摘要:

The mitochondrial respiratory chain encephalomyopathies represent an important group of multisystem disorders. No curative treatment is currently available. A number of measures have been reported to have a theoretical potential to improve respiratory function. These treatment strategies have variable scientific support, many reports being anecdotal. We critically review the various therapeutic measures employed and suggest future treatment directions.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00130.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

The relation between nightly growth hormone (GH) secretion and sleep is poorly understood. To examine whether disturbances in GH secretion are reflected in abnormal sleep patterns 8 subjects with isolated GH deficiency and 9 subjects with excess of GH (acromegalics) underwent all night sleep studies, polysomnography. Moreover, the effect of correcting GH concentration on sleep patterns were examined in the same subjects. The results showed that all subjects with GH disturbances had abnormal REM and delta sleep and normalization of GH concentration was followed by correction of the sleep stages. By power spectrum analysis of the sleep EEG it was showed that during low GH concentration the sleep energy was low, and high GH concentration was associated with high sleep energy, and correction of abnormal plasma GH levels resulted in normalization of REM and delta sleep energy per time unit.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00131.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

An association between late‐onset Alzheimer's disease, vascular dementia and the common polymorphic alleles of the gene coding for apolipoprotein E, ɛ2, ɛ3 and ɛ4, was assessed in a population sample of 393 elderly Finnish men aged 70 to 89 years. Of them, 7%, suffered Alzheimer's disease and 3% had vascular dementia. Among those who suffered Alzheimer's disease, there was a statistically significant excess of the ɛ4 allele. No such an association was observed between the apolipoprotein E alleles and vascular dementia. We conclude that the apolipoprotein E polymorphism confers information about a risk of Alzheimer's disease in this population sample of elderly Finnis

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00132.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Using a case registry, we investigated the temporal occurrence of motor neuron disease (MND) on Guam. MND with onset during 1941–85 was documented in 434 Chamorros and 9 non‐Chamorro migrants who had lived on Guam before onset. Increased median age at onset and decreased age‐adjusted incidence rates (since the early 1960s) were observed for Chamorros of both sexes. Our evidence about MND on Guam is consistent with: 1) The latent period duration has varied from years to decades; 2) With time, the exposure period or latent period, or both, have lengthened; 3) The high risk of acquiring the condition has been reduced since, at least, the early 1950s, and the most recent years of meaningful risk were the early to middle 1960s; 4) The critical age for acquiring the condition is in adolescence and adulthood; 5) Change of environment from Guam to overseas during childhood resulted in decreased risk of acquiring the cond

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00133.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Athetosis and dystonia are well known clinical signs, described in disorders of basal ganglia. As opposed to pseudoathetosis, true athetosis was hitherto not reported in cord lesions. We here report three patients with athetosis and dystonia of hands due to intramedullary lesions of cervical cord: two patients with syringomyelia and one with glioma. Even though pseudoathetosis can be produced by lesions of posterior columns and likely to be confused with the involuntary movements of our patients, they had clinical and EMG findings consistent with true athetosis. A possible explanation for the athetosis and dystonia due to cord lesion is being postulated.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00134.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

We performed DNA analysis in four families with hereditary neuropathy with liability to pressure palsy (HNPP). An interstitial deletion of the 17 p11.2 region was found in typically affected patients as well as in as yet asymptomatic patients. The opportunity for an individual genotyping permitted to ascertain ade novodeletion in one clinically affected case with no relevant familial history. DNA analysis thus becomes the most sensitive tool in diagnosing HNPP, since potentially affected patients may lack either informative familial history, or clinical symptoms or even suggestive EMG or histopathological data (tomaculas).

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00135.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

To investigate the influence of diabetes mellitus on higher cognitive functions electrophysiologically, we studied auditory P300 event‐related potentials (P300) in 40 NIDDM patients, taking into account wave I‐V latencies (I‐V) in auditory brainstem evoked potentials, clinical parameters and head MRI findings. Compared with 20 controls, diabetics had significantly longer P300 and I‐V latencies. P300 latencies in diabetics correlated with neither I‐V. HbA1, blood glucose levels, nor disease duration. Of the 13 diabetics investigated neuroradiologically, four had lacunar infarcts with prolonged electrophysiological values. The remaining nine had normal MRI scans, but their physiological parameters were still significantly longer than those of controls. These findings suggest that NIDDM can independently alter higher cognitive and the central auditory pathway functions. Our data also suggest that these alterations occur regardless of the recent metabolic derangement and disease duration. Cerebrovascular ischemia, if present, also appears to contribute in part to cognitive al

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00136.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Introduction‐ Explanations of sentence processing difficulty in aphasia have implicated slowed information processing speed. We tested this hypothesis by evaluating sentence comprehension in multiple sclerosis (MS), and relating comprehension performance to measures of information processing speed.Material&methods‐ Twenty right‐handed, high school‐educated, non‐demented, native English speakers with clinically definite MS and 16 age‐ and education‐matched control subjects were examined on 3 different sentence comprehension measures that stress grammatical appreciation. Performance was related to quantitative assessments of mental information processing speed.Results‐ Group‐wide analyses demonstrated a trend toward sentence comprehension difficulty in MS. Analyses of individual patient profiles identified a subgroup of MS patients who were consistently impaired to a significant extent across all sentence comprehension tasks. Their sentence comprehension difficulty was associated with selectively compromised mental information processing speed.Conclusion‐ Sentence comprehension difficulty in MS is associated with slowed information processing speed. This finding supports the claim that information processing speed contributes to

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00137.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Introduction‐ In patients with cervical root syndromes, the relation between clinical findings and EMG results, the value of the registration of the H‐reflex latency of the flexor carpi radial muscle (HFCR) and the rate of recovery of EMG abnormalities following surgery are unclear.Methods‐In 68 patients with cervical radicular syndromes caused by intervertebral disc lesions, EMG was made shortly before anterior cervical discectomy and four months later. EMG consisted of needle myography and bilateral determination of the HFCR.Results‐ Results of HFCR were unrelated to findings on needle myography. Preoperative EMG abnormalities were related to more severe clinical and myelographic findings. A preoperative abnormal HFCR correlated with good clinical outcome. No relation was found between the clinical outcome and EMG‐findings during follow‐up.Conclusion‐ Determination of HFRC is a useful EMG‐test, but further comparison to tendon reflexes is necessary. EMG identifies patients with more severe root lesions, but cannot be used for evaluation of persistent complaints within the first half year fo

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00138.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY

摘要:

Transient global ischemia leads to glutamate mediated delayed neuronal death in the CAI but not in the CA3 region of the rat hippocampus, and changes in AMPA receptor subunit composition has been proposed to cause a difference in excitatory input to the CAI and CA3 regions. In situ hybridization with riboprobes for AMPA receptor subtype GluR1–4 mRNA was performed on sections from the brain of sham operated and ischemic rats in two models (neck cuff and 4‐vessel occlusion combined with hypotension) with identical results: the content of the GluR1–3 mRNA species was down regulated in the hippocampal regions CAI and CA3 but only weak changes were observed in the dentate gyrus. The down regulation observed in CA1 was non‐selective among GluR1–3, i.e. all GluR mRNA species showed approximately the same degree of down regulation. A change in calcium permeability of the AMPA channels mediated by a shift in channel sub‐unit composition and corroborating an increased calcium influx is thus not supported by the

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1995.tb00139.x

出版商:Blackwell Publishing Ltd    

年代:1995

数据来源: WILEY