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Biomedical subjects

A Culebras

Publications and source records attributed to A Culebras.

At least 55 records · Page 3Linked to original sources
PubMed1981

Coincidence of neurofibromatosis and myotonic dystrophy in a kindred.

Neurofibromatosis and myotonic dystrophy have occurred in ten members of a nonconsanguineous family with a high degree of concordance. The expression of neurofibromatosis is peripheral, and the expression of myotonic dystrophy has produced at least moderately severe disability. Neither disease has appeared to alter the phenotypic expression of the other when both have occurred simultaneously. Secretor typing supports the assumption that the myotonic dystrophy in this family is the commonly recognised secretor-linked entity. The segregation pattern of the two disorders in this family suggest the possibility of close linkage between the loci for neurofibromatosis and myotonic dystrophy.

Female↗
PubMed1979

Paget's disease and the nervous system.

Paget's disease usually is found in patients past the age of 40. Early presenting symptoms include headache, deafness, tinnitus, and pain due to radicular compression. The diagnosis is confirmed by radiographic features and elevated levels of serum alkaline phosphatase and urinary hydroxyproline. Bony overgrowth results in pressure on nearby soft tissues such as the brain, spinal cord, and certain peripheral nerves. The abnormally soft quality of the calvarial bone permits distortion by the weight of the brain. Dorsal inclination of the plane of the foramen magnum and the projection of the odontoid process into the posterior fossa lead to stretching of the brain stem over the odontoid process and the ventral margin of the foramen. Obstructive hydrocephalus may result. Sarcoma of the crainial vault may develop in cases of Paget's disease. Once cervicomedullary or spinal compression has occurred, surgical decompression may be necessary. Three drugs--calcitonin, disodium etidronate, and mithramycin--have been used with some benefit in the treatment of Paget's disease.

Adult↗
PubMed1977

Absence of sleep-related growth hormone elevations in myotonic dystrophy.

There is evidence that in myotonic dystrophy, the endocrine and central nervous systems are affected. To study a possible relationship between both defects, we investigated nocturnal sleep patterns and associated growth hormone secretion in two men and three women with myotonic dystrophy. In three patients who were clinically the most severely affected by myotonic dystrophy, plasma growth hormone elevations related to the slow-wave phase of sleep were absent. The two least severely affected patients had plasma growth hormone increases of low magnitude and brief duration (from 0.4 ng per milliliter to 13.0 ng per milliliter). These data suggest a failure of integration at a subcortical level of the slow-wave phase of sleep with the hypothalamic-pituitary mechanisms of growth hormone secretion. Thalamic neuronal lesions occurring in myotonic dystrophy could be responsible for such failure.

Adult↗
PubMed1976

Effect of papaverine on cerebral electrogenesis.

A double-blind, placebo-controlled study was conducted in 20 elderly patients with dementia associated with diffuse cerebrovascular disease to determine the effects of papaverine on the electrical activity of the brain. Frequency spectrum analysis of electroencephalograms showed the mean electrical content of the delta-theta band (0 to 7.5 Hz) was decreased significantly and content of the alpha band (7.6 to 12.5 Hz) was increased significantly from baseline. The changes in electroencephalographic activity observed in this study suggest that papaverine favorably affects neuronal metabolism, possibly through improved perfusion of the tissue, although the exact mechanism of action remains unknown.

Aged↗
PubMed1975

Cytoplasmic inclusion bodies in superior rectus muscle of the eye.

Cytoplasmic inclusion bodies are found in fibers of superior rectus muscle of the eye. They occur in middle-aged and old individuals dying with various unrelated diseases not affecting muscle and in patients with myotonic dystrophy. With light microscopy they can be observed in fibers with pathologic changes, and with electron microscopy they appear to be associated with crystals of probable viral origin. It is suggested that cytoplasmic inclusion bodies in superior rectus muscle are the result of viral activity and may represent an incidental viral infection of the senescent period.

Aged↗
PubMedDate not supplied

Effect of interictal epileptiform discharges on nocturnal plasma prolactin concentrations in epileptic patients with complex partial seizures.

To investigate the effect of interictal epileptiform discharges (IEDs) on plasma prolactin (PRL) level, we studied 18 epileptic patients with complex partial seizures (CPS) who did not experience clinical or subclinical ictal events during all-night monitoring with polygraphic recording and video imaging. The density of IEDs peaked during non-REM stages and was significantly reduced during REM stage. Mean plasma PRL concentrations in epileptic patients, when sampled at 30-min intervals, showed a moderate but significant elevation during non-REM (p less than 0.001) and awake stages (p less than 0.005), but not during REM stage, when compared with 10 nonepileptic control subjects studied in a similar fashion. The data obtained in this physiologically controlled environment indicate that the cumulative effect of IEDs may modify PRL regulatory mechanisms, resulting in a modest elevation of plasma concentrations independent of ictal discharges.

Adult↗