ISSN:0014-3022    

DOI:10.1159/000116968

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

A family study in 29 patients with idiopathic adult-onset blepharospasm (n = 16) and cranial-cervical dystonia (n = 13) was undertaken by examining 189 first-degree relatives. Six relatives with dystonia were identified in 6 families. A further 3 affected relatives, now deceased, were from 2 other families. All the secondary cases were parents or siblings. There was a tendency for affected relatives to have the same type of dystonia of index patients. To assess the significance of secondary cases we compare the incidence of affected siblings between probands and their spouses. Dystonia was found in 5 out of 120 proband siblings whereas none of the 142 spouse siblings was affected (p < 0.05). Segregation analysis suggested an autosomal-dominant transmission and reduced penetrance or, alternatively, polygenic inheritance.

ISSN:0014-3022    

DOI:10.1159/000116969

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Fifteen patients with Friedreich’s ataxia (FA) and 15 others with olivopontocerebellar atrophy (OPCA) were evaluated with a comprehensive neuropsychological battery of tests. They were pair-matched with normal controls for age, sex and education. Depressed patients were excluded from the study as were those with extrapyramidal signs. The following results were obtained: (1) the Raven test, untimed block design performance in OPCA and quantitative analysis of Rey figure drawing revealed a visuospatial deficit suggestive of a mild parietal-like syndrome; signs of mild frontal-like syndrome were also found; (2) cognition was slowed in the FA group; (3) simple visual and auditory reaction times were increased in both patient groups. It appears that the cerebellum may interfere indirectly with cognition through various physiological and neurochemical 2-way cerebellocortical loops. Finally, the cerebellum seems to interfere directly also with basic speed of information processin

ISSN:0014-3022    

DOI:10.1159/000116970

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

In order to examine the plasminogen activator (PA) induction involved in the pathogenesis of acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS), PA activity in peripheral blood lymphocytes derived from 5 ADEM and 3 MS patients was investigated. There was no PA induction in any ADEM, MS or control lymphocytes treated with N-methyl-N’-nitro-N-nitro-soguanidine (MNNG) alone. PA activity, however, in lymphocytes exposed to human interferon-y (HulFN-y) prior to MNNG treatment was elevated during the active phase of ADEM and MS, whereas the PA induction disappeared in association with improvement of the neurologic symptoms. The PA activity was abolished by mixed treatment with HuIFN-y and anti-HulFN-y antibody. No such PA induction by any HulFN was observed in any normal controls or cases of other neurologic diseases. Among the cytokines tested other than HulFN, tumor necrosis factor-a, in combination with MNNG, also induced PA activity in lymphocytes from ADEM and MS patients during the active phase. Thus, the PA induction observed in lymphocytes on combined treatment with MNNG and cytokines may be involved in the progression of neurologic disorders in these demyelinating diseases, and indicates the possibility of therapeutic strategies involving anticytokine usag

ISSN:0014-3022    

DOI:10.1159/000116971

出版商:S. Karger AG    

年代:1993

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000203103

出版商:S. Karger AG    

年代:1913

数据来源: Karger

摘要:

We performed a quantitative sudomotor function test on 10 patients with early Parkinson’s disease (PD) and 11 age-matched control subjects. Thermal warming increased the sweat rate in both PD and controls. There was no difference in sweat rate between the forearm and the thigh in either PD or controls. In PD, thyrotropin-releasing hormone (TRH) did not increase the sweat rate, whereas it did so in the controls. These results suggest that sudomotor dysfunction in early PD is minor but that there may be an impairment of TRH-induced sympathetic response in the early stages of P

ISSN:0014-3022    

DOI:10.1159/000116972

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Quantitative assessment of regional cerebral blood flow (rCBF) in the bilateral cerebral and cerebellar cortices was performed in 15 epileptic adult patients receiving chronic high-dose antiepileptic drug therapy and 22 normal volunteers matched for sex and age, using single photon emission computed tomography with N-isopropyl-(iodine-123)-p-iodoamphetamine. The entire averaged rCBF value in the epileptic patients, i.e. 52.8 ± 13.7 ml/100 g/min (range: 25-78 ml/100 g/min), was significantly lower as compared with that in the normal subjects, i.e. 69.1 ± 14.2 ml/100 g/min (range: 46-102 ml/100 g/min). Six of the 15 patients showed absolute rCBF values less than the minimum of the normal range, i.e. 46 ml/100 g/min, in all or most of the measured brain tissues. There was a significant correlation between the diffuse cerebral hypoperfusion and simultaneous ingestion of phenytoin and phenobarbital. The possible effects of antiepileptic drugs on rCBF are discusse

ISSN:0014-3022    

DOI:10.1159/000116973

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

We studied auditory event-related potentials (ERPs) in 17 patients with systemic lupus erythematosus (SLE). Latencies of N100 and P200 components were normal in all patients, whereas the latencies of target P300 and nontarget P300 components were significantly prolonged in SLE patients with and without cognitive dysfunction. The P300 component of ERPs can be applied to evaluate the cognitive aspects of central nervous system manifestations in SLE patients.

ISSN:0014-3022    

DOI:10.1159/000116974

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

The case of a 30-year-old man with posttraumatic syringomyelia involving the cervical and upper thoracic spine is reported. The patient was followed clinically and with magnetic resonance (MR) imaging over a 3-year period. Spontaneous complete remission of the syrinx-related symptoms correlated with a partial collapse of the syrinx, as proved by serial MR imaging.

ISSN:0014-3022    

DOI:10.1159/000116975

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Vertebrobasilar dolichoectasia is a relatively uncommon pathological entity and generally asymptomatic. We report a quite unusual case of unilateral motor trigeminal involvement with trismus, due to VD. Apart from tetanus or local morbid conditions of the mouth, trismus is often attributed to disturbed programming and co-ordination of the masticatory muscles within the mesencephalic nucleus. The possibility of truncal compression of the trigeminal motor root by vertebrobasilar dolichoectasia being responsible for masseter muscle spasm, in analogy with the pathogenetic mechanisms proposed in hemifacial spasm, is proposed as an alternative explanation in the present case.

ISSN:0014-3022    

DOI:10.1159/000116976

出版商:S. Karger AG    

年代:1993

数据来源: Karger