摘要:

Typical cases of MELAS present a combination of clinical and neuroradiological features, lactacidaemia, and ragged red fibers (RRFs) in striated muscle. We have observed a MELAS‐like syndrome in monozygotic twins. They developed seizures typically in conjunction with physical exertion, sleep deprivation or febrile episodes. Stroke‐like episodes occurred usually during seizures. In twin 2 the course was fatal at age 20 years. Neuroradiological findings were typical of MELAS. Plasma lactate was normal in both. CSF lactate was normal in twin 1 and normal/elevated in twin 2. RRFs were not seen in muscle biopsies of the twins. Complex I activity was reduced in muscle in twin 1. Brain tissue removed at epilepsy surgery in twin 2 showed the presence of mitochondrial angiopathy. The commonest mitochondrial DNA mutation in MELAS, at base pair 3243, was absent. Lactacidaemia and mitochondrial myopathy with RRFs constitute part of the diagnostic criteria of MELAS. However, the absence of these features does not exclude mitochondrial disorder with the serious manifestations of MELAS (seizures and stroke‐like episodes) as seen in these

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07058.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Introduction– Down's syndrome patients express a neurodegenerative disorder and mental retardation. We studied the reactivity of EEG and its correlation with neuropsychological test score in Down's syndrome.Material and methods– We studied 32 patients with Down's syndrome and 31 controls for blocking of occipital EEG activity. The temporo‐occipital EEG with eyes open (EO) was compared with resting EEG with eyes closed (EC), (EC/EO ratio).Results– Both Down patients and controls showed significant diminution of alpha, beta and theta activity and decrease of EEG frequency with EO. However, there was a significant impairment in Down patients in the EC/EO ratio in alpha band, compared to controls. The controls had no correlation of the alpha EC/EO ratio with age or gender. The Down patients showed a significant correlation of this variable with age which is in accordance with a gradually progressing disease. They had also significant correlations of the alpha EC/EO ratio and neuropsychological test scores which indicates that this ratio may be a more general measure of cerebral or hemispherical dysfunction than a mere impairment of visual activation. Down patients also showed significant differences in resting EEG variables, compared to the controls, even if the conventional EEG showed normal or mildly slowed dominant occipital rhythm in most of the patients. The correlation analysis between resting EEG and EC/EO ratio variables pointed out that they are relatively independent, representing different factors in the regulation of EEG.Conclusions– We believe that the alpha EC/EO ratio of EEG add a new domain in the assessment of cerebral dysfunction in Down's

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07059.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

The antiepileptic effect of lamotrigine (Lamictal®) was assessed in a double‐blind, placebo‐controlled, crossover trial in 56 adult patients with refractory partial seizures. Lamotrigine or placebo was added to the patients' existing antiepileptic drugs (AEDs). The dose of lamotrigine varied from 75 to 400 mg daily. Thirty‐eight patients completed the trial and 7 withdrew because of adverse experiences. There was a statistically significant reduction in seizure counts on lamotrigine compared with placebo for total seizures (30.3% reduction, 95% CI 8.4%, 47.0%), complex partial seizures (29.2.% reduction, 95% CI 3.8%, 47.9%) and secondary generalised seizures (37.9%, CI 18.9%, 52.4%). The analysis of total seizure days showed a similar significant reduction during lamotrigine treatment for the same seizure categories. There was no statistically significant difference in reporting of adverse events between lamotrigine and placebo except for dizziness which was reported more frequently on lamotrigine than on placebo. There were no differences in abnormal haematological or biochemical findings between lamotrigine and placebo, and lamotrigine had no effect on plasma concentrations of concomitan

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07060.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

The function of thick and thin sensory nerve fibers after surgical decompression in carpal tunnel syndrome were evaluated using quantitative sensory testing (QST). The thin nerve fibers were studied using tests for thermal thresholds, and the thick myelinated fibers by vibrametry. The tests were performed before surgery and at 6 weeks, 4 and 10 months after surgery. The improvement of function in thin nerve fibers came within 6 weeks (P=0.001). The improvement of function in thick myelinated fibers continued to improve until 4 months after the operation (P=0.0001). This difference in the time course of the recovery indicates that the thick myelinated nerve fibers were more affected than the thin nerve fibers. The function of both type sensory nerve fibers did also improve in the fifth finger (P=0.05). The function in thin nerve fibers worsened somewhat between 6 weeks and 4 months after surgery, possibly due to postoperative scar tissue.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07061.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

We report the clinical features, electrophysiological studies, sural nerve pathology and recovery course of carbon disulfide‐(CS2) induced polyneuropathy in a 48‐year‐old man who worked in a viscose rayon plant. Sural nerve biopsy 2 years later still showed degeneration of both axon and myelin with a predominant loss of large myelinated fibers and remyelination. Electrophysiologic studies revealed mixed axonal and demyelinating polyneuropathy. To our knowledge, this is the first human report of sural nerve pathology in the recovery stage due to CS2 intoxication. After diagnosis, the patient was removed from the toxic environment. In the following three years, he showed part recovery predominantly in motor function compatible with the serial nerve conduction studies. We conclude that CS2 polyneuropathy may partly recover years after cessation of exp

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07062.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

