Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030552 (paresis)
 5,831 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 65-year-old female had polyglucosan body myopathy, usually called "polysaccharide storage myopathy" that presented with increasing distal paresis and only slight weakness of the proximal limb girdle musculature. Muscle biopsy revealed dystrophic changes that could have been mistaken for muscular dystrophy, and the characteristic light as well as electron microscopic features of polyglucosan bodies varying in number at the three sites of muscle biopsies studied (deltoid, quadriceps femoris, and anterior tibial muscle). In addition, there were occasional nonspecific paracristalline mitochondrial inclusions. No abnormal polyglucosan deposits were found in the sural nerve biopsy. Morphometric evaluation of nerve fiber cross sectional areas revealed some degree of demyelination and remyelination, and of nerve fiber degeneration and regeneration. Unlike a series of 10 unselected control sural nerves with Renaut bodies, hypomyelinated nerve fibers were more numerous adjacent to Renaut bodies. This is the first case of polyglucosan body myopathy in which the axon/myelin ratio and the axonal circularity factor in the sural nerve is evaluated and in which a definite lack of polyglucosan bodies or other abnormal glycogen storage products in a peripheral nerve is documented.
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PMID:Adult polyglucosan body myopathy with subclinical peripheral neuropathy: case report and review of diseases associated with polyglucosan body accumulation. 285 83

The case of a 42-year-old man is reported, who on four occasions developed a hypokalaemic periodic paralysis after an intramuscular injection of "Ambene". The detailed examination of this patient shows, that it is the primary, autosomal dominant inherited form of hypokalaemic periodic paralysis, and not the secondary form, which is caused by a renal or gastrointestinal loss of potassium. Clinical and electrophysiological, as well as histopathological and electron microscopic findings are presented, showing the typical vacuolar myopathy with submicroscopic tubular structures. In the literature there is evidence for an increased sensitivity of the muscle membrane to insulin with an increased potassium-shift inside the cell in hypokalaemic periodic paralysis. "Ambene" is a combination, which contains amongst other substances dexamethasone and the local anaesthetic drug lidocain. In the present case the paresis was possibly caused by a combined effect of dexamethasone with a consequent hyperglycaemia and lidocain with a change in the excitability of the muscle membrane. The pathophysiological mechanism of hypokalaemic periodic paralysis is discussed in terms of the release by the combination of these two drugs. It has not previously been reported that "Ambene" can provoke a hypokalaemic periodic paralysis. This is a severe side effect because of the resulting cardiac and respiratory problems.
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PMID:[Hypokalemic periodic paralysis provoked by "Ambene"]. 293 67

The feeding of monensin as a coccidiostat in three separate flocks of turkeys was associated with increased mortality, posterior paresis, and a skeletal muscle myopathy. Mortality attributed to the disease was 1.65%, 1.86%, and 4.80% in the three flocks. Samples of monensin-supplemented feed fed to the flocks when showing clinical signs contained 88, 85, and 106 g/ton of complete feed, respectively. Clinically, the turkeys showed posterior paresis, inability to rise, incoordination, reluctance to move, and leg trembling and weakness. Necropsy findings included consistent lesions of pallor within the type I muscles of the legs, wings, and backs. Microscopic lesions included myofiber degeneration and necrosis with massive cellular proliferation interpreted as sarcolemmal nuclei proliferation. Occasional axonal degeneration with loss of axons was present in peripheral nerves embedded in the damaged musculature. In the youngest flock, multifocal areas of acute coagulation necrosis of the myocardium were also present. These outbreaks occurred following intake of monensin in the complete feed at levels considered therapeutic; however, no associated predisposing clinical condition, drug/toxin interaction, or excessive monensin levels in the feed could be demonstrated.
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PMID:Skeletal muscle lesions in turkeys associated with the feeding of monensin. 319 76

Partial cystectomy was performed for myogenic decompensated bladder with excessive residual urine in 11 patients, in whom training instructions and pharmacological treatment were unsuccessful. Postoperatively, the patients were followed for 2-13 years (median 4 years). Both symptoms and residual urine were reduced permanently. Urodynamic testing had demonstrated underactive detrusor function in all. The supposed etiology was infravesical obstruction in 4, overdistension due to sensory bladder paresis in 3 and unknown in 4. Histological examination of the resected bladders showed focal degeneration of the smooth muscle cells (detrusor myopathy) in 7, transmural edema and vast deposits of collagen in 8, mastocytosis in 3 and eosinophilic cystitis in 1. In conclusion we regard partial cystectomy an alternative to clean intermittent self-catheterization in selected patients with excessive residual urine.
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PMID:Partial cystectomy for the myogenic decompensated bladder with excessive residual urine. Urodynamics, histology and 2-13 years follow-up. 323 29

