Neuromuscular diseases of domestic animals: a summary of muscle biopsies from 159 cases.
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[Epidemiology of neuromuscular diseases in Macedonia].
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Adolescent↗
Clinical interpretations of the measurement of folic acid and vitamin B12 in neuromuscular disease.
56 patients were studied in an attempt to define neurologic complications associated with folic acid and vitamin B12 deficiency. 2 patients had abnormal levels of vitamin B12. 1 of these showed decreased vibRATORY SENSE IN THE LEGS. 10 of 15 patients with serum folate less than 4.0 ng per ml and 16 of 23 patients with serum folate less than 6.0 ng per ml by radioassay had neurologic abnormalities. The control group demonstrated 17/31 with neurologic abnormalities, a figure not significantly different from the deficient group. It is suggested that more patients with "pure folate deficiency" must be analyzed to eliminate the possible role of toxic effects of alcohol and other diseases of the nervous system in patients with low serum folate and neurologic problems.
Adolescent↗
[Systolic time intervals of the left ventricle in neuromuscular diseases].
In 64 patients having neuromuscular disease and in 11 healthy persons, preejection period (PEP), ejection time index (LVETI) and PEP/LVET ratio of the left ventricle were studied by indirect method. PEP was significantly longer, ejection time index was significantly shorter and PEP/LVET ratio was significantly higher in patients with progressive muscular dystrophy than healthy persons. In 75% of patients having this disease LVETI was shortened and only in 30% of them Ecg abnormalities could be detected. These findings are postulated to be due to diffusely decreased contractility of the left ventricular heart muscle in progressive muscular dystrophy.
Adult↗
Stress in families of children with neuromuscular disease.
Compared parents of children with neuromuscular disease to parents of children with psychiatric diagnoses using the Questionnaire on Resources and Stress. The groups showed different patterns of stress, with three of the subscales correctly classifying 93% of the cases in a discriminant function analysis. Parents of neuromuscular cases were more pessimistic, while those of psychiatric cases reported more problems in family integration and in the child's personality. For the neuromuscular group alone, parents whose children were in wheelchairs scored significantly higher than those whose children were ambulatory on six QRS scales: Excess Time Demands, Overcommitment/Martyrdom, Limits on Family Opportunity, Physical Incapacitation for Index Case, Lack of Activities for Index Case, Occupation Limitations. Hence, not only does the type of personal and family stress differ for the Neuromuscular and Child Psychiatry respondents, but within the neuromuscular sample the increase in stress as the disease progresses is related to higher scores on QRS scales.
Child↗
Neuromuscular disease in secondary hyperparathyroidism.
Neuromuscular function was evaluated in six patients with osteomalacia or secondary hyperparathyroidism, or both, as demonstrated by bone biopsy showing osteomalacia or increased immunoreactive parathyroid hormone, or both. Each patient had weakness, atrophy, and fatigability of proximal muscles, especially of the lower extremities. Most also showed involuntary fine movements of the tongue, hyperactive tendon reflexes with abnormal spread, and decreased vibration sensation, abnormalities similar to those observed in primary hyperparathyroidism. Every patient studies had evidence of neuropathic muscle disease, either on electromyography or muscle biopsy studies histochemically or both. Muscle biopsies showed no definite myopathic features. Treatment of the osteomalacia improved muscle strength. Patients with osteomalacia therefore have a treatable neuromuscular disease that is neuropathic in nature and resembles closely that found in primary hyperparathyroidism.
Adolescent↗
Creatine kinase isoenzymes in neuromuscular diseases.
Determination of the creatine kinase isoenzyme pattern in 62 biopsy samples obtained from patients with neuromuscular disease revealed changes mainly in Duchenne muscular dystrophy. The BB isoenzyme was detected in 10 out of 17 cases with Duchenne muscular dystrophy and the relative amount of MB+BB isoenzyme was significantly increased in this group (P less than 0.005). In serum the MB isoenzyme was detected in all 28 cases with progressive muscular dystrophy and frequently also in other neuromuscular diseases. Among 152 control samples the MB isoenzyme was detected only in 2 cases. It is suggested that the finding of MB isoenzyme in the serum with normal or only slightly elevated total CK activity may be a further proof of neuromuscular disorder, but the finding is not specific for any particular disease.
Adult↗
The significance of the estimation of serum myoglobin in neuromuscular diseases.
