Abstract:
:Two patients are described, in whom MRI detected unilateral signal abnormalities in the region of the inferior olivary nuclei, suggesting an early stage of olivary hypertrophy. MRI was performed 4 and 7 weeks respectively after traumatic brain-stem injury. Palatal myoclonus was concomitantly observed in one patient, while the other showed no evidence of segmental myoclonus at the time of examination. The authors conclude that MRI is highly sensitive in the detection of olivary hypertrophy and of traumatic lesions of the dentato-rubro-olivary pathway.
journal_name
J Neuroljournal_title
Journal of neurologyauthors
Birbamer G,Buchberger W,Kampfl A,Aichner Fdoi
10.1007/BF00867352subject
Has Abstractpub_date
1993-07-01 00:00:00pages
407-9issue
7eissn
0340-5354issn
1432-1459journal_volume
240pub_type
杂志文章abstract::Recently, a frequent prion protein gene (PRNP) polymorphism consisting of a methionine (M) for valine (V) substitution at codon 129 has been associated with cognitive impairment in elderly individuals. Down syndrome (DS) is associated with mental retardation and development of Alzheimer-like brain abnormalities. In th...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-003-1057-5
更新日期:2003-06-01 00:00:00
abstract::Acute relapses of multiple sclerosis (MS) are treated with intravenous methylprednisolone (IVMP), which speeds recovery from exacerbation. It is known that IVMP suppresses the immunological activation which occurs during an acute attack of MS. However, the specific target genes affected by this therapy remain obscure....
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-004-0516-y
更新日期:2004-10-01 00:00:00
abstract::The concentration of neopterin was measured in serum samples taken from individuals infected with HTLV-I: 5 from asymptomatic individuals, 1 from a patient with adult T-cell leukaemia and 30 from patients with tropical spastic paraparesis (TSP). In addition, cerebrospinal fluid (CSF) was available from 22 of the TSP p...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00810351
更新日期:1992-05-01 00:00:00
abstract::Familial amyloid polyneuropathy (FAP) is a progressive systemic autosomal dominant disease caused by pathogenic mutations in the transthyretin (TTR) gene. We studied clinical, electrophysiological, histopathological, and genetic characteristics in 15 (13 late-onset and two early-onset) patients belonging to 14 familie...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-013-7124-7
更新日期:2013-12-01 00:00:00
abstract::Although most patients with spinal muscular atrophy (SMA) are homozygous for deletion of the SMN1 gene, some patients bear one SMN1 copy with a subtle mutation. Detection of such an intragenic mutation may be helpful not only in confirming diagnosis but also in elucidating functional domains of the SMN protein. In thi...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0410-x
更新日期:2007-05-01 00:00:00
abstract::Stiff-person syndrome (SPS) is a rare condition of progressive muscular rigidity and spasm, frequently accompanied by other autoimmune conditions, an association which has been further strengthened by the discovery of anti-GAD antibodies and the response of SPS to immunotherapies. Intravenous immunoglobulin (IVIg) is ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6015-z
更新日期:2011-10-01 00:00:00
abstract::Pontocerebellar hypoplasia type 2 (PCH2) is a very rare autosomal recessive disorder. We report two unrelated female patients born to consanguineous parents presenting with this condition. Patient 1 showed a classical clinical/neuroradiological phenotype of PCH2 with dyskinesia/dystonia. Patient 2 had a neonatal onset...
journal_title:Journal of neurology
pub_type: 杂志文章
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更新日期:2002-05-01 00:00:00
abstract::Disease management is defined as any medical or pharmaceutical intervention designed to improve both outcomes for the patient and overall cost-effectiveness of the health plan. Disease management focuses on the patient throughout the entire course of the disease, involving both health providers and third-party payers....
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/pl00014806
更新日期:1998-08-01 00:00:00
abstract::Extra- and intraneural ganglionic cysts rarely involved peripheral nerves. They are found in the neighbourhood of large joints. Intraneural cysts prefer the deep peroneal nerve and cause intermittent pain and severe nerve damage. The ulnar nerve is affected most often at the wrist. There are different types of distal ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314606
更新日期:1975-06-09 00:00:00
abstract:OBJECTIVES:Evidences from either small series or spontaneous reporting are accumulating that SARS-CoV-2 involves the Nervous Systems. The aim of this study is to provide an extensive overview on the major neurological complications in a large cohort of COVID-19 patients. METHODS:Retrospective, observational analysis o...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-020-10251-5
更新日期:2020-10-07 00:00:00
abstract:OBJECTIVE:Amyotrophic lateral sclerosis type 8 (ALS8) is a familial form of motor neuron disease, with predominance of lower motor neuron degeneration, and is caused by mutation of the vesicle-associated membrane protein-associated protein B (VAPB). We aimed to compare the cognitive profile of patients with ALS8 and he...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09369-y
更新日期:2019-08-01 00:00:00
abstract::Ten female patients suffering from catamenial epilepsy were treated with a synthetic analogue of the gonadotrophin releasing hormone (GnRH) in addition to antiepileptic drugs. Three of the patients became seizure free, in four patients seizure frequency decreased and in one patient seizures were of shorter duration. I...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00810354
更新日期:1992-05-01 00:00:00
abstract::Non-motor symptoms (NMS) of Parkinson's disease (PD) have only recently been increasingly recognized for their impact on a patient's quality of life. In this study, we applied the validated, comprehensive self-completed NMS questionnaire for PD (NMS Quest) to 101 patients with young-onset PD (onset between 21 and 45 y...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-012-6600-9
更新日期:2013-01-01 00:00:00
abstract::Leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate (LBSL) is a rare, autosomal recessive disorder caused by mutations in the gene encoding a mitochondrial aspartyl-tRNA synthetase, DARS2. The disease is characterized by progressive spastic ataxia and magnetic resonance imaging (MRI) sh...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-011-6176-9
更新日期:2012-02-01 00:00:00
abstract::We conducted an open-labeled clinical trial of interferon beta-1b (IFNB) treatment in 20 patients with primary progressive multiple sclerosis (PPMS) and longitudinally monitored autoantibodies against double-stranded DNA (dsDNA), thyroid peroxidase (TPO),myelin basic protein (MBP), myelin oligodendrocyte glycoprotein ...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章
doi:10.1007/s00415-004-0580-3
更新日期:2004-12-01 00:00:00
abstract::A 37-year-old man presented with an acute amnestic syndrome of Korsakoff's type and an upper brain-stem oculomotor syndrome. After a moderate improvement with steroid therapy, he developed progressive behavioural changes due to a frontal lobe syndrome, in addition to motor and visual impairment. Memory performance was...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00315338
更新日期:1991-09-01 00:00:00
abstract::A 57-year-old male was repeatedly admitted to hospital because of complex neurological symptoms, including radicular pain, disturbance of micturition, seizures, and severely impaired mental state. The diagnosis was encephalomyeloradiculitis possibly of viral origin, and treatment with immunosuppressants was initiated....
