Electromyographic and computed tomographic findings in five patients with monomelic spinal muscular atrophy.

Abstract:

:Five patients with monomelic spinal muscular atrophy are described. Clinical features included insidious onset of wasting and weakness of one limb, lack of involvement of the cranial nerves, brain stem, pyramidal tracts and sensory system, and a stable condition over a period of 4-20 years. Clinical findings, electromyography and/or muscle biopsy were consistent with anterior horn cell lesion. Central cavities were excluded by magnetic resonance imaging studies of the spinal cord. Computed tomography of skeletal musculature and electromyography indicated more diffuse lower motor neuron involvement by revealing abnormalities in clinically unaffected muscles in 4 of the 5 patients. Myokymic discharges were found in the affected limb of 1 patient.

journal_name

Eur Neurol

journal_title

European neurology

authors

de Visser M,Ongerboer de Visser BW,Verbeeten B Jr

doi

10.1159/000116249

subject

Has Abstract

pub_date

1988-01-01 00:00:00

pages

135-8

issue

3

eissn

0014-3022

issn

1421-9913

journal_volume

28

pub_type

杂志文章
  • Inhibition of the tyrosine hydroxylase system by MPTP, 1-methyl-4-phenylpyridinium ion (MPP+) and the structurally related compounds in vitro and in vivo.

    abstract::MPTP, 1-methyl-4-phenyl-pyridinium ion (MPP+) and the structurally related compounds including N-methyltetrahydroisoquinilinium ion inhibited the tyrosine hydroxylase (TH) system in tissue slices of rat striatum at 10(-5) M. Mouse striatum is more susceptible to MPTP than rat striatum. A similar degree of inhibition b...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000116350

    authors: Nagatsu T,Hirata Y

    更新日期:1987-01-01 00:00:00

  • Dilemma in the treatment of Parkinson's disease with L-dopa.

    abstract::L-Dopa pioneered the symptomatic therapy of Parkinson's disease. While this treatment proved effective in the treatment of parkinsonian akinesia, rigidity and tremor, prolonged L-dopa treatment was often noted to result in dyskinesia, psychosis and 'on-off' phenomena. This increasing disability of L-Dopa-treated parki...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000119537

    authors: Kuno S

    更新日期:1994-01-01 00:00:00

  • Hypertrophy of the inferior olivary nucleus in patients with progressive supranuclear palsy.

    abstract::Hypertrophic changes in the inferior olivary nuclei have been occasionally described in patients with progressive supranuclear palsy (PSP). To elucidate the incidence of olivary hypertrophy, we investigated morphologically the olives and their associated pathways in 20 autopsied cases: 11 cases of PSP, 3 cases of Mach...

    journal_title:European neurology

    pub_type: 临床试验,杂志文章

    doi:10.1159/000007915

    authors: Hanihara T,Amano N,Takahashi T,Itoh Y,Yagishita S

    更新日期:1998-01-01 00:00:00

  • Effects of nicorandil in neuroprotective activation of PI3K/AKT pathways in a cellular model of Alzheimer's disease.

    abstract::Nicorandil, an ATP-sensitive potassium (KATP) channel opener, is known to have protective effects on ischemic injury in heart and brain. One of the most important protective mechanisms is the anti-apoptotic effect on cardiomyocytes and neurons. This study explored the anti-apoptotic effect of nicorandil against neurot...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000351247

    authors: Kong J,Ren G,Jia N,Wang Y,Zhang H,Zhang W,Chen B,Cao Y

    更新日期:2013-01-01 00:00:00

  • Fisher's syndrome following trigeminal herpes zoster.

    abstract::A case of Fisher's syndrome associated with trigeminal herpes zoster is reported for the first time. Retrograde propagation of the virus to the brainstem through the trigeminal root was thought to be the most probable pathogenic mechanism. We provide additional evidence suggesting that the focus of Fisher's syndrome i...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000115817

    authors: Uematsu D,Satoh T,Tanahashi N,Koto A,Gotoh F

    更新日期:1985-01-01 00:00:00

  • Left tuberothalamic artery territory infarction: neuropsychological and MRI features.

