has been associated with a reduction in the efficacy of the treatment and an increase of adverse events. A total of 134 multiple sclerosis patie...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s00415-013-6991-2
更新日期:2013-09-01 00:00:00
abstract::Discontinuation of natalizumab can lead to severe rebound of disease activity in patients with relapsing-remitting multiple sclerosis (RRMS); nevertheless, the treatment regimen in this clinical situation remains controversial. We report the case of a 25-year-old male patient with RRMS who was clinically stable under ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-017-8622-9
更新日期:2017-12-01 00:00:00
abstract::Four patients from three unrelated families, with clinical and electrophysiological findings compatible with the diagnosis of hereditary motor and sensory neuropathy, are presented. The molecular analysis showed that the affected individuals were homozygous for the mutation in the X25 gene, characteristic of Friedreic...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-002-0902-2
更新日期:2002-11-01 00:00:00
abstract::The type and frequency of neurological manifestations of Behçet's disease (BD) vary with ethnicity. We analyzed the neurological manifestations of BD in Japanese patients. All patients undergoing treatment at one of the two Yokohama City University hospitals from July 1991 to December 2007 and who fulfilled the Japane...
journal_title:Journal of neurology
pub_type: 杂志文章,多中心研究
doi:10.1007/s00415-010-5454-2
更新日期:2010-06-01 00:00:00
abstract::The hereditary spastic paraplegias (HSPs) are a group of genetic conditions in which spastic paralysis of the legs is the principal clinical feature. This is caused by a relatively selective distal axonal degeneration involving the longest axons of the corticospinal tracts. Consequently, these conditions provide an op...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7598-y
更新日期:2015-07-01 00:00:00
abstract:OBJECTIVE:Gluten neuropathy (GN) is the term used to describe peripheral neuropathy that occurs in patients with gluten sensitivity (GS) or coeliac disease (CD) in the absence of other risk factors. We aimed to describe the neurophysiological progression rate of GN across time and look into the potential role of geneti...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10137-6
更新日期:2021-01-01 00:00:00
abstract::Using a novel trial design, we prospectively examined the effect of intravenous immunoglobulin in seven patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a double-blind, placebo-controlled cross-over study. We suggest that the commonly used manual muscle testing and Rankin scale are not...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1007/BF00868527
更新日期:1996-03-01 00:00:00
abstract::Stroke in young adults is not a rare entity, and often provides difficult management decisions for neurologists. The knowledge gained from stroke in older adults does not transfer easily to this younger group given the different causes of stroke observed. Cardiac causes of stroke are common in this group, but often co...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-010-5647-8
更新日期:2010-11-01 00:00:00
abstract::Inherited ataxias are heterogeneous disorders affecting both children and adults, with over 40 different causative genes, making molecular genetic diagnosis challenging. Although recent advances in next-generation sequencing have significantly improved mutation detection, few treatments exist for patients with inherit...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-014-7476-7
更新日期:2014-11-01 00:00:00
abstract::We measured changes in brain magnetization transfer ratio (MTR) as a potential indicator of myelin density in brain tissue of patients with relapsing-remitting multiple sclerosis (RRMS) treated with delayed-release dimethyl fumarate (DMF) in the Phase 3 DEFINE study. DEFINE was a randomized, double-blind, placebo-cont...
journal_title:Journal of neurology
pub_type: 杂志文章,随机对照试验
doi:10.1007/s00415-014-7504-7
更新日期:2014-12-01 00:00:00
abstract::Thirteen patients with Wallenberg's lateral medullary syndrome (WLMS) were studied. Clinical and magnetic resonance imaging (MRI) evidence demonstrated infarction in the dorsolateral medulla which produced loss of pain and temperature sensation on one side of the face ipsilateral to the lesion in seven patients. Howev...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00874113
更新日期:1993-09-01 00:00:00
abstract::A case of CT-diagnosed brain-stem haematoma associated with signs of dorsal medullary involvement is reported. The patient recovered completely within 1 month. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313834
更新日期:1985-01-01 00:00:00
abstract::We present evidence that Willy Loman, the protagonist of Arthur Miller's play "Death of a Salesman", meets current diagnostic criteria for probable dementia with Lewy bodies. In particular, he presents with attentional deficits and executive dysfunction (with additional subtle visuoperceptual deficits) in addition to ...
