摘要:

Thirty‐nine patients with hereditary ataxia (HA), hereditary spastic paraplegia or Charcot‐Marie‐Tooth disease were investigated with computerized cranial tomography (CT). Infratentorial as well as supratentorial atrophies were registrated and scored. These were compared with the patient's neurological symptoms, which were related to the lesion in the central nervous system, and scored. There was correlation between the distribution of brainstem/cerebellar symptoms and the distribution and degree of infratentorial atrophy.HA cases with cerebral cortical atrophy had significantly higher dementia scores than those without wide sulci. Intravenous injection with contrast medium was of no diagnostic aid, as no focal changes were found. It is concluded that CT is an aid in the diagnosis of hereditary ataxias and hereditary spastic parap

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02864.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

β‐Glucosidase and N‐acetyl‐β‐glucosaminidase activities were measured with synthetic substrates in peripheral leucocytes, urine and serum from patients with juvenile type of Gaucher's disease.Our findings in urine and serum make it clear that diagnosis by using synthetic substrate is not possible. In peripheral leucocytes reduced level was found for β‐glucosidase activity in patients with Gaucher's disease but also there occurred some overlapping with controls.The possible explanation to these findings a

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02865.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

B and T cells were defined in cerebrospinal fluid (CSF) and blood of 18 multiple sclerosis (MS), five optic neuritis, and 17 mumps meningitis patients by counting immunoglobulin‐bearing lymphocytes for B cells, and the capacity of rosette formation with sheep erythrocytes for T‐cell determination. Patients with MS and mumps meningitis had significantly higher T‐cell values in CSF compared to blood, while the B cell value was significantly lower in CSF in mumps meningitis only. MS patients also displayed significantly lower B‐cell values in blood compared to mumps meningitis patients and blood donors. No significant differences were observed between MS patients in exacerbation and in re

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02866.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Carbamazepine (CBZ), carbamazepine‐10,11‐epoxide (CBZ‐epoxide) and phenytoin (DPH) were measured in brain tissue in two epileptic children undergoing temporal lobectomy. The patients had been treated with the anticonvulsants in question for 2 years.The CBZ concentration in brain tissue was higher or equal to the plasma concentration. Brain/plasma ratio for CBZ was 1.0 and 1.4, respectively (grey substance). Brain/plasma ratio of CBZ‐epoxide was 1.0. Concomitant treatment with DPH increased the percentage of CBZ‐epoxide relative to CBZ in both brain and plasma. In white brain substance the concentration of CBZ, CBZ‐epoxide and DPH was higher or equal to the corresponding concentration in grey substance.No major age‐related differences in the distribution of anti‐epileptic drugs between brain and plasma in these two children compared to adult epileptic patients were noted.A new quantitative thin‐layer chromatographic method for the determination of DPH and phenobarbital (PB) in brain tissue and pl

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02867.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Epidemiologic survey over a restricted area of Romanian territory, i.e. the city of Bucharest with a population of 1,934,052 and a surface of 605 km2, established the following values for the MS parameters studied.On January 5, 1977 the prevalence rate for “probable” and “possible” cases was 46.4 per 100,000 inhabitants and for “probable” cases alone (with certain clinical diagnosis) 41.3 per 100,000.The specific 20‐ to 60‐year age adjusted prevalence rate was 79.4 per 100,000. The incidence rate over the 1969–1976 period was 1.78 per 100,000.The prevalence and incidence rates in this survey are similar to those reported for two other large urban centers of Romania (population over 100,000 each), Romania being therefore situated in a geographical area of “high risk” for MS.The 0.61 per 100,000 nationwide and the 0.65 per 100,000 Bucharest mortality rates are lower than those reported in other geographical areas

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02868.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Three out of 40 patients with myasthenia gravis had chronic thyroiditis. The thyroid disease preceded the neuromuscular disorder in two patients, while the third developed a slowly progressive hypothyreosis during the course of the myasthenia. One patient had an initial thyrotoxicosis with subsequent development of hypothyreosis. Of the remaining 37 patients, two had enlarged thyroid glands, positive family history of thyroid disease and antibodies to thyroid globulin, but were euthyreot.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02869.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Two cases of adrenoleukodystrophy are described. The pathological picture was consistent with earlier observations about the disease with the difference that the brain tissue revealed material which stained metachromatically with toluidine blue and was insoluble in fat solvents.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02870.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

摘要:

Deanol and placebo were administered to 10 parkinsonian patients with levodopa‐induced dyskinesias in a double‐blind, crossover fashion. Deanol and placebo did not differ significantly in their effects on dyskinesias. The reported properties of deanol seem to be attributable to a placebo effect. There was no correlation with the results of the physostigmine test. Despite these disappointing results deanol remains intriguing, because in individual cases remarkable improvements on dyskinesias are repor

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02871.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1978.tb02872.x

出版商:Blackwell Publishing Ltd    

年代:1978

数据来源: WILEY