    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

    authors: Lisovoski F,Koskas P,Dubard T,Dessarts I,Dehen H,Cambier J

    更新日期:1993-01-01 00:00:00

  • Dopamine, noradrenaline and serotonin spinal fluid metabolites in temporal lobe epileptic patients with schizophrenic symptomatology.

    abstract::Cerebrospinal fluid metabolites of dopamine, norepinephrine and serotonin were studied in two groups of left temporal lobe epileptic patients, the only difference between the two groups being that one group had paranoid schizophrenic symptomatology. Homovanillic acid accumulation was decreased in this group. This find...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000115048

    authors: Peters JG

    更新日期:1979-01-01 00:00:00

  • Indications for surgical treatment of cerebrovascular insufficiency.

    abstract::197 cases of patients with CVI, subjected to surgery by carotid endarterectomy, are examined. The results were nearly always good and hence the technique is recommended. Particular interest is attached to the risk connected with carotid clamping, through intraoperative study of CBF with 133Xe, such a risk is practical...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000115010

    authors: Fiorani P,Pistolese GR,Faraglia V,Spartera C

    更新日期:1978-01-01 00:00:00

  • Autosomal dominant familial Parkinson disease: older onset of age, and good response to levodopa therapy.

    abstract::Autosomal dominant Parkinson's disease in 5 generations of a family living in Sagamihara City is reported. Clinical features of this family did not differ from common PD, however, neuropathological findings were different from PD. In autopsied cases, the loss of melanin-containing cells was mild to moderate, and the n...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000113460

    authors: Hasegawa K,Kowa H

    更新日期:1997-01-01 00:00:00

  • Paralysis of upward gaze as a presenting symptom of vitamin B12 deficiency.

    abstract::A 47-year-old woman presented with paralysis of upward gaze associated with Addison's pernicious anaemia. Administration of cyanocobalamin resulted in significant improvement of eye movements and haematological parameters. Isolated paralysis of upward gaze may be a feature of vitamin B12 deficiency. ...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000115702

    authors: Sandyk R

    更新日期:1984-01-01 00:00:00

  • Migraine and Headache Prevalence and Associated Comorbidities in a Large Saudi Sample.

    abstract:BACKGROUND:We aimed to assess the prevalence of headache and migraine, along with comorbidities, in a large -Saudi sample. METHODS:Self-reported information was collected about headache, migraine and migraine comorbidities including depression, restless legs syndrome (RLS), syncope, bruxism, hypertension and ischaemic...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000487317

    authors: Muayqil T,Al-Jafen BN,Al-Saaran Z,Al-Shammari M,Alkthiry A,Muhammad WS,Murshid R,Alanazy MH

    更新日期:2018-01-01 00:00:00

  • Pure eye muscle involvement in endocrine orbitopathy.

    abstract::We present a long-term follow-up examination concerning patients with isolated extra-ocular muscle involvement in thyroid-related orbitopathy. Within the previous 13 years we observed 7 patients with endocrine orbitopathy and marked myopathy of the extra-ocular muscles. Five of these patients had no detectable proptos...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000131894

    authors: Gerlach M,Ferbert A

    更新日期:2008-01-01 00:00:00

  • Malpighi and the discovery of capillaries.

    abstract::Leonardo da Vinci clearly observed and described capillaries. Using the microscope, Marcello Malpighi examined the brain and major organs to demonstrate their finer anatomical features. This led to his discovery in 1661, of capillaries that proved fundamental to our understanding of the vascular system in the brain an...

    journal_title:European neurology

    pub_type: 传,历史文章,杂志文章

    doi:10.1159/000107974

    authors: Pearce JM

    更新日期:2007-01-01 00:00:00

  • European Association of Young Neurologists and Trainees: position paper on teaching courses for Generation Y.

    abstract::The European Association of Young Neurologists and Trainees (EAYNT) is a non-profit organization which acts on behalf of young neurologists in Europe and concertedly exerts influence on the formation of a new generation of neurologists [Struhal et al.: Eur J Neurol 2009;16:e146-e148]. This concerns particularly the Ge...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000327696

    authors: Struhal W,Falup-Pecurariu C,Sztriha LK,Grisold W,Sellner J

    更新日期:2011-01-01 00:00:00

  • Statins provoking MELAS syndrome. A case report.

