摘要:

ABSTRACTThe prevalence of myasthenia gravis (MG) in Finland was 264 patients per 4.7 mil. inhabitants or 0.006 per cent. Of these 264 patients 19 (17 females and 2 males) were familial cases from eight families. MG occurred in 11 siblings, in two mothers and their child, and in six cousins. MG was not found in three or more successive generations. The familial MG resembled the non‐familial disease. No concordance of MG among five sets of twins was found. The birth localities of grand‐parents seemed to have a slight tendency for accumulation, but no definite clusters were formed. Consanguinity between parents was found in seven of 192 families. An increased frequency of collagen or thyroid diseases was found both in patients and parents, but they were not specially accumulated to the relatives of familial myasthenics. Neither autosomal nor sex‐linked, dominant or recessive, nor some other uniform mode of inheritance in MG could be confirmed. Some genetical predisposition to MG seems to exist, but it may be common to autoimmunity as a

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01445.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTPatients with the DMC syndrome have been suggested to possess a specific sulfatase abnormality and/or to be deficient in a proteinase cleaving glycoprotein‐acid mucopolysaccharide (AMP) linkage. We have previously found in DMC patients an abnormal excretion of urinary AMPs of which hyaluronic acid and chondroitin sulfate (A+C) were oversulfated and keratosulfate and heparan sulfate were undersulfated. Lysosomal acid proteinase, i. e. cathepsin D (EC 3.4.23.5) and neutral proteinase: elastase (EC 3.4.21.11) and cathepsin G were found to be normal in DMC patients. However, α2‐macroglobulin in serum was raised. This increase may be associated with a complex formation of α2‐macroglobulin with a neutral proteinase released from the cells. Increased levels of chondroitin sulfate N‐acetylgalactosamine‐6‐sulfate sulfatase and sulfamidase and decreased enzymic levels of arylsulfatase A and B (EC 3.1.6.1) were found in leucocytes of DMC patients. The sulfatase activities assayed in the present study support our theory that a specific sulfatase abnormality may exist in the

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01446.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTHemodynamic and morphometric studies on spontaneously hypertensive rats (SHR) have suggested that the lumen to wall ratio of the resistance vessels is decreased due to hypertrophy of the media. As the tension in the vessel wall increases with the pressure and the radius and decreases with the vessel wall thickness, such vessels would be expected to withstand higher pressure than vessels of normotensive rats (NR) unless local wall defects were present. Bicuculline‐induced epileptic activity and amphetamine intoxication, two conditions with cerebral vasodilatation and high blood pressure, give rise to extensive protein leakage in the brain of NR. Consistent with the hypothesis presented above, less protein leakage occurred in brains of SHR than in brains of NR after amphetamine and bicuculline administratio

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01447.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTIn a double‐blind crossover trial of two 12‐week treatment periods with a 4‐week treatment free interval, to which 21 patients were admitted, there was no statistically significant difference between clonidine 75 μg twice daily and placebo in the total number of headache days, migraine indices, duration of attacks, number of severe attacks and consumption of acute attack treatment. However, there was a marked reduction in number of headache days, migraine indices, duration of attacks and consumption of acute attack treatment during the second treatment period compared to the first treatment period, regardless of the treatment regime. This was presumably a result of prolonged treatment and frequent attention and not an effect of the active drug. 32 patients entered the trial, but 11 dropped out. Of the 21 patients completing the trial, 16 were women; the median age was 34 years (range 17–54 years) and the median duration of headaches 12 years (range 1–40 years). Only mild side‐effects were registered and no laboratory abnormaliti

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01448.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTThe catabolism of orally and subcutaneously administered14C histamine was studied in myotonic dystrophy patients. No definite abnormalities were observed as for subcutaneously administered histamine. The catabolism of orally administered histamine followed two distinct patterns: In patients with a relatively low endogenous urinary conjugated histamine excretion at the time of testing, small quantities of radioactivity were excreted in the stools (mean 0.25 per cent), whereas the quantity of exhaled14CO2was in the control range (mean 14.4 per cent), and the exhalation curve diphasic as in controls. In patients with a relatively high urinary conjugated histamine excretion, the catabolic pattern differed: There was a relatively high faecal excretion of radioactivity (mean 20.1 per cent), whereas the exhalation of14CO2was markedly reduced (i. e. mean 0.5 per cent, versus a mean control level of 8.9 per cent). Diphasicity of the exhalation curve was not present in these patients. It has previously been demonstrated that a patient with myotonic dystrophy may shift between being a normal and a high conjugated histamine excretor. It is thus possible that all patients with myotonic dystrophy may pass through phases with abnormal catabolism of intestinal histamine.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01449.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTThe electromyographic activity in several leg muscles during the elicitation of “the crossed up‐going toe sign” (CUT) was studied in patients and compared with that obtained in connection with the Babinski sign and in normal subjects. The results indicate that the patterns of activation and the responsible pathways are different for the CUT and Babinski signs. The difference between the normal subjects and the CUT positive patient is a matter of quantitative difference of coactivation of distal leg muscles during the test procedure rather than a change of reflex pat

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01450.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTA procedure is described where by ultrasonification one can separate large neurons from their surrounding neuropil from either unfixed brain and peripheral ganglion or from similar tissue fixed in 10 per cent neutral formalin for prolonged periods. The availability of such a technique permits one to readily assess the accumulation of3H‐labeled protein precursors into a wide variety of neurons, utilizing standard liquid scintillation techniques. The separation technique has been applied in this report to determine the effects of morphine, morphine plus naloxone, naloxone given alone and saline on the accumulation of3H‐1‐lysine into ventral horn, Purkinje and dorsal root ganglion neurons in Sprague‐Dawley rats. The data from the control and morphine‐treated animals has then been compared with similar data previously obtained from Wistar rats. In Sprague‐Dawley rats, morphine had no effect on3H‐1‐lysine accumulation into ventral horn neurons and stimulated accumulation into Purkinje and dorsal root ganglion neurons. Naloxone stimulated lysine accumulation into dorsal root ganglion and ventral horn neurons, but had equivocal effects on Purkinje neuron3H‐lysine accumulation. When Wistar and Sprague‐Dawley rats were compared, marked differences in the effect which morphine had on lysine accumulation into neurons were noted between the two strains of rat. Ventral horn and dorsal root ganglion neurons from Wistar rats had markedly higher levels of accumulation in both control and morphine‐treated rats than were observed in the Sprague‐Dawley animals. With Purkinje neurons, accumulation levels between the two strains overlapped each other. Morphine inhibited lysine accumulation in Wistar Purkinje neurons but stimulated it in the Sprague‐Dawley animals. The profiles of the accumulation curves from two rat strains suggest that there are not only differences in rates of uptake of3H‐lysine into protein followed by degradation between various types of neurons, but differences betw

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01451.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY

摘要:

ABSTRACTTwo brothers suffering from cluster headache of the chronic variant as well as hyperlipidemia are described. The mother very likely suffered from the same combination. Eleven consecutive cases of cluster headache were studied for lipid abnormalities. Among five patients of the intermittent type, one suffered from xantelasmata but had normal blood lipids. Among six patients of the chronic variant two had essential hyperlipidemia type IV. An etiological relationship between some cases of cluster headache and hyperlipidemia is therefore suggested.

ISSN:0001-6314    

DOI:10.1111/j.1600-0404.1977.tb01452.x

出版商:Blackwell Publishing Ltd    

年代:1977

数据来源: WILEY