Abstract:
:Mitochondrial abnormalities and effectiveness of replacement therapy were examined in a murine model of systemic carnitine deficiency, namely the juvenile visceral steatosis (JVS) mouse. Homozygous JVS mice revealed severe lipid deposition and abnormal mitochondria in liver, heart, skeletal muscle, and kidney, but there was no pathological change in the nervous system, though they showed cerebral signs. There were numerous ragged-red fibers in muscles, but enzyme activities of the respiratory chain were intact. Histograms of oxidative and nonoxidative muscle fibers showed an increase in small and oxidative muscle fibers in 4-week-old JVS mice, but this difference no longer existed in 8-week- or 1-year-old JVS mice. On the contrary, Mn-superoxide dismutase immunostaining of muscle showed a focal increase in every age of JVS mice. With L-carnitine treatment, JVS mice could survive for a year, but to some extent, there were the same pathological changes as those seen in untreated mice.
journal_name
Eur Neuroljournal_title
European neurologyauthors
Kaido M,Fujimura H,Ono A,Toyooka K,Yoshikawa H,Nishimura T,Ozaki K,Narama I,Kuwajima Mdoi
10.1159/000113399subject
Has Abstractpub_date
1997-01-01 00:00:00pages
302-9issue
4eissn
0014-3022issn
1421-9913journal_volume
38pub_type
杂志文章abstract::In order to quantitatively assess the motor performance characteristics of 14 patients with congenital myotonia, the reaction time, speed of movement, synergy of different muscle groups and accuracy were measured. The Human Performance Measurement/Basic Elements of Performance device was used for recordings. Warned si...
journal_title:European neurology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1159/000117129
更新日期:1995-01-01 00:00:00
abstract::This study assessed gender differences in diabetic patients with ischemic stroke with respect to vascular risk factors, clinical features and early outcome. Ischemic stroke was diagnosed in 261 diabetic women and 300 diabetic men of 2,446 ischemic stroke patients included in a prospective stroke registry over 17 years...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000094249
更新日期:2006-01-01 00:00:00
abstract::Morphological findings of 3 cases of Kaeser's scapulo-peroneal muscular atrophy are described. 1 of them is the first complete autopsy report on this disease. While the number of motor neurons in the spinal cord was grossly normal, the neuropil of the anterior horn exhibited axonal swellings, accumulations of possibly...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114899
更新日期:1977-01-01 00:00:00
abstract::Transient ischemic attacks (TIAs) are warning episodes predicting that such patients are at high risk for stroke which potentially could be life-threatening or leave an individual with substantial disability. TIAs result from large or small vessel disease, cardiogenic embolic events and hematological abnormalities. Th...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000052065
更新日期:1998-01-01 00:00:00
abstract::Electrophysiological findings in 10 patients with polyneuropathy and nonmalignant IgMk plasma cell dyscrasia are reported. The amplitude of sensory action potentials and maximum conduction velocity along the sural and median nerves are related to the structural changes found at the biopsy and to the immunologic result...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115979
更新日期:1986-01-01 00:00:00
abstract:BACKGROUND/AIMS:Albuminuria and carotid atherosclerosis are predictors of cardiovascular disease and potential predictors of cognitive decline. Our aim was to study whether albuminuria was an early predictor of cognitive function independent of carotid atherosclerosis in a general population. METHODS:The study populat...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000353701
更新日期:2013-01-01 00:00:00
abstract::Human slow wave sleep (hSWS) is an important form of sleep that seems to take priority over REM sleep. Although hSWS is commonly thought to be associated with enhanced tissue repair, evidence supporting this view is questioned. For example, total sleep deprivation and loss of hSWS have little or no adverse effect on b...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116076
更新日期:1986-01-01 00:00:00
abstract::Bilateral choroid plexus papillomas, or villous hypertrophy of the choroid plexus, is a very rare entity that presents serious therapeutical problems in the management of secondary oversecretive hydrocephalus. The authors report the cases of 2 infants affected by this condition, and cured with removal of the lateral v...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000117402
更新日期:1997-01-01 00:00:00
abstract:BACKGROUND:Patients with multiple sclerosis (MS) report sleep disturbances (SD) and excessive daytime sleepiness (EDS) more frequently than the general population. Our objectives were to evaluate SD and EDS in MS patients and to test the reliability of subjective sleep questionnaires. METHODS:Demographic and clinical ...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000335076
更新日期:2012-01-01 00:00:00
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, electrom...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116249
更新日期:1988-01-01 00:00:00
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
更新日期:1978-01-01 00:00:00
abstract::The urinary clearance of pyridostigmine was studied in six patients with myasthenia gravis. In three patients on pyridostigmine alone, renal clearance ranged from 349 to 481 ml/min, corresponding to a pyridostigmine:creatinine clearance ratio of 2.64 to 3.46. In a patient on bendrofluazide as well as pyridostigmine, a...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114882
更新日期:1977-01-01 00:00:00
abstract::An 18-year-old mongoloid girl (trisomy 21) of Asian descent presented two symptoms rarely associated with mongolism (Down's syndrome). (1) Brain stem calcification; the first case diagnosed in vivo in mongolism on computerized axial tomography. (2) Hallucinatory psychotic depression; the first case successfully treate...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114963
更新日期:1978-01-01 00:00:00
abstract::Sixteen children with definite multiple sclerosis (MS; aged 6-17 years, mean 11.4 +/- 2.7; 8 boys, 8 girls) were reviewed. Cerebellar symptoms and signs were frequent at initial presentation. Among laboratory studies, the cerebrospinal fluid (CSF) IgG index or oligoclonal bands were informative in 75%, evoked potentia...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000117213