    abstract:BACKGROUND:Statins inhibit the production of 2,3-dimethoxy,5-methyl,6-polyisoprene parabenzoquinone also known as ubiquinone or coenzyme Q10 (CoQ10), which is required for mitochondrial electron transport. Idiopathic or primary CoQ10 deficiencies have been known to cause mitochondrial encephalomyopathy. METHODS:We pre...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000101287

    authors: Thomas JE,Lee N,Thompson PD

    更新日期:2007-01-01 00:00:00

  • Current clinical spectrum of neurosyphilis in immunocompetent patients.

    abstract:BACKGROUND:Neurosyphilis (NS) is nowadays a less frequent disease. Its incidence and clinical spectrum have changed over time. OBJECTIVE:To estimate the incidence of NS and describe the clinical spectrum of NS in immunocompetent patients in the last decade. METHODS:Demographic and clinical features, cerebrospinal flu...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000079391

    authors: Conde-Sendín MA,Amela-Peris R,Aladro-Benito Y,Maroto AA

    更新日期:2004-01-01 00:00:00

  • The myxomatous mitral valve. A risk factor for ischemic stroke in young patients?

    abstract::3 out of 24 patients with mitral valve prolapse (MVP), mean age 25.7 +/- 3.1 displayed markedly thickened redundant and shaggy mitral leaflets in the two-dimensional echocardiogram. The anterior leaflet appeared in the parasternal long axis plane club-shaped in diastole. These three female patients had a history of is...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000115766

    authors: Zenker G,Boné G,Ladurner G,Lechner H

    更新日期:1985-01-01 00:00:00

  • Reports of patients and relatives from the CogniCIS study about cognition in clinically isolated syndrome: what are our patients telling us?

    abstract:OBJECTIVE:To assess the Multiple Sclerosis Neuropsychological Questionnaire (MSNQ) in patients with clinically isolated syndrome (CIS). METHODS:130 European CIS patients and 60 relatives completed the MSNQ. RESULTS:The mean (SD) MSNQ score for CIS patients was 15.5 (10.8) and for their informants 11.3 (9.6). Neither ...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000345698

    authors: Langdon DW,Benedict RH,Wicklein EM,Beckmann K,Fredrikson S

    更新日期:2013-01-01 00:00:00

  • Neurofibrillary tangles and Alzheimer's disease.

    abstract::The neuropathological diagnosis of Alzheimer's disease relies on the presence of both neurofibrillary tangles and senile plaques. The number of neurofibrillary tangles is tightly linked to the degree of dementia, suggesting that the formation of neurofibrillary tangles more directly correlates with neuronal dysfunctio...

    journal_title:European neurology

    pub_type: 杂志文章,评审

    doi:10.1159/000007969

    authors: Brion JP

    更新日期:1998-10-01 00:00:00

  • The side and somatotopical location of single small infarcts in the corona radiata and pontine base in relation to contralateral limb paresis and dysarthria.

    abstract::The aim of this study was to investigate whether the side and location of single small infarcts (< or = 3 cm) in the corona radiata (28 patients) and pontine base (36 patients) influence the incidence of contralateral upper or lower limb paresis and dysarthria. While the severity of contralateral limb paresis was not ...

    journal_title:European neurology

    pub_type: 临床试验,杂志文章

    doi:10.1159/000117290

    authors: Tohgi H,Takahashi S,Takahashi H,Tamura K,Yonezawa H

    更新日期:1996-01-01 00:00:00

  • Medical therapy of cerebral ischemia. Vasoactive and eumetabolic therapy.

    abstract::After briefly evaluating the contraindications and indications of vasoactive and eumetabolic therapy of cerebral ischemia, the paper considers the main drugs at present in use in therapy of the syndrome induced by diffused cerebral arteriosclerosis. Among the drugs with a mainly vasoactive effect, consideration is giv...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000115018

    authors: Strano A,Pinto A,Davì G,Novo S,Riolo F

    更新日期:1978-01-01 00:00:00

  • T lymphocytes in cerebrospinal fluid from patients with neurological diseases.

