摘要:

ABSTRACTInspired by the works of William House, the authors formed an otoneurosurgical team in order to improve the results after surgery for acoustic neuromas. This paper deals with the preliminary results obtained with the translabyrinthine approach in 13 patients with acoustic neuromas. In 9 patients it was possible to remove the tumor totally with this approach, in 4 patients a second suboccipital operation was necessary to secure total removal. One small, 7 medium and 5 large tumors were encountered. The facial nerve was preserved in 83 per cent of the patients. One patient with a large tumor died after the second suboccipital operation. The relation between size of the tumor and the outcome of the operation is stressed, and in order to reduce the number of large tumors it is suggested that all patients with unilateral hearing loss should be suspected of having a neuroma, until the diagnosis has been disproved. It is concluded that the surgery for acoustic neuromas is otologic‐neurosurgical teamwork, and that the treatment should be centralize

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01435.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTContent, composition and molecular weight distribution of the urinary glycosaminoglycans (GAG) were determined in five patients with progressive myoclonus epilepsy (PME). In one patient (Family B) this syndrome was associated with cerebral Lafora bodies and in four siblings of Family A, no Lafora bodies were present in brain biopsy. Only one of the five patients had a moderate increase of urinary GAG excretion as expressed by 24‐h output or creatinine. The heparan sulfate component of the GAG was moderately increased in two other patients. The molecular weight distribution of the urinary GAG was normal. The results do not support the contention that urinary GAG excretion is abnormal in PME. Among nine lysosomal enzymes in leucocytes, only the activity of α‐mannosidase was increased 3‐fold in the four si

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01436.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTIn order to evaluate the prognosis and possible prognostic factors associated with a benign course, 118 cases of motor neuron disease diagnosed 1948–1975 were followed up. Survival curves, based on the actuarial method showed a survival rate of 18.7 per cent (95 per cent confidence limits: 11.4–26.0) and 7.6 per cent (95 per cent confidence limits: 2.7–12.5) after 5 and 10 years respectively. Comparing the survival curves of males/females, cases with bulbar/spinal‐and upper/lower extremity onset, cases with/without upper motor neuron signs, only bulbar onset was associated with a significantly poorer prognosis. However, cases with bulbar onset had a significantly higher mean age of onset. Based on cases from Funen county with a 1970‐population of 432,699 we found an average annual incidence rate, mortality rate and period prevalence rate of 0.85, 0.86 and 2.5 per 100,000 respectively. Comparing the incidence per 5‐year period through 1948–1972, no significant deviations indicating a changing environmental fact

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01437.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTA family from Western Norway is described in which 5 out of 9 members in one generation developed a progressive encephalopathy in middle life. Massive, symmetrical calcifications located in basal ganglia, dentate nuclei and cerebral sulci of the brain were seen on roentgenograms of the skull. All affected members exhibited a clinical syndrome which included mental deterioration, extrapyramidal motor deficit, cerebellar ataxia and tremor. The biochemical investigation showed normal serum calcium and phosphorous and concentration of immunoreactive parathyroid hormone was normal. The Ellsworth‐Howard test with exogenously administered parathyroid extract revealed a subnormal phosphorous diuresis while urinary excretion of cyclic AMP was normal. Thus, the defect appears to be an insufficient intracellular response to cyclic AMP. The late onset of symptoms is compatible with the slight disturbance in calcium‐phosphorous metabolism we have demonstrated. The family probably represents an unusual type of pseudo‐pseudohypoparathyroidism of which only one other family has been reported earlier. The investigations and pedigree analysis of the present kindred is suggestive of an autosomal recessive inheritance of the dis

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01438.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTIn a consecutive series of 11 young patients with clinical evidence of cerebral infarction, platelet function and plasma lipid levels were examined. The results show that increased platelet aggregability characterizes the patient group as a whole. Hyperlipidemia was frequently found, and the number of heavy smokers was high. It is suggested that all younger patients with cerebrovascular disease are examined with regard to these presumed risk factors.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01439.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTThe influence of shortlasting (less than 1 min) epileptic seizures on the permeability to protein of the blood‐brain barrier (BBB) was studied in rats. The protein tracer, horseradish peroxidase (HRP) was used as marker substance. Monitoring arterial blood pressure (BP) and electroencephalogramme (EEG) seizures were induced electrically after HRP was given intravenously. Following a single electroshock seizure slight staining of brain tissue was seen, while after 10 electroshock stimuli followed by sustained seizure activity, this phenomenon was more pronounced. If 10 electroshock stimuli were preceded by transsection of the spinal cord, blood pressure increase was abolished and no tissue staining was seen in spite of epileptic seizure activity recorded on EEG. This means that the acute hypertension and not the seizure activityper seis the mechanism behind the breakdown of the BBB during epileptic seizures. Electron microscopy revealed an increased vesicular transport (pinocytosis) across the endothelial cells, while the vascular structure remained intac

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01440.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTHypoxic brain injury is the most important neurological problem in the neonatal period and accounts for more neurological deficits in children than any other lesion. The neurological deficits are notably mental retardation, epilepsy and cerebral palsy. The pathogenesis has hitherto been poorly understood. Arterial hypoxia has been taken as the obvious mechanism but this does not fully explain the patho‐anatomical findings. In the present investigation we have examined the arterial blood pressure and the cerebral blood flow in eight infants a few hours after birth. The 133Xe clearance technique was used for the cerebral blood flow measurements. The study confirmed that perinatal distress may be associated with low arterial blood pressure, and it was shown that cerebral blood flow is very low, 20 ml/100 g/min or less, in hypotensive perinatal distress. It is concluded that cerebral ischaemia plays a crucial role in the development of perinatal hypoxic brain injur

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01441.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTAfter treatment with diphenylhydantoin for 2 years because of postinfarct focal motor epileptic seizures, a 35‐year‐old woman was admitted to hospital because of symptoms of intoxication. Almost every sign of the previous left‐sided paresis and hypaesthesia had disappeared. A pronounced gingival hyperplasia was found almost exclusively in the left side. The concentration of immunoglobulin A in serum was found far below normal. Admitting that she had not been careful brushing her teeth in the left side because of the strange feeling there (dysaesthesia), the patient had actually carried out a controlled trial proving that she, being predispposed to gingival hyperplasia with a low level of immunoglobulin A in serum, could almost prevent the gingival hyperplasia in the right side by brushing her teeth carefully

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01442.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTRecent identification of glutamate as a transmitter in neocortical efferents and discovery of toxic effects of excessive amounts of extracellular glutamate or its analogues on some brain neurons offer an explanation of the pathogenesis of Huntington's chorea. Cell death as well as consequent biochemical changes and clinical symptoms in this disease possibly result from excessive excitation of the neostriatal, and of some other neurons by glutamate released from neocortical axon terminals. If so, the development of Huntington's chorea could be prevented or arrested by blockers of glutamate transmission, and folate should be reduced to the minimum in the diet of the genetically marked families.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01443.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01444.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY