摘要:

Neurotrophic factors which act on motor neurons may provide an efficient treatment for amyotrophic lateral sclerosis. In order to overcome the difficulty of administering proteins into the central nervous system, it is possible to use a gene therapy approach, i.e. the polymer encapsulation of cells which have been genetically engineered to release one of these neurotrophic factors. The polymer-encapsulated cells can be implanted subcutaneously or even intrathecally and permit continuous, slow release of proteins. There is no rejection of the cells due to their isolation by a semi-permeable membrane, no risk of tumor formation and the polymer device can be removed in the event of a problem.

ISSN:0014-3022    

DOI:10.1159/000117095

出版商:S. Karger AG    

年代:1995

数据来源: Karger

摘要:

Here we review a total of 17 patients, including 4 of our own, with young-adult-onset arteriosclerotic leukoencephalopathy, alopecia and lumbago without hypertension. All were Japanese and the illness seemed to be transmitted in an autosomal recessive fashion. The male to female ratio was 7.5:1. The age of onset usually ranged from 25 to 30 years. Acute lumbago, spondylosis deformans, diffuse baldness, and progressive motor and mental deterioration were common clinical features. Cerebral arteriosclerosis and white mater disease were demonstrated pathologically or radiologically. Although the pathogenesis has remained obscure, we think this is a new clinico-pathological entity distinct from the classic type of Binswanger’s diseas

ISSN:0014-3022    

DOI:10.1159/000117096

出版商:S. Karger AG    

年代:1995

数据来源: Karger

摘要:

A study was performed in order to investigate how infarcts located in the white matter of the right hemisphere may affect processing of spatial and perceptual abilities. A battery of neuropsychological tests was applied to 7 right-handed patients with CT-verified infarcts of the right internal capsule or periventricular white matter. Examinations were performed 1-3 weeks post stroke and 3-7 months later. The patients were assessed by neurological examinations, EEG, and cortical regional cerebral blood flow (rCBF) measurements. – This study suggested that deep white matter infarcts of the right hemisphere may result in persistent spatial impairment in the absence of decreased cortical rCBF. Perception was disturbed in cases of low mean cortical CBF of both hemisphere

ISSN:0014-3022    

DOI:10.1159/000117097

出版商:S. Karger AG    

年代:1995

数据来源: Karger

摘要:

Using multivariate analysis, we investigated the influence on cognitive functions of aging, brain atrophy, incidental cerebral hyperintensities (CHs), medication, and severity, duration and the inital symptoms of the disease in 53 patients with idiopathic Parkinson’s disease (PD). We semiquantitatively assessed the degree of brain atrophy and CHs based on previously established methods. Cognitive functions were significantly and diffusely impaired in PD when compared with controls who were matched for age as well as for the degree and location of CHs. Patients with PD, however, had larger ventricles. Prefrontal dysfunctions were associated with a variety of predictors such as CHs, brain atrophy, severity of PD and medication whereas dorsolateral frontal functions were related simply to age and CHs in the periventricular region. Posterior brain functions had association with severity of illness, ventricular dilatation and total CH score. Clinically observed cognitive impairments in PD may consist of cognitive defects intrinsic to the disease which are variously modified by these factors. It is essential to consider all these predictors simultaneously in any discussion of cognitive functions in P

ISSN:0014-3022    

DOI:10.1159/000117098

出版商:S. Karger AG    

年代:1995

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000117099

出版商:S. Karger AG    

年代:1995

数据来源: Karger

摘要:

We examined the characteristics of specific components of the evoked electrospinogram (EESG) in response to tibial nerve stimulation in 28 patients with traumatic injury of the dorsal and cervical spinal cord. The data were correlated with clinical, urodynamic and additional neurophysiological findings. In the majority of patients (82%), 11 with complete and 12 with partial spinal cord lesions, the lumbar components of the EESG were normal. In 4 of these patients with complete lesion above T2 the dorsal EESG was absent. In 5 cases (18%), the lumbosacral EESG was altered in the presence of an atypical clinical syndrome characterized by persistent urinary retention associated with lower leg atrophy and reduced tendon jerks. In these cases, MRI provided evidence of an unexpected sacral lesion. Unlike the dorsal EESG reflecting the afferent dorsal column volley, the lumbar cofnponents of the EESG are usually unaffected by interruption of the cord pathways; these last events thus originate from segmental neuronal activity, the involvement of which bears witness to additional lumbosacral damage.