    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

    authors: Naess A

    更新日期:1979-01-01 00:00:00

  • Chronic partial denervation is more widespread than is suspected clinically in paralytic poliomyelitis. Electrophysiological study.

    abstract::Clinical evaluation, quantitative analysis of the EMG, and motor unit fiber density were carried out on 34 selected patients that suffered paralytic poliomyelitis. 50% of the subjects developed a late and slowly progressive weakness. Automatic analysis of the electromyogram showed a great increase in mean amplitude in...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000115578

    authors: Cruz Martínez A,Pérez Conde MC,Ferrer MT

    更新日期:1983-01-01 00:00:00

  • Synaesthesia.

    abstract::Synaesthesia is the intriguing, involuntary experience of feeling one sensation in response to a different sensory stimulus. Recognised since described in 1890 by John Locke and clarified by Galton in the 1880s, it has been analysed in the last 50 years. Grapheme-colour synaesthesia is the commonest form, but many oth...

    journal_title:European neurology

    pub_type: 历史文章,杂志文章,评审

    doi:10.1159/000098101

    authors: Pearce JM

    更新日期:2007-01-01 00:00:00

  • Ineffectiveness of folic acid supplementation against phenytoin-induced decrease in salivary immunoglobulin A concentration of epileptic patients.

    abstract:AIMS:This study was designed to investigate if folate treatment is able to reverse the phenytoin-induced deficiency of salivary immunoglobulin A (IgA). METHODS AND MATERIAL:Twenty-five epileptic patients who had been under phenytoin therapy for at least the last 6 months were randomly selected and subjected to folic a...

    journal_title:European neurology

    pub_type: 杂志文章,随机对照试验

    doi:10.1159/000121419

    authors: Zare M,Ghazvini MR,Yazdi HR,Nezhad VS,Chitsaz A

    更新日期:2008-01-01 00:00:00

  • Effects of Metabolic Syndrome on Cognitive Impairment with White Matter Lesions.

    abstract:OBJECTIVE:To explore the relationship between metabolic syndrome (MetS) and cognitive impairment in elderly patients with white matter lesions (WML). METHODS:From -January 2016 to June 2017, a cross-sectional study was conducted on the clinical data of 358 WML patients over 65 years old in the Department of Neurology ...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000505242

    authors: Chen L,Hong W,Yang H,Dong S,Peng Z,Zhou H

    更新日期:2019-01-01 00:00:00

  • Multiphasic perfusion computed tomography as a predictor of collateral flow in acute ischemic stroke: comparison with digital subtraction angiography.

    abstract:BACKGROUND:Assessing collateral status is important in acute ischemic stroke. The purpose of this study was to compare multiphasic perfusion computed tomography (MPCT) with digital subtraction angiography (DSA) in predicting leptomeningeal collateral flow in acute middle cerebral artery (MCA) infarction. METHODS:Conse...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000334867

    authors: Kim SJ,Noh HJ,Yoon CW,Kim KH,Jeon P,Bang OY,Kim GM,Chung CS,Lee KH

    更新日期:2012-01-01 00:00:00

  • Motor fluctuations in levodopa treatment: clinical pharmacology.

    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

    authors: Stocchi F,Bonamartini A,Vacca L,Ruggieri S

    更新日期:1996-01-01 00:00:00

  • Effectiveness of low-frequency rTMS and intensive speech therapy in poststroke patients with aphasia: a pilot study based on evaluation by fMRI in relation to type of aphasia.

    abstract:AIM:To assess the safety and clinical efficacy of low-frequency repetitive transcranial magnetic stimulation (LF-rTMS) combined with intensive speech therapy (ST) in poststroke patients with aphasia. SUBJECTS AND METHODS:Twenty-four patients with left-hemispheric stroke and aphasia were subjected. During 11-day hospit...

    journal_title:European neurology

    pub_type: 临床试验,杂志文章

    doi:10.1159/000338773

    authors: Abo M,Kakuda W,Watanabe M,Morooka A,Kawakami K,Senoo A

    更新日期:2012-01-01 00:00:00

  • Thermal thresholds and motor sensory conduction measurements in Guillain Barré syndrome: 12-month follow-up study.

    abstract::We performed serial neurophysiological tests in 22 patients with confirmed Guillain Barré syndrome (GBS) for 12 months following onset of the disease. Twenty-five age- and sex-matched healthy controls were also tested. Tests included nerve conduction and automated quantitative thermal threshold measurements. The commo...

    journal_title:European neurology

    pub_type: 杂志文章

    doi:10.1159/000116841

    authors: Thomaides TN,Kerezoudi EP,Zoukos Y,Chaudhuri KR

    更新日期:1992-01-01 00:00:00