ISSN:0014-3022    

DOI:10.1159/000117245

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

31P magnetic resonance spectroscopy (MRS) was used to study an open therapeutic trial of coenzyme Qio (CoQ) in mitochondrial encephalomyopathies. Eight patients were treated with 150 mg CoQ per day for 6 months. 31P MRS spectra of calf muscle were recorded at rest, during exercise and in the immediate postexercise recovery period. Although there was an improvement of the mean ratio of phosphocreatine (PCr) to inorganic phosphate during the post-exercise recovery period after 3 months of treatment, this finding was mainly due to a single therapy responder and did not reflect a beneficial effect on the whole group. Improved repletion of PCr persisted after 6 months of therapy. Our study identified a single responder to this therapy, whose response could not be predicted on the basis of clinical, biochemical or molecular data. These findings suggest that therapeutic trials of CoQ should be performed under close metabolic monitoring in order both to identify responders for subsequent long-term treatment and to evaluate possible mechanisms of this supportive therapy.

ISSN:0014-3022    

DOI:10.1159/000117246

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

This study aimed at examining correlations between quantitative REM sleep EEG, regional cerebral blood flow measured by single photon emission computerized tomography (SPECT) and global cognitive functioning in nine patients with mild to moderate Alzheimer’s disease. REM sleep EEG was not correlated with SPECT measures in any of the 6 regions studied. However, degrees of interhemispheric asymmetry calculated by both measures were concordant for the parieto-occipital region. Mini-mental state score correlated with REM sleep EEG slowing for left frontal, left and right parieto-occipital and left temporal regions but was not correlated with SPECT measures for any of the six regions. These results suggest that quantitative REM sleep EEG would be more useful than SPECT for evaluating cerebral dysfunctions in mild to moderate Alzheimer’s dise

ISSN:0014-3022    

DOI:10.1159/000117247

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

The importance of immunoglobulin M (IgM) determination in cerebrospinal fluid (CSF) has increased parallel to the need for early diagnosis of infectious and inflammatory disorders of the central nervous system. Current reference values are based on analysis of CSF from ‘reference groups’ consisting of patients with psychiatric and/or neurological symptoms but without positive clinical findings. Therefore, accurate reference values for CSF IgM are of utmost importance. The present study presents reference values for IgM in CSF in a large sample (n = 111) of healthy individuals with an age span of 18-88 years. The upper reference limit, calculated as the 0.95 fractile, was 0.36 mg/l for CSF IgM, 0.40 for CSF/serum IgM ratio, and 0.045 for the IgM index. These parameters showed no significant difference between sexes nor any significant correlations with age. The correlations between CSF/serum albumin ratio and CSF/S IgM ratio were linear and statistically highly significant, suggesting that the IgM index values do not depend on the blood-CSF barrier function within the normal range of the CSF/serum albumin ra

ISSN:0014-3022    

DOI:10.1159/000117248

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Sixty patients, 53 men and 7 women, referred for excessive snoring or suspected sleep apnea syndrome were analyzed by polysomnography, Mueller maneuver, cephalometric roentgenography, and pulmonary function testing, to evaluate the contribution of static and dynamic upper airway obstruction in the pathogenesis of sleep-related breathing disorders and OSAS. Desaturation index and maximal desaturation were used in the analysis as indicators of severity of sleep-related breathing disorders. Body Mass Index, increased collapsibility at the base of the tongue by the Mueller maneuver, increased distance between hyoid and mandibular plane, and increased soft palate diameters by cephalometry as well as a decreased peak inspiratory flow by pulmonary function testing were found to be related to increased oxygen desaturation parameters. In a multivariate analysis the significant obstruction parameters could explain only 37% of the variability of maximum desaturation and 31% of the variability of desaturation index, 63 and 69%, respectively, must therefore be explained by other mechanisms. We conclude that instability of respiratory and muscular control, possibly enhanced by sleep-phase-related changes, is more important in the pathogenesis of sleep-related breathing disorders than pure anatomic narrowing of the airway.

