1/37. Dominant-inherited hypokalemic periodic paralysis in a large Chinese family.Familial hypokalemic periodic paralysis (HoPP) is a rare condition among Chinese. We studied a large Chinese family (48 members in six generations) with dominant-inherited HoPP, using incidental tracing of family history of a proband who presented with the typical features of HoPP. Fifteen family members were found to have the disease. We found the familial type of HoPP to differ from the sporadic type in taiwan, in that the familial type has an equal gender distribution, earlier onset of paralytic attacks, and more severe clinical features in both frequency and extent. When these patients were compared with Caucasian families, the common features were the involvement of the respiratory and the bulbar muscles, and the eye muscles in Chinese patients. Cold-induced attacks and permanent muscle weakness were not common in Chinese subjects. Age and history of paralytic attacks were not the major determinants for the development of permanent muscle weakness. Two family members died during attacks because of severe involvement of the respiratory and bulbar muscles.- - - - - - - - - - ranking = 1keywords = hypokalemic (Clic here for more details about this article) |
2/37. Thyrotoxic hypokalemic periodic paralysis case report and review of the literature.A rare complication of hyperthyroidism consists of sudden diffuse muscle weakness associated with severe hypokalemia. The clinical presentation is similar in most respects to familial periodic paralysis; however, the therapies proven to be effective differ in the two syndromes. A case of thyrotoxic hypokalemic periodic paralysis is presented and the literature is reviewed.- - - - - - - - - - ranking = 1keywords = hypokalemic (Clic here for more details about this article) |
3/37. Progressive spinal muscular atrophy in a case of hypokalamic periodic paralysis.A 47-year-old man with well-documented hypokalemic periodic paralysis demons-rated permanent myopathy as shown by biopsy. In addition there was clinical and biopsy evidence of progressive spinal muscular atrophy. The feature of the combination of these diseases is reported in a case and discussed.- - - - - - - - - - ranking = 0.2keywords = hypokalemic (Clic here for more details about this article) |
4/37. Modifications in the sarcoplasmic reticulum and subcellular calcium distribution in skeletal muscle in a case of Westphal's disease (hypokalemic periodic paralysis).In a case of hypokalemic periodic paralysis with characteristic alterations of the sarcoplasmic reticulum (SR) in the skeletal muscle, subcellular calcium re-partition, as revealed with the pyroantimonate technique, appears disturbed during paralysis. Pyroantimonate precipitates, normally concentrated in the terminal cisternae of the SR, were localized in the T tubules, whereas the terminal cisternae appeared empty. The increase (about 14%) in muscular calcium during paralysis may result from the accumulation of calcium in the extracellular compartment (T tubules). Defects in calcium uptake and storage by the SR may be involved in the pathogenesis of the periodic paralysis syndrome.- - - - - - - - - - ranking = 1keywords = hypokalemic (Clic here for more details about this article) |
5/37. Expression of heat shock protein epitopes in tubular aggregates.Tubular aggregates may be found in a variety of conditions and have been associated with a wide range of chemical and ischemic insults. We report clinical and histological features in a case of myopathy with tubular aggregates. The structure of these tubular aggregates was examined using antibodies to cytoskeletal proteins and heat shock proteins. epitopes of the 72 kD heat shock protein were expressed in the areas of abnormality in this case and in a case of hypokalemic periodic paralysis with tubular aggregates. Heat shock proteins have a role in the modulation of the tertiary structure of proteins and may be involved in the pathogenesis of tubular aggregates and other microtubular abnormalities in muscle.- - - - - - - - - - ranking = 0.2keywords = hypokalemic (Clic here for more details about this article) |