journal_title:Journal of neurology
pub_type: 信件
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更新日期:2019-07-01 00:00:00
abstract::We report a rare association of spinocerebellar ataxia and motor neuron disease (MND) in a woman with genetically confirmed SCA2 who subsequently developed a rapidly progressive and fatal form of MND. Considering the rarity of these two neurological conditions, it is interesting to note that the concomitant occurrence...
journal_title:Journal of neurology
pub_type: 信件,评审
doi:10.1007/s00415-009-5237-9
更新日期:2009-11-01 00:00:00
abstract::Multiple Sclerosis (MS) is an inflammatory disease of the Central Nervous System with multifocal areas of demyelination. Although its etiology and pathogenesis remain controversial, several lines of evidence indicate that MS is mediated by a misdirected immune response against one or several myelin proteins. The invol...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150200026
更新日期:2002-04-01 00:00:00
abstract::Six patients with congenital myotonia and 4 patients with myotonic dystrophy have been examined clinically before and after the administration of N-propyl-ajmalin, an alkaloid frequently used as a cardiac antiarrhythmic drug. All patients but one reported a good to moderate improvement of their myotonic muscle stiffne...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00316381
更新日期:1975-09-01 00:00:00
abstract::Several reports in the literature describe the effects of low-dose (5 mg/kg/day) idebenone in significantly reducing cardiac hypertrophy in patients with Friedreich ataxia. However, the effects of idebenone on neurological function have not been reliably determined in these studies; when neurological parameters were r...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-009-1008-x
更新日期:2009-03-01 00:00:00
abstract::Brain metastasis is a common complication occurring in about 15-20% of all cancer patients. For the initial management, distinguishing between three types of presentation is essential: de novo brain metastasis, simultaneous presentation of both brain metastasis and the primary tumour (usually lung carcinoma), and the ...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s004150050191
更新日期:1998-03-01 00:00:00
abstract:BACKGROUND:The symptomatic treatment of myotonia and myalgia in patients with dystrophic and non-dystrophic myotonias is often not satisfactory. Some patients anecdotally report symptoms' relief through consumption of cannabis. METHODS:A combination of cannabidiol and tetrahydrocannabinol (CBD/THC) was prescribed as c...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09593-6
更新日期:2020-02-01 00:00:00
abstract:OBJECTIVE:To identify factors that impact on the ability of patients with multiple sclerosis (MS) to remain in work in order to make recommendations for future clinical management. METHODS:Cross-sectional studies using qualitative (phase one) and quantitative (phase two) methods. In phase one, 62 patients were intervi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-005-0765-4
更新日期:2005-08-01 00:00:00
abstract::This meta-analysis aims to compare percutaneous transluminal angioplasty (PTA) to medical treatment (MT) for symptomatic vertebral artery stenosis (SVAS) treatment. We searched PubMed, Springer, Google Scholar, Clinical Trials, Cochrane Central, Chinese National Knowledge Infrastructure, and China Biological Medicine ...
journal_title:Journal of neurology
pub_type: 杂志文章,meta分析,评审
doi:10.1007/s00415-016-8267-0
更新日期:2017-05-01 00:00:00
abstract::In vivo nuclear magnetic resonance spectroscopy (MRS) of the human brain is a recently developed technique which allows to assay noninvasively in vivo key molecules of brain metabolism. After a review of the origin of the signals detected by phosphorus and proton MRS of human brain, the impact of MRS on clinical neuro...
journal_title:Journal of neurology
pub_type: 社论,评审
doi:10.1007/BF02033352
更新日期:1994-05-01 00:00:00
abstract::Two siblings with multiple intracranial haemangiomatosis are reported, each having psychomotor retardation, epilepsy and gyriform type calcification in the occipital and frontal lobes. There were haemangiomas in one autopsy case with calcification in the deep layers of the cortex and subcortical white matter. ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313911
更新日期:1985-01-01 00:00:00