We present the results of the first epidemiological study of ALS in Belgrade. The distribution of 58 newly discovered cases in a 7‐year survey period (1985–1991) showed that the average annual age‐adjusted incidence rate was 0.42 per 100,000 population (95% confidence interval, 0.18–0.83). The rate for males was 1.5 times higher than the rate for females. The greatest age‐specific average incidence rate was observed in patients between 60 and 64 (3.66 per 100,000 population; 95% confidence interval, 2.17–5.78). The actual age‐adjusted prevalence rate on December 31, 1991 was 1.07 per 100,000 (95% confidence interval, 0.71–1.71). The mean age at onset of the disease was 56.2 ± 9.8 and it ranged from 24 to 74. We studied the natural course of the disease through the mean duration and cumulative probability of survival. The mean duration of the disease was 27.7 ± 18.2 months. The cumulative probability of survival was 27% for the whole population in a 5‐year interval. Elderly patients and those with bulbar signs at onset had a poorer prognosis. Patients under 49 at onset and those with the spinal form of the dise

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07063.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Background– Despite many reports on metastatic spinal cord compression, only very few prospective studies of the clinical outcome of spinal cord compression have been carried out.Methods– 153 consecutive patients with a known malignant solid tumor and a myelographically verified diagnosis of spinal cord compression were followed with regular neurological examination.Results– At time of diagnosis 79 patients were walking, while the remaining were bedridden. In total 21 of the 74 initially non‐walking patients began walking after therapy. There was a need for urinary catheter in 57 (37%) patients at the time of diagnosis. During follow‐up, 10 of 57 patients (18%) dispensed with the catheter. A total of 116 patients experienced radicular pain at the time of diagnosis, while in 95 of 116 patients (83%) the pain disappeared after therapy.Conclusion– the present study confirms, that early diagnosis, i.e., while the patients are still ambulatory, is most important, but the prognosis for recovery of ambulatory function is not as pessimistic as earlier described. In addition the results indicate that supplementary systemic therapy, when available, may have a positive influence

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07064.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Electrophysiological examinations were done on 20 patients aged 40–71 years with recently diagnosed high grade non‐Hodgkin's lymphomas. General chemotherapy and intrathecal chemotherapy in order to prevent central nervous system (CNS) involvement were begun. On the first day of chemotherapeutic cycle patients received intrathecally methotrexate (ITMTX) and prednisolone. Electrophysiological study was carried out twice in each subject: before ITMTX injection and a day after injection. The study procedure included: a conventional nerve conduction examination (peripheral conduction velocity and compound muscle action potential amplitude), the F wave latency and amplitude measurement and F ratio (F‐M‐1/2M) calculation for peroneal and tibial nerve bilaterally. Results of the first and the second examinations were statistically compared by t‐Student's test. No significant differences between values of estimated parameters were found. The study revealed no recent alterations in proximal, paraspinal motor conduction and motor neuron excitability due to antidromical activation after single ITMTX admin

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07065.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Introduction– Focal neurologic deficits in sepsis frequently result from parenchymal lesions due to cerebral embolism. The aim of this study was to characterize clinical, laboratory and radiologic patterns of those patients.Patients and methods –‐ Medical records of 30 patients with focal neurologic symptoms during sepsis were analyzed retrospectively.Results– 24 patients (22 with infective endocarditis) had ischemic stroke. Cerebrospinal fluid (CSF) analyses revealed inflammation in 11 of 12 patients. Patients who died (11/24) suffered more frequently from secondary intracerebral hemorrhage (p = 0.0031), which was significantly associated with intravenous high‐dose anticoagulation (p = 0.0059). Six patients had slowly progressive focal neurologic deficits without evidence for stroke. All showed CSF inflammation and three developed multiple cerebral abscesses.Conclusions– There are two distinctive groups of patients with focal neurologic deficits during sepsis. One presents with stroke and CNS inflammation (septic embolic focal encephalitis). The other group develops slowly progressive focal neurologic deficits and sometimes multiple cerebral abscesses (septic metastatic focal en

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07066.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY

摘要:

Introduction– In HTLV‐1 associated myelopathy/tropical spastic paraparesis (HAM/TSP), we correlated human leukocyte antigen (HLA) haplotypes to the fine specificities of intrathecally synthesized IgG antibodies against HTLV‐1.Patients and methods– HLA haplotypes of HAM/TSP patients were determined by the standard NIH microcytotoxicity test and family HLA studies. IgG antibodies against HTLV‐1 synthetic peptides in paired CSF and serum were measured by enzyme immunoassay, and intrathecal synthesis of antibodies was evaluated.Results– HAM/TSP patients with particular HLA haplotypes (A24Cw7B7DR1DQ5, A2Cw7B7DR1DQ5, A24Cw‐B52DR15DQ6, A11Cw1B54DR4DQ4, and A24CwlB54DR4DQ4) showed more frequently intrathecal synthesis of antibodies against HTLV‐1 synthetic peptides, especially against HTLV‐1 env gp21 synthetic peptides.Conclusion– In HAM/TSP, a retrovirus‐induced human chronic inflammatory disease of the CNS, this is the first report to provide evidence that the intrathecal antiviral immune response is influenced by immunogenetic facto

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1996.tb07067.x

出版商:Blackwell Publishing Ltd    

年代:1996

数据来源: WILEY