The transmission of ciguatera toxicity: another first isn't.
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Biomedical subjects
Publications and source records attributed to T R Swift.
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A 33-year-old woman developed headaches, papilledema, and evanescent neurologic signs. MRI demonstrated thrombosis of the sagittal, transverse, and sigmoid sinuses. After initial heparinization and three months of oral anticoagulation therapy, papilledema was still present but the neurologic signs and symptoms had markedly improved. Repeat MRI scan revealed improvement in all dural thromboses.
One hundred four normal subjects ranging in age from 17 to 77 years and in height from 115 to 203 cm underwent nerve conduction studies of sural, peroneal, tibial, and median nerves. Foot temperature was measured in each patient. A strong inverse correlation was found between height and sural (r = -0.7104), peroneal (r = -0.6842), and tibial (r = -0.5044) conduction velocities. These correlations were significant at the P less than 0.001 level. Median conduction velocity was not correlated with height. Height was correlated with the distal latencies of all nerves studied (sural r = 0.6518, peroneal r = 0.4583, tibial r = 0.7217, median r = 0.5440). These correlations were significant at the P less than 0.001 level. Age was inversely correlated with both tibial (r = -0.4071) and median (r = -0.3464) nerve conduction velocities but not with sural and peroneal conductions. There were no correlations between distal latencies and age. If the variation in conduction velocity accounted for by the linear relationship with height was removed, then age would be inversely correlated to all conduction velocity measurements with the exception of the sural. Temperature is inversely correlated with the sural (r = -0.2233), peroneal (r = -0.2102), and tibial (r = -0.2710) distal latencies. In all instances, the effects of age and temperature were minor determinants when compared with the effects of height. Diagnostic conclusions made from nerve conduction data without correcting for height may be invalid in patients taller and shorter than normal.(ABSTRACT TRUNCATED AT 250 WORDS)
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A 25-year-old woman with Kearns-Sayre syndrome (KSS) had complete external ophthalmoplegia, short stature, ataxia, cardiac conduction defects, and pigmentary retinopathy. Muscle biopsy revealed ragged-red fibers. Electron microscopy showed increased numbers of mitochondria with disordered structure and paracrystalline inclusions. Enzymatic analysis revealed a deficiency of complex II of the mitochondrial respiratory chain, and, more specifically, a deficiency of succinic dehydrogenase, although both subunits of this enzyme proved to be present by immunologic analysis. Therapy with vitamin cofactors did not result in short-term improvement. This appears to be the first report of complex II deficiency in a patient with KSS.
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We studied the fate of skeletal muscle obtained from patients with amyotrophic lateral sclerosis (ALS) after transplantation into immunodeficient nude mice. The transplanted muscle consistently survived in the nude mice without immunological rejection. The myofibers in these muscles underwent degeneration, followed by regeneration, maturation, and eventual functional innervation by the mouse motor neurons. The ability to grow diseased human muscle successfully over a prolonged period in nude mice offers an in vivo model to study the etiology of ALS and possibly of other neuromuscular disorders.
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Four workers were exposed to ethylene oxide gas. Acute encephalopathy occurred in one, and peripheral neuropathy in three, two of whom were symptomatic. Nerve conduction velocity studies were abnormal in three, including the asymptomatic patient. Decreasing the amount of exposure resulted in relief of symptoms. The abnormal nerve conduction studies did not change in two patients who continued to work at a lower level of exposure, whereas the conduction abnormalities improved in the patient who was removed from exposure.
A female infant with neonatal myasthenia gravis remained weak for an excessively long period compared to the usual situation in this disease process. At 71 days of age, electrophysiological studies revealed evidence of a defect in neuromuscular transmission that improved with intravenous Tensilon therapy. Repeat study at 183 days of age was normal. This case represents an unusually long time for an affected infant to have neonatal myasthenia gravis. Electrophysiological studies are of value in the diagnosis and management of patients with neonatal myasthenia gravis.
Clinical, electrophysiological, and laboratory data are reported on a 21-year-old black woman with systemic lupus erythematosus, myasthenia gravis, and Ehlers-Danlos syndrome. The first two diseases are related by similar pathogenetic mechanisms. To our knowledge, this is the first patient reported with this unusual combination of rare diseases.
This paper reports the findings on two patients with McArdle syndrome (myophosphorylase deficiency) in whom conventional bone scans with Tc-99m pyrophosphate revealed intense muscle labeling following exercise tests. The temporal pattern observed was similar to that seen with other types of muscle damage. The prolonged cramps often occurring with this entity appears to produce muscle damage that is readily demonstrable using conventional bone-scanning techniques.
Two adult patients had painful enlargement of the shoulder and complete destruction of the humeral head. Angiograms of the shoulder showed highly vascular lesions in both patients. Both patients had syringomyelia, for which the swollen shoulder was the first manifestation.
A 20-year-old man with confirmed Hunter syndrome had entrapments of median and ulnar nerves. Sural nerve biopsy specimen revealed clear, lamellated, and granular inclusions in Schwann cells, fibroblasts, and perineurial cells. The clear inclusions are believed to represent storage of mucopolysaccharide, and the zebra bodies, ganglioside. Focal alterations in myelin were found, associated with moderately dense granular inclusions. Following carpal tunnel release operations, the patient experienced dramatic releif of symptoms.
Fourteen whole-body rectilinear bone scans using technetium 99m-polyphosphate were done in nine patients with well-documented inflammatory myopathy (either polymyositis or dermatomyositis). In all nine patients, the scans showed evidence of increased muscle labeling. Muscle uptake was markedly increased in one patient, moderately increased in two patients, and minimally increased in six patients. The degree of muscle labeling correlated with the severity of the muscle weakness at the time the scan was done. In four patients, who received high-dose corticosteroid treatment, muscle uptake was decreased following therapy. These findings suggest that radioisotope scanning may be useful in the diagnosis and management of patients with inflammatory muscle diseases.
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