ISSN:0014-3022    

DOI:10.1159/000117100

出版商:S. Karger AG    

年代:1995

数据来源: Karger

摘要:

Exophthalmos and thyrotropin-inhibitory immunoglobulin (TBII) were examined in myasthenia gravis (MG) patients with normal thyroid function. Exophthalmos was found in 7 of 31 (22.6%) MG patients, and TBII was detected in 4 of 21 (19.0%) MG patients. The grades of exophthalmos did not decrease after extraocular muscle weakness was improved by treatment. Patients with MG whose diplopia was Tensilon unresponsive were only found among those with exophthalmos. TBII was more frequently detected in MG patients with exophthalmos than in those without. The present study revealed that exophthalmos is not rare in MG patients, even if they do not show any signs of Graves’ disease other than exophthalmos and their thyroid functions are within the normal limit. The presence of TBII in MG patients may relate to the development of exophthalmos, and exophthalmos itself might enhance ocular symptoms of M

ISSN:0014-3022    

DOI:10.1159/000117101

出版商:S. Karger AG    

年代:1995

数据来源: Karger

摘要:

In a study to determine the prevalence of monoclonal gammopathy (MG) among patients with motor neuron disease (MND), 6 out of 56 (10.7%) were found to have a monoclonal paraprotein. Of these 6 patients, 4 had an IgG and 2 had an IgA paraprotein. The clinical syndromes consisted of amyotrophic lateral sclerosis in 2 patients, lower motor neuron syndrome with preserved reflexes in at least one limb in 3 patients, and motor neuropathy with multifocal conduction block in 1 patient. The presence of gammopathy appears to correlate with the absence of marked upper motor neuron involvement and with elevated CSF protein concentration. An underlying malignant disorder was ruled out in all 6 patients, and they were considered to have MG of undetermined significance (MGUS). In a control group of 121 age-matched patients with other neuroimmunological disorders, 5 patients (4.13%) had MG. Four of these had gammopathy associated with malignant myeloma, and 1 had MGUS. These results support previous reports of increased prevalence of MGUS in patients with MND and suggest that an autoimmune mechanism may play a role in the disease.

ISSN:0014-3022    

DOI:10.1159/000117102

出版商:S. Karger AG    

年代:1995

数据来源: Karger

摘要:

Damage to the vascular system, of which endothelial cells are the main constituent, may occur in sarcoidosis. Evidence of blood-brain barrier (BBB) disruption is shown in sarcoidosis with central nervous system (CNS) involvement by means of magnetic resonance imaging or computed tomography. We investigated the presence of antiendothelial cell antibodies by culture of endothelial cells derived from human umbilical cord vein and from human brain using an enzyme-linked immunosorbent assay, and the presence of circulating immune complexes using a Raji cell assay, in the sera of patients with sarcoidosis. The patients with sarcoidosis displayed significantly high levels of immunoglobulin G (IgG) binding to endothelial cells after blocking Fc receptor compared to controls (p < 0.01). The sera of neurosarcoid patients with CNS involvement still showed significantly increased levels of IgG binding to brain endothelial cells after blocking Fc receptor compared to controls and those of pulmonary sarcoidosis (p < 0.001 and p < 0.01 respectively). These results suggest that the presence of autoantibodies to endothelial cells may be involved in endothelial cell damage, including BBB disruption.

ISSN:0014-3022    

DOI:10.1159/000117103

出版商:S. Karger AG    

年代:1995

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000203039

出版商:S. Karger AG    

年代:1914

数据来源: Karger