6/37. atracurium use in a patient with familial periodic paralysis.We describe a patient with the hypokalemic type of familial periodic paralysis (FPP) who received atracurium for muscle relaxation as required for diagnostic laparoscopy. Electrocardiographic (EKG) T-wave changes suggestive of hypokalemia were not supported by blood determinations. Arterial blood measurements of potassium (K ), pH, and arterial carbon dioxide tension (PaCO2) and the patient's esophageal temperature were maintained within normal limits. The degree of muscle relaxation was closely monitored by a peripheral nerve stimulator and train-of-four (TOF) measurement of muscle twitch height. At the conclusion of the surgical procedure, no reversal to the muscle relaxant was needed or given. The patient regained preoperative muscle strength, and her postoperative course was uneventful.- - - - - - - - - - ranking = 0.2keywords = hypokalemic (Clic here for more details about this article) |
7/37. calcium-tension relationships of muscle fibers from patients with periodic paralysis.Lateral gastrocnemius muscle biopsies from a 26-year-old man with hyperkalemic periodic paralysis and a 23-year-old man with hypokalemic periodic paralysis were studied. Both patients came from families in which older relatives had developed a vacuolar myopathy in association with their periodic paralysis. Muscle fibers were chemically skinned, and individual fibers were studied with a low-compliance strain gauge. The tension generated by fibers was studied in baths with calcium concentrations from 10(-8) mol/L to 2.5 x 10(-5) mol/L. The Ca-tension relationships and maximal tensions (normalized to fiber cross-sectional area) of fast and slow twitch fibers were indistinguishable from those found in fibers from 5 normal subjects. The results reinforce earlier findings which suggested that loss of Ca-induced myofibril contraction was not the cause of paralysis in periodic paralysis.- - - - - - - - - - ranking = 0.2keywords = hypokalemic (Clic here for more details about this article) |
8/37. Potential role of acute hypophosphatemia during hypokalemic periodic paralysis attack.A paralysis attack was induced by glucose load in a patient with hypokalemic periodic paralysis. A profound drop in serum phosphorus was observed (from 3.0 to 0.8 mg/dl) in parallel to the serum potassium decrease. The potential role of phosphorus metabolism in the pathophysiology of muscle weakness in this disease is discussed.- - - - - - - - - - ranking = 1keywords = hypokalemic (Clic here for more details about this article) |
9/37. Thyrotoxic periodic paralysis and the sodium/potassium pump.The hypothesis of altered Na /K transport in thyrotoxic periodic paralysis (TPP) was tested in an investigation of the K influx into erythrocytes from two patients with episodes of thyrotoxic muscle weakness. A patient with primary hypokalemic periodic paralysis (HPP) and three healthy volunteers served as controls. The TPP patients were of Oriental and Caucasian origin and differed in their clinical symptoms. For the Caucasian patient, the Na content of the erythrocytes was twice the control, for the Oriental patient it was normal. The K dependence of the ouabain-inhibitable K influx (the pump action) was also abnormal in the Caucasian patient, the flux being 70% of control at 2 mM [K ]e and normal at 4 mM [K ]e. The K influx was normal in the Oriental patient. By contrast, the K leak of the cells was normal in the Caucasian and was increased in the Oriental patient. The pump/leak ratio was thus reduced in both TPP patients. All parameters investigated were normal in the patient with primary HPP. It is concluded that the ion transport systems of muscle may be altered in TPP, but that the patho-mechanism might be different in the rare Caucasian cases and the rather more common Oriental cases.- - - - - - - - - - ranking = 0.2keywords = hypokalemic (Clic here for more details about this article) |
10/37. Hypokalaemic, hypophosphatemic thyrotoxic periodic paralysis.Thyrotoxic periodic paralysis is an uncommon neuromuscular disorder frequently associated with severe hypokalemia. We describe a patient with hypophosphatemia occurring in the setting of hypokalemic thyrotoxic periodic paralysis, an association reported only once previously. A review of the literature indicates that this combined biochemical derangement may not be uncommon and that thyrotoxic periodic paralysis should be recognized as a potential cause of hypophosphatemia. The correction of both hypokalemia and hypophosphatemia may lead to a more rapid resolution of the associated acute neurologic syndrome.- - - - - - - - - - ranking = 0.2keywords = hypokalemic (Clic here for more details about this article) |
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