Reversible limb-girdle muscular dystrophy.
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Biomedical subjects
Publications and source records attributed to K V Sørensen.
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22 patients with severe migraine resistant to previous prophylactic treatments participated in a prospective open trial of valproate in migraine prophylaxis; 17 had common and 5 classic migraine. The attack frequency was from 4 to 16(30)/month (1 patient suffered from attacks every day). The dose of valproate was 600 mg twice a day from start of treatment, successively adjusted to a serum level about 700 mumol/l. Follow-up was from 3 to 12 months; 11 patients were free from migraine attacks, 6 had a significant reduction of the frequency; in one patient there was no effect; 4 dropped out because of lack of cooperation; 3 who had been free from migraine for 3 months, participated in a withdrawal experiment during which they all experienced relapse. After reinstitution of valproate the attacks disappeared again. How valproate acts in migraine prophylaxis is not known, but this prospective open trial demonstrates that valproate has an appreciable prophylactic effect in patients suffering from severe migraine, and calls for controlled clinical trials.
The detection of somatostatin, a 14 aminoacid peptide, in human brain and cerebrospinal fluid (CSF) initiated examinations by radioimmunoassay and immunocytochemical technique to elucidate its origin, localization, function, and possible significance in central nervous system disorders. The present survey deals with these aspects with special reference to multiple sclerosis (MS) and to correlation between disease activity and somatostatin content and variations in CSF.
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Examination of the cortex and the subcortical white matter by use of an immunocytochemical technique--the per oxidase anti-per oxidase method--shows that somatostatin is located in a widespread neuron system with cell bodies localized in both the cortex and the subcortical white matter of the human brain. In the cortex, the somatostatin cell bodies and fibers are found in all layers, but the fibers are especially numerous in layer I located tangentially to the brain surface. The fibers are very long and subdivide into many branches which form a network of pathways in the deeper cortical layers. There are numerous varicosities along the fibers and they come into close contact with other non-immunoreactive neuronal cells. The somatostatin cells located in the white matter are larger than the somatostatin cells in gray matter. They are giant cells with a size ranging from 50 to 120 micrometers. The fibers from these cells are varicose and can be followed both rostrally into the cortical gray matter and caudally in the subcortical white matter. The localization and the morphology of the somatostatin neurons in the cortex and the subcortical white matter indicate that somatostatin may be able to exert sustained influence in various brain areas and thereby modulate integrative and/or specific functions, not only via connection in the gray matter but also by influencing the neuronal circuits passing through the subcortical white matter.
The present study deals with the origin of somatostatin in cerebrospinal fluid. Two groups of experiments were performed: (1) Diagnostic lumbar puncture was performed in 37 patients admitted for various neurological diseases. Immunological determination of albumin and gamma-globulin, and radioimmunological analysis of somatostatin in successive cerebrospinal fluid taps demonstrated that while the protein concentration was approximately 20% lower in the 11th ml compared to the 1st ml drawn, the somatostatin concentration was constant. (2) Intravenous arginine infusion (30 G/30 min) induced identical patterns of plasma growth hormone in 8 patients with multiple sclerosis in relapse, in 6 patients with multiple sclerosis in the stable phase, and in 7 patients in whom no neurological disease was eventually diagnosed. Cerebrospinal fluid somatostatin was significantly lower in the patients with multiple sclerosis in relapse than in the two other groups, while cerebrospinal fluid growth hormone concentration was identical. There was no correlation between basal or arginine provoked plasma growth hormone and the cerebrospinal fluid content of somatostatin. The results indicate that cerebrospinal fluid somatostatin is released dispersely from the central nervous system including the spinal cord - and that it offers no indication of the activity or tone of hypothalamic growth hormone release inhibiting control of the pituitary gland.
In 27 patients with multiple sclerosis (MS), and in 10 control subjects of comparable age, percent ideal body weight and sex ratio, the cerebrospinal fluid (CSF) content of somatostatin was measured by radioimmunoassay. The results showed that the group of patients in relapse (n = 16) had significantly lower somatostatin content in CSF (95 +/- 4.1 (SEM) pg/ml) than both the control group (142 +/- 8.4 pg/ml) and the group of MS patients (n = 11), who had been in a clinical stable phase for more than 6 months (131 +/- 3.2 pg/ml). Duration of the disease and degree of neurological impairment were apparently without relation to the reduction of somatostatin content in the CSF. There was no relationship between CSF content of somatostatin and the content of total protein or IgG, neither of which showed any relationship to the activity of the disease.