更新日期:1996-01-01 00:00:00
abstract::Serum from patients with multiple sclerosis (MS) and other diseases was added to the medium perfusing isolated hemisected frog spinal cord, and the effect on ventral root responses (VRR) tested. As control, a standardized commercial human serum (Moni-trol I) was used. In 25 out of 40 spinal cord preparations Moni-trol...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114824
更新日期:1977-01-01 00:00:00
abstract:BACKGROUND/AIMS:We assessed the, hitherto unknown, impact of intravenous methylprednisolone (ivMP) pulses during relapses of multiple sclerosis (MS) on the kinetics of anti-interferon-beta neutralizing antibodies (Nabs) and binding antibodies (Babs). METHODS:Babs (ELISA) and Nabs (antiviral cytopathic effect assay) ti...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000444319
更新日期:2016-01-01 00:00:00
abstract:BACKGROUND/AIM:In this case-control study, our aim was to check for a possible independent role of hypertension in increasing the risk of developing Bell's palsy. METHODS:All patients diagnosed as having Bell's palsy referred to a neurology clinic were enrolled as cases. Controls were selected among other patients ref...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000151701
更新日期:2008-01-01 00:00:00
abstract:OBJECTIVES:Cerebral arteriovenous malformations (AVMs) harbor a network of abnormal vasculatures, namely the nidus between arterial and venous components. The pressure gradient between these two components results in abnormal high-velocity arteriovenous shunts flowing through the nidus and alternate intracranial hemody...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000292431
更新日期:2010-01-01 00:00:00
abstract::The role of genetics in Parkinson's disease (PD), previously controversial, is now supported by several studies. A major breakthrough has been the discovery of a single gene defect in familial Parkinson's disease. A single base pair change at position 209 from G to A (G209A) in the fourth exon of the alpha-synuclein g...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000008008
更新日期:1999-01-01 00:00:00
abstract::Familial cases of HTLV-I-associated myelopathy (HAM) and adult T-cell leukemia (ATL), developing in a daughter and father, respectively, are reported. The coexistence of both diseases in a family has not been reported before. This supports the recent findings that ATL and HAM may be brought about by an identical virus...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116373
更新日期:1989-01-01 00:00:00
abstract::To clarify the function of human atrial natriuretic peptide (hANP) in the cerebrospinal fluid (CSF), we examined hANP levels in the CSF of patients with various neurological diseases. The subjects were 16 controls without neurological disease and 45 patients with neurological disease. The 45 patients with neurological...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000113166
更新日期:1997-01-01 00:00:00
abstract::A 9-year-old girl with huge, double arachnoid cysts at the cerebellopontine angle is reported. After a cyst-peritoneal shunt operation, she developed fine, high-frequency gaze nystagmus towards the lesion side combined with coarse, large amplitude nystagmus in the opposite direction, which is the reverse of the Bruns'...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000116903
更新日期:1993-01-01 00:00:00
abstract::In a 5-year follow-up of a case of adult metachromatic leukodystrophy, already diagnosed in the preclinical stage, the development of the symptoms of this disease could be studied in detail: initially, lack of drive, emotional lability and depressive mood. At the same time, pain in the arms and beginning gait disturba...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000114818
更新日期:1977-01-01 00:00:00
abstract::The effect of dosage schedules on plasma drug levels was evaluated in two groups of ambulant epileptic patients, whose treatment with diphenylhydantoin (DPH) or phenobarbital (PB) had been simplified by reducing the daily frequency of the dosage regimens. Although the patients received the same milligram per kilogram ...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115099
更新日期:1979-01-01 00:00:00
abstract::A double-blind controlled trial was performed on 6 patients affected with Steinert's disease in order to evaluate the efficacy of two different dosages of diphenylhydantoin (PHT, 200 and 300 mg/day) and carbamazepine (CBZ, 600 and 800 mg/day) on the myotonic afterdischarge. Both dosages of PHT and CBZ induced a signif...
journal_title:European neurology
pub_type: 临床试验,杂志文章
doi:10.1159/000115546
更新日期:1983-01-01 00:00:00
abstract::70 cases of multiple sclerosis were seen at the Department of Neurology of the Capital Hospital in 1949--1977. There were 25 males and 45 females averaging 30.6 years of age. The most frequent sites of the lesions based on the clinical symptomatology were the spinal cord and optic nerve, the cerebellum was much less f...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115267
更新日期:1981-01-01 00:00:00
abstract::The authors report a case of a giant aneurysm of the distal portion of the posterior inferior cerebellar artery presenting the classical picture of a tumor of the posterior fossa. CT scan of a giant aneurysm reveals the clear margin and high density area of aneurysm wall, and the homogeneous and high density area of t...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115108
更新日期:1979-01-01 00:00:00
abstract::A case of post-convulsive spinal spidural haematoma in a patient with ankylosing spondylitis is presented. As acute tetraplegia developed, surgery was performed with finding of blood clots in the extradural space from C6 to D8. Lethal evolution due to late referral to medical care stresses the need for prompt decompre...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000115081
更新日期:1979-01-01 00:00:00
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
更新日期:1983-01-01 00:00:00
abstract::Fatigue is one of the most frequent and most disabling symptoms in multiple sclerosis (MS). We investigated the possible association of the MS-related fatigue syndrome with the available disease-modifying therapies and the main disease characteristics in a cross-sectional study on 320 consecutive patients. The prevale...
journal_title:European neurology
pub_type: 杂志文章
doi:10.1159/000111876
更新日期:2008-01-01 00:00:00