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Biomedical subjects

W Nix

Publications and source records attributed to W Nix.

31 records · Page 2Linked to original sources

The anal sphincter in patients with myotonic muscular dystrophy.

The objective of this prospective study was to determine anal sphincter function and thickness of the anal musculature in patients with myotonic muscular dystrophy. Manometric studies were performed in 16 patients with myotonic dystrophy and in 16 healthy controls. Patients had significantly lower basal and squeeze pressures than control subjects (P less than 0.01). The results of ultrasonographic studies of the anal canal in 7 patients and 7 control subjects suggest that this decrease in muscle strength is partly explained by muscular atrophy. In addition, patients with myotonic dystrophy showed exaggerated rebound contractions following and sphincter relaxation that was induced by rectal distention. The pattern of this response and the results of electromyographic studies in 6 patients with myotonic dystrophy suggest that such abnormalities are explained by a neurogenic defect rather than a myotonic response of the anal musculature. It is concluded that patients with myotonic dystrophy show a multitude of defects in the anal sphincter that are an expression of myopathy, muscular atrophy, and neural abnormalities.

Adult↗

Internalized myofiber capillaries: observations on their origin and clinical features.

Internalized capillaries limited to type 1 muscle fibers were noted in seven patients. They occurred in each case in association with a similar admixture of neurogenic and myopathic features that included atrophic and hypertrophic fibers, internal nuclei, fiber splitting, and endomyseal and perimyseal fibrosis. Internalized capillaries in enlarged type 1 fibers arose from fiber splits on step section study of four patients. They occurred in the gastrocnemius, quadriceps, and soleus muscles from patients with a variety of disorders that included Becker dystrophy, diabetes mellitus and strenuous leg activities, Achilles tendon rupture, and myotonic dystrophy. Exercise-induced myalgias were noted in the four patients with the most plentiful intramuscular capillaries, and in three of these muscle hypertrophy was present. The concurrence of internalized myofiber capillaries and exercise-induced myalgias may represent an associated biochemical/pathological defect.

Adolescent↗

Esophageal motor function in patients with muscular dystrophy.

In a study designed to evaluate esophageal motor function in muscular dystrophy we examined 13 patients with myotonic dystrophy, 14 patients with "nonmyotonic" muscular dystrophy, and 8 healthy control subjects by manometric and radionuclide transit studies. Patients with myotonic dystrophy exhibited a marked weakness of esophageal contractions and upper esophageal sphincter pressure. Coordination of sphincter relaxation and peristaltic sequences remained unaltered. These changes led to delayed esophageal emptying in all patients with myotonic dystrophy. Although esophageal function was also impaired in the distal esophagus, on histologic studies, morphologic alterations were confined to esophageal striated muscle in a single patient with myotonic dystrophy. In contrast to the marked dysfunction of esophageal motility in patients with myotonia, no such alterations were observed in the "nonmyotonic" form of muscular dystrophy.

Adult↗

Rare association of herpes simplex virus IgM-specific antibodies and Guillain-Barré syndrome successfully treated with plasma exchange and immunosuppression.

Herpes simplex virus (HSV) has been associated with various neurological disorders. In contrast, HSV infection is very rarely found in acute polyneuroradiculitis. In this report, a patient is described with a severe course of Guillain-Barr¿ syndrome (GBS). HSV IgM-specific antibodies and a rise of complement-fixation antibodies were detected. During the acute phase of neurologic syndrome, a nerve biopsy showed myelin damage and IgM deposits on the inner layer of the perineurium. Plasma exchange, in combination with immunosuppression, was successfully applied as a treatment in the relapsing course of GBS. Finally, after recovery, HSV-specific IgM antibodies disappeared.

Aged↗

Computed tomography of the "armored brain".

A calcified chronic subdural hematoma may cover the surface of the cerebral hemispheres to such an extent that one can talk of an "armored brain". Pathogenesis, clinical course and treatment are discussed based on the computed tomograms of five cases.

Adolescent↗

Multiple sclerosis patients show an increased spontaneous activity of their peripheral blood monocytes as measured by chemiluminescence.

I has been reported that myelin basic protein (BP) reacts extremely sensitively to peroxide, which is formed when monocytes/macrophages are stimulated to produce a "respiratory burst" (RB). We measured the RB activity by means of chemiluminescence in peripheral blood monocytes/macrophages (MO) of 17 MS patients, 5 patients with a viral infection of the CNS, and 14 control persons. The median of the spontaneous RB activity of MS patients compared with the median of our control group showed a highly significant increase (P = 0.0002). All MS patients examined possessed a clearly increased MO activity. The highest values, however, were found in MS patients in a bout (means = 315%, means = 296%). Since a viral infection is discussed as being involved in the pathogenesis of MS, we also examined patients with a viral infection of the CNS, but this group of patients did not show a significant increase (P = 0.34). Our data indicate that MO in MS patients generate and/or secrete an increased amount of unspecific mediators of inflammation. The possibility is discussed that this altered MO function might be caused by an altered reaction of MO in MS patients to a viral infection or superinfection.

Central Nervous System Diseases↗

Comparison of vascular reactivity in spinal cord and brain.

The local tissue PO2 in the brain cortex and in the spinal cord of rats was examined with ultramicroelectrodes. In the spinal cord the PO2 was highest in white matter, intermediate in dorsal horn gray matter, and lowest in the ventral horn gray matter. In the gray matter of the cord, as well as in the brain, the PO2 at a fixed locus was found normally to oscillate. CO2 responses were more brisk in the cord than in the brain while the responses to hypoxia were similar. Therefore, it appears that the physiological regulation of blood flow in the spinal cord is qualitatively similar to that of the brain.

Animals↗

Morphologic cerebral changes in patients undergoing dialysis for renal failure.

Cranial computed tomographic examinations were performed on 22 patients before and after hemodialysis, and on 11 of those patients 8 months later. In 11 cases widening of the cerebrospinal fluid spaces due to brain atrophy was recognizable. Density measurements disclosed decreased absorption of cerebral parenchyma after dialysis in 73% of the cases. This finding is partly explained by cerebral edema or hydration, although the necessary contemporaneous narrowing of cerebrospinal fluid spaces existed in only six patients (27%). Long-term follow-up studies have not displayed any realizable changes in results. Finally, despite striking computed tomographic findings and alterations after dialysis, only mild neurologic symptoms or disequilibrium syndromes have been registered.

Adult↗