ISSN:0014-3022    

DOI:10.1159/000117268

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Mitochondrial encephalomyopathies are a heterogenous group of disorders with various biochemical defects of the respiratory chain (RC). Due to the considerable phenotypic diversity of the RC encephalomyopathies, they are included in the differential diagnosis of many cases of multisystem disease. Aside from clinical evaluation and family history, diagnosis can be supported by many other sources. The complexity of diagnostic information can make it difficult for the clinician to establish the level of certainty at which a RC cy-topathy is diagnosed in atypical cases. We review the parameters (clinical, pathological, biochemical and molecular) which are used to aid diagnosis of RC encephalomyopathy and identify levels of abnormality in each that strongly or less strongly support the diagnosis. A system is developed that allows classification of the diagnosis in possible, probable and definite categories of certainty, which will be of value to the clinician.

ISSN:0014-3022    

DOI:10.1159/000117269

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Cerebral atrophy (CA) in stroke patients is associated with poststroke dementia and may reflect underlying neurodegenerative pathology. Therefore, regional CA may be valuable to study in patients who develop poststroke dementia. The aim of this study was to test the reproducibility of a qualitative rating scale of CA on MRI. MRI scans were performed in 50 consecutive patients (age range 19-81) admitted for an acute hemispheric ischemic stroke. CA was assessed on 2 occasions 24 h apart, on axial T2-weighted sequences by 4 independent observers. We evaluated CA in 13 regions on a 0-3 scale. The sum of the subscores was called the CA score (range: 0-39). The level of agreement was expressed by kappa statistics as well as by analysis of variance for interexaminer reproducibility studies. The mean CA scores ranged from 2.8 to 11.0, indicating the low prevalence of CA in this sample. Complete agreement was reached in 41.7% during the first assessment and in 44.1% in the second assessment. The interobserver agreement was moderate in the first session (mean overall kappa: 0.48) and substantial in the second (mean overall kappa: 0.67). The intraobserver agreement was good for all raters (mean kappa: 0.65). Standardized to the range of the scale, standard deviations of the differences between CA scores of the 4 raters in the 2 sessions were 11.1 and 11.2%; within raters it was 4.4%. We conclude that the assessment of CA using this rating scale is possible in stroke patients. It provides regional atrophy measurements and is reproducible when performed by 1 rater.

ISSN:0014-3022    

DOI:10.1159/000117270

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

The aim of this study was to evaluate the relationship between phenytoin medication and cerebellar atrophy in patients who had experienced clinical intoxication. Five females and 6 males, 21-59 years of age, were examined with a 1.5-T whole-body system using a circular polarized head coil. Conventional spin echo images were acquired in the sagittal and transverse orientation. In addition, we performed a high-resolution 3D gradient echo, Tl-weighted sequences at a 1-mm slice thickness. The images were subsequently processed to obtain volumetric data for the cerebellum. Cerebellar volume for the patient group ranged between 67.66 and 131.08 ml (mean 108.9 ml). In addition 3D gradient echo data sets from 10 healthy male and 10 healthy female age-matched volunteers were used to compare cerebellar volumes. Using linear regression we found that no correlation exists between seizure duration, elevation of phenytoin serum levels and cerebellar volume. However, multiple regression for the daily dosage, duration of phenytoin treatment and cerebellar volume revealed a correlation of these parameters. We conclude that phenytoin overdosage does not necessarily result in cerebellar atrophy and it is unlikely that phenytoin medication was the only cause of cerebellar atrophy in the remaining patients. Quantitative morphometric studies of the cerebellum provide valuable insights into the pathogenesis of cerebellar disorders.

ISSN:0014-3022    

DOI:10.1159/000117271

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Previous studies have demonstrated that hip fractures in stroke patients occurred almost exclusively on the hemiplegic side. We examined the bone changes in the second metacarpal of the hemiplegic side in terms of microden-sitometric indices in 93 stroke patients with hemiplegia. All six indices indicated a significant decrease in bone mass on the hemiplegic side compared with the contralateral side. Differences in the indices between the hemiplegic and contralateral sides were correlated well with the duration of the illness and Brunstrom’s stage for finger, arm, and leg. The same degree of osteopenia occurred in both small capsular and large hemispheric lesions. The same osteopenia was demonstrated in paralyzed and immobilized patients with myopathy. Thus a combination of weakness and immobilization is thought to be responsible for the osteopenia in the hemiplegic metacarpal bone. The osteopenia noted in the second metacarpal in the affected limb may account for the fact that hip fractures in stroke patients occur almost exclusively on the hemiplegic sid