ISSN:0014-3022    

DOI:10.1159/000117249

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

The autonomic cardiovascular system was studied by means of autonomic tests and heart rate variability related to body movements during sleep, in 20 patients with relapsing-remitting multiple sclerosis in a stable phase and in 9 normal subjects. Responses to autonomic tests in multiple sclerosis and control subjects were similar. Heart rate variability, instead, showed a lower degree of adaptability in patients with multiple sclerosis than in controls during sleep, because of sympathetic system dysfunction. No significant correlation between magnetic resonance lesions and cardiovascular sleep indexes was found.

ISSN:0014-3022    

DOI:10.1159/000117250

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Syringomyelia is characterized by a longitudinal cavity or several cavities in the spinal cord, and syringobulbia demonstrates similar pathology in the brainstem. Both conditions typically present with slowly progressing dissociative sensory deficits as well as upper and lower motor neuron deficits. We recently encountered 3 patients who presented with the rapid onset of such signs and symptoms and had cervical syringes. All 3 patients would have been diagnostic dilemmas had it not been for MRI, which identified a syrinx in each patient, as well as an Arnold-Chiari malformation in 2 of the 3. Syringomyelia and syringobulbia should be considered in the differential diagnosis of patients who have acutely evolving brainstem or cervical spinal cord deficits. MRI is the imaging modality of choice for detecting syringomyelia or syringobulbia presenting atypically.

ISSN:0014-3022    

DOI:10.1159/000117251

出版商:S. Karger AG    

年代:1996

数据来源: Karger

ISSN:0014-3022    

DOI:10.1159/000117252

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Acute disseminated encephalomyelitis (ADEM) in middle-aged or elderly adults is rarely reported. We present here three ADEM patients of these age groups, who manifested behavioral changes, mutism or psychosis. Single clinical episodes, widespread encephalopathic disturbances and magnetic resonance imaging (MRI) findings favored the diagnosis of ADEM. However, no obvious preceding infections could be discovered. Two of them had traveled to mainland China prior to admission to psychiatric wards. All three patients had fair-to-good recovery after steroid treatment. A review of the literature showed that clinical and neuroimage findings of ADEM in the elderly group did not differ from those in the younger group. However, ADEM has rarely been reported in older age groups, probably due to: (1) differences between the elderly and young people in susceptibility or immune response to infectious agents, and (2) lack of a sensitive tool to examine patients prior to the widespread use of MRI. We suggest ADEM as one of the possible etiologies of acute or subacute onset of psychosis in adult patients, especially in those with a history of traveling.

ISSN:0014-3022    

DOI:10.1159/000117253

出版商:S. Karger AG    

年代:1996

数据来源: Karger

摘要:

Charcot-Marie-Tooth (CMT) type-1 (CMT1) neuropathy is characterized by peripheral nerve demyelination and has been divided into several subtypes. The most frequent among these, subtype 1 A, is related to a microduplication of the region p11.2 of chromosome 17. This region contains the PMP-22 gene which is involved in peripheral nerve myelination. Since motor nerve conduction velocity (MNCV) is closely related to nerve myelination, we compared type-1 A patient MNCVs versus non-A CMT1 patient MNCVs, in 57 CMT1 A patients and 21 non-A type-1 patients. Patients with the 17p11.2 duplication have MNCVs that are significantly more reduced (about 20 m/s) compared to patients without the 17p11.2 duplication (about 30 m/s). This study also permits a model of the MNCV in the median nerve (MedMNCV) of CMTl patients, with age, gender and molecular status as parameters. Furthermore, in order to help clinicians to diagnose subtypes of CMT1 patients, the probability for type 1A is modeled as a function of MedMNCV only.

ISSN:0014-3022    

DOI:10.1159/000117254

出版商:S. Karger AG    

年代:1996

数据来源: Karger