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314303
更新日期:1988-01-01 00:00:00
abstract::Dopamine agonists have been proved safe and effective as initial therapy in early stages of Parkinson's disease. Prospective long-term clinical trials demonstrated that initial symptomatic treatment with a dopamine agonist is associated with a significantly reduced risk for motor complications in comparison with levod...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-004-1605-7
更新日期:2004-09-01 00:00:00
abstract::Two sexually active female patients presented with acute meningitis. The CSF abnormalities were severe and persistent. In spite of the absence of genital lesions, serological studies revealed a primary infection by herpes simplex virus type 2. An immunoblot study revealed intrathecal synthesis of anti-herpes antibodie...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314743
更新日期:1990-08-01 00:00:00
abstract:BACKGROUND:Dysarthria may be classified as flaccid, spastic, ataxic, hypokinetic, choreatic, dystonic, or mixed. We hypothesized that in routine neurological practice the reliability and accuracy of perceptual analysis alone in the classification of dysarthria is low and that this classification is mainly based on the ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-008-0978-4
更新日期:2008-10-01 00:00:00
abstract::Tibial muscular dystrophy (TMD) or Udd myopathy is an autosomal dominant distal myopathy with late onset, at first described in the Finnish population. We report here the first Italian cases of TTN mutated titinopathy. The proband, a 60 year-old female, had the first muscular signs at the age of 59 years, with difficu...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-009-5372-3
更新日期:2010-04-01 00:00:00
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journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0491-6
更新日期:2007-08-01 00:00:00
abstract::Eighty patients were studied by angiography within 6 h of an ischaemic stroke. Angiography was carried out with digital equipment and was usually limited to the vascular territory responsible for the neurological deficits. In 12 of 19 patients with internal carotid occlusion, the contralateral side was also studied. T...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00314376
更新日期:1989-09-01 00:00:00
abstract::A boy was suspected of having meningoencephalitis probably of viral origin, although all microbiological, including virological, tests were negative. Postmortem examination revealed a dissecting aortic aneurysm and disseminated intravascular coagulation involving the central nervous system exclusively. The clinical ev...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/BF00313465
更新日期:1981-01-01 00:00:00
abstract::Whereas the effect of interferons (IFNs) on magnetic resonance imaging (MRI) outcome measures in patients with multiple sclerosis (MS) has been convincingly shown, little work has been done to define the between-patient heterogeneity of treatment response. Our aim was to assess the distribution of the effect of IFNbet...
journal_title:Journal of neurology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1007/s00415-005-0885-x
更新日期:2005-12-01 00:00:00
abstract:BACKGROUND:Adequate cerebral blood flow (CBF) is mainly governed by neurovascular coupling (NC) which adapts local CBF to underlying cortical activity,and cerebral autoregulation (CA)that tends to maintain constant CBF despite changes in arterial blood pressure (BP). Since it was suggested that resistance vessels play ...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-006-0338-1
更新日期:2007-02-01 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:OBJECTIVE:The aim of this study was to investigate the relationship between type II diabetes mellitus (T2DM) and ALS incidence using the National Health Insurance Research Database and Serious Disabling Disease database of Taiwan. METHODS:This was a population-based cohort study. The index date was the date of the fir...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-019-09405-x
更新日期:2019-09-01 00:00:00
abstract::Few regional and seasonal Guillain-Barré syndrome (GBS) clusters have been reported so far. It is unknown whether patients suffering from sporadic GBS differ from GBS clusters with respect to clinical and paraclinical parameters, HLA association and antibody response to glycosphingolipids and Campylobacter jejuni (Cj)...
journal_title:Journal of neurology
pub_type: 杂志文章
doi:10.1007/s00415-016-8237-6
更新日期:2016-10-01 00:00:00
abstract:BACKGROUND AND PURPOSE:The efficacy and safety of mechanical thrombectomy (MT) for M2 segment occlusion of middle cerebral artery in patients with acute ischemic stroke (AIS) was investigated. METHODS:We searched PubMed and EMBASE from inception to 16 April 2019 for relevant studies, calculated the pool relative risks...
journal_title:Journal of neurology
pub_type: 杂志文章,评审
doi:10.1007/s00415-020-09710-w
更新日期:2020-01-22 00:00:00