Eleven patients with Welander distal myopathy were subjected to detailed sensory testing including measurements of perception thresholds for vibration and temperature in both hands and feet. The threshold values were compared with normal, age-corrected values and also with data from an age-matched control group consisting of patients with antecedent poliomyelitis with the same degree of paresis. The screening examination indicated impaired thermal sensibility in all 11 patients and impairment for at least one other sensory modality in 9 patients. In comparison with age-corrected normal values, the measured warm-cold difference limen was abnormal in the feet of 9 patients and the vibratory threshold at least at one test point in 6 patients. When compared with the data from the paretic controls, the thermal abnormality was significant with regard to warm and cold thresholds, the warm-cold difference limen and the heat pain threshold. The vibration threshold abnormality was significant in the feet. It is concluded that sensibility impairment is present in Welander's myopathy indicating a peripheral sensory neuropathy involving both unmyelinated (C-fibers) and myelinated fibers.
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PMID:Sensory involvement in distal myopathy (Welander). 368 36

We describe a kindred with a rare autosomal dominant myopathy limited to the limb-girdle muscles, beginning insidiously any time from the late second through the sixth decades and followed by slow progression. Pelvifemoral precedes scapulohumeral weakness, and proximal appendicular involvement antedates limited distal paresis. Expressivity varies and includes an asymptomatic myopathy (preclinical or subclinical) and a nonmanifesting carrier state that extends well into the eighth decade. A variety of nonspecific changes are present in muscle on light, enzyme histochemical, and electron microscopic examination; of these changes, "rimmed" or autophagic vacuoles are the most characteristic. We identified one very similar previously reported genealogy. The similarities between the two unrelated families clearly establish this dystrophic process as a distinct genetic entity; their differences suggest genetic heterogeneity.
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PMID:Adult-onset autosomal dominant limb-girdle muscular dystrophy. 375 67

During the 3 weeks of his life, an infant born at term presented pronounced hypotonia, areflexia and generalized paresis with severe respiratory and feeding problems. He was the fourth male in two generations to die in the perinatal period, therefore suggesting an X-linked inheritance. Post-mortem examination revealed a centronuclear or myotubular myopathy. The difficulty in distinguishing the signs due to muscle disorder from those due to hypoxaemic encephalopathy is stressed. Infants with centronuclear myopathy have in any case a high risk for hypoxaemic encephalopathy. The literature concerning neonatal centronuclear myopathy with X-linked inheritance is reviewed.
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PMID:Neonatal myotubular myopathy with a probable X-linked inheritance: observations on a new family with a review of the literature. 651 77

The results of conventional strabismus surgery are always somewhat uncertain, despite the most careful preoperative assessment. Yet it is important to achieve good alignment in patients with fusion potential who have intermittent exotropia, extraocular muscle paresis or a restrictive syndrome. Planning surgery for patients with thyroid myopathy and planning reoperations is particularly difficult. In 30 patients with various strabismus disorders the use of adjustable sutures proved safe and of value; the results were unacceptable in only 5 of the 28 patients with horizontal deviations. The inconvenience for the surgeon and the extra discomfort for the patient seemed outweighed by the predictability of the surgical result.
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PMID:Adjustable sutures in strabismus surgery. 675 11

Motor neuron abnormalities have been implicated in the pathogenesis of Duchenne muscular dystrophy. Evidence concerning the effect of injury on motor neurons of human Duchenne muscular dystrophy (DMD) is lacking. We report a DMD patient having, in addition, an obstetric paresis on his left arm. EMG and muscle histochemistry showed signs of reinnervation superimposed on myopathy in his left arm. This suggests that sprouting is preserved in DMD motor neurons and that muscle fibers retain the capability of accepting reinnervation.
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PMID:Reinnervation in Duchenne muscular dystrophy. 686 9

Carnitine is a carrier for the transport of long-chain fatty acids from the cytoplasmic to the mitochondrial space. So far 18 cases of carnitine deficiency myopathy have been recognized, generally occurring as progressive or relapsing myopathy. In contrast episodic exercise-induced myalgia and paresis was found in a 46-year-old patient with carnitine deficiency myopathy which was quickly reversible at rest. As a consequence of this observation carnitine deficiency myopathy must be added to the differential diagnosis of the McArdle syndrome (muscular phosphorylase deficiency) and other "exercise myopathies".
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PMID:[Carnitine deficiency myopathy (author's transl)]. 736 6


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