ISSN:0014-3022    

DOI:10.1159/000117272

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

In order to quantify muscle strength in recessive myotonia congenita (MC) the peak and average peak torques (PT and APT, respectively) of knee flexion and extension of 19 MC patients were measured at speeds of 607s and 2007s. Muscle endurance was measured at a speed of 2007s. No differences were found between the patient and control groups for PT and APT values for flexion at the high speed, nor were there any differences between the patients and the controls for PT and APT measured at the low speed for knee flexion and extension or muscle endurance at the high speed. However, PT and APT values of the patients for extension at the high speed, 100 ± 41 Nm(mean ± SD) and 95 ± 39, were significantly lower than those of the controls, 129 ± 43 and 124 ± 42, respectively. There was no correlation between muscle strength or endurance and disease severity. The muscle strength of the patient group was diminished (p < 0.0001) during the beginning of the measurement at the high speed. The results suggest that after myotonic inhibition subsides the muscle strength of MC patients ranges within normal limits except in rapid and powerful motor activi

ISSN:0014-3022    

DOI:10.1159/000117273

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

The effect of oral treatment with acyclovir (ACV) on sensory axonal neuropathy, segmental motor paresis and postherpetic neuralgia (PHN) was studied in 105 patients with herpes zoster. Forty-seven patients were treated with ACV at a dose of 4 g/day in 5 doses for at least a week; the others did not undergo any kind of treatment. Electrodiagnostic examination of the nerves and muscles corresponding to the dermatomeric lesions was performed, including sensory and motor nerve conduction studies, blink reflex and electromyography (EMG). The patients treated with ACV showed a significant reduction in the number of cases in which there was electrophysiological evidence of axonal damage in afferent fibres of nerves arising from roots corresponding to affected dermatomes. The treated group also showed a smaller incidence of segmental motor neuritis, clinically evident or only detectable by EMG as denervation. However, there was no significant difference between groups as far as the incidence of PHN was concerned. Oral treatment with ACV therefore reduces peripheral sensory axonopathy due to ganglion damage and prevents the possibility of spread to anterior roots and spinal motoneurones. In this way it reduces the incidence of segmental motor neuritis, but does not reduce the incidence of PHN.

ISSN:0014-3022    

DOI:10.1159/000117274

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

In a retrospective brain magnetic resonance imaging study, we evaluated the prevalence and severity of signal hyperintensities in 30 elderly depressed patients and 30 controls matched for age, sex and cerebrovascular risk factors. A semiquantitative scoring method was used to grade findings in T2-weighted and proton density images. The elderly depressed patients had more extended periventricular hyperintensities, especially in the frontal region (depressed vs. control, 87 vs. 57%, p < 0.05), pons (33 vs. 7%, p < 0.05) as well as hyperintensities in the putamen and globus pallidus (57 vs. 27%, p < 0.05). The third ventricle was more dilated in depressed patients than controls after adjustment for age and cerebrovascular risk factors. The global index for ventricular enlargement was correlated significantly (r = 0.36, p < 0.05) with the severity of the hyperintensity in depressed patients. Our results indicate that these hyperintensities, especially in the frontal region, pons and lenticular nuclei, and the dilatation of the third ventricle play an important role, through the frontal-subcortical circuits, in mood regulation of elderly depressed patients.

ISSN:0014-3022    

DOI:10.1159/000117275

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Urinary pyridinoline and deoxypyridinoline were analyzed in 23 patients undergoing long-term anticonvulsant drug therapy and compared with those in an age-matched control group, which consisted of 218 healthy premenopausal women. Values of urinary pyridinoline and deoxypyridinoline in the patient group were significantly higher than those in the control group. However, mean serum levels of alkaline phosphatase in the patient group were within the control range. Our data demonstrate that bone resorption is accelerated by long-term anticonvulsant drug therapy and there may be an imbalance between bone resorption and bone formation in the patient group.

ISSN:0014-3022    

DOI:10.1159/000117276

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

A group of young patients with insulin-dependent diabetes mellitus (n = 14; 8 men, 6 women; 33.1 ± 8.9 years) were examined by topographic EEG mapping under normoglycemic and hypoglycemic conditions (glucose levels after intravenous insulin injection down to 32.6 ± 7.6 mg/dl). From the clinical aspect, 7 of them had a good and 7 had a poor awareness of hypoglycemia. During hypoglycemia, a decrease in alpha activity (p < 0.05), an increase in delta (p < 0.05), and especially in theta activity (p < 0.05) were found. The most sensitive parameter was the alpha/theta ratio. In the range of slight hypoglycemia (50-60 mg/dl) the increase in delta and theta activity showed a topographic maximum in lateral frontal regions. During deep hypoglycemia there was a topographic maximum of slow frequencies in posterior parts of the brain (centrotemporal to parieto-occipital regions). The differences between the group with good and with poor awareness of hypoglycemia were most pronounced during slight hypoglycemia in C3, C4, and Pz (p < 0.05). At lower glucose levels group distinction was no longer possible. These EEG changes correspond to a temporary organic brain syndrom

ISSN:0014-3022    

DOI:10.1159/000117277

出版商:S. Karger AG    

年代:1996

数据来源: Karger