The serum myoglobin concentration was determined and compared with the serum creatine kinase activity in 230 patients suffering from various neuromuscular diseases. No correlation was found between the two levels. In general serum creatine kinase activity estimation seemed more sensitive for the detection of neuromuscular disease than serum myoglobin estimation. In myotonic dystrophy, however, determination of serum myoglobin was distinctly the more sensitive method.
Adolescent↗
Respiratory failure in neuromuscular disease. Management in a respiratory intensive care unit.
Patients with neuromuscular disease frequently experience acute respiratory failure. Most require endotracheal intubation or tracheostomy and mechanical ventilation because of paralysis and inability to maintain adequate spontaneous respiration. The prognosis is usually excellent if ventilatory management is successful.
Acute Disease↗
Freeze-fracture studies of erythrocyte plasma membrane in human neuromuscular diseases.
Freeze-fracture studies were conducted in erythrocyte plasma membrane from 8 patients with Duchenne muscular dystrophy (DMD), 8 age-matched controls, 3 adult controls, 10 patients with myotonic muscular dystrophy, and 26 other neuromuscular disease controls. There was marked depletion of intramembranous particles in Duchenne dystrophy, whereas intramembranous particle density counts in other neuromuscular diseases were within normal limits. Therefore, the internal molecular architecture of the erythrocyte membrane is abnormal in Duchenne dystrophy, supporting the concept that a membrane defect involving multiple tissues is present in this disorder.
Erythrocyte Membrane↗
3-Methylhistidine excretion as an index of myofibrillar protein catabolism in neuromuscular disease.
Myofibrillar protein catabolism has been calculated in a variety of neuromuscular diseases from the amount of 3-methylhistidine excreted in the urine. It was found to be significantly raised in Duchenne type muscular dystrophy, motor neurone disease, polymyositis, and thyrotoxic myopathy. In Becker type muscular dystrophy the level was slightly raised. It was normal in scapuloperoneal and limb girdle dystrophy, dystrophia myotonica, extrapyramidal disease, and multiple sclerosis. It was significantly decreased in hypothyroid myopathy.
Histidine↗
Intercostal muscle biopsy in human neuromuscular disease. Histochemical and electron microscopic studies.
External intercostal muscle biopsies were examined histochemically and by electron microscopy. The use of this muscle allowed correlation with physiological and pharmacological studies on the same specimens. Changes observed in musclar dystrophy and motor neurone disease resembled those previously described in biopsied limb muscle and underline the particular usefulness of this preparation in the study of human neuromuscular disease.
Adenosine Triphosphatases↗
Morphologic and metabolic studies in a case of oculo-cranio-somatic neuromuscular disease.
A case resembling the syndrome of "ophthalmoplegia plus" or "oculo-cranio-somatic neuromuscular disease" is reported. A biopsy of deltoid muscle showed that 23% of the fibers were "ragged-red fibers" and were all type 1. Study of their ultrastructure revealed clusters of abnormal skeletal muscle mitochondria in subsarcolemmal and intermyofibrillar spaces. A liver biopsy also revealed a considerable increase in the number and size of the mitochondria. In some instances the mitochondria contained osmiophilic rounded inclusions surrounded by myelin-like structures. Metabolic studies revealed an increase of blood lactate concentration after very light exercise, while the O2 consumption was increased within the expected range. It is concluded that: a) the association of ophthalmoplegia and ultrastructural alterations of the mitochondria in muscle fibers may represent a specific nosographic entity: b) mitochondrial abnormalities are not limited to the skeletal muscles and c) the dysmetabolic basis of such a clinico-pathological entity might lie in an alteration of the mechanism which regulates the mitochondrial oxidative phosphorylation.
Adult↗
Pyruvate kinase: diagnostic value in neuromuscular disease.
Pyruvate kinase activity was examined in the sera of a group of patients with neuromuscular disease and in carriers, and compared with that of creatine kinase. The following observations were made: 1. Pyruvate kinase activity was elevated in all 14 patients with Duchenne muscular dystrophy, with very high values generally correlating inversely with age or disease duration. Elevated values of pyruvate kinase were usually, but not invariably, associated with elevated values of creatine kinase. 2. Almost all patients with other muscle diseases and those with neural atrophy had modest elevations in pyruvate kinase activity. 3. ten of 17 individuals were identified as carriers of muscle disease by using both pyruvate kinase and creatine kinase while eight and nine, respectively, were detected using either assay alone. 4. When frozen stored EDTA-plasma was used for pyruvate kinase estimation, higher levels, as compared with the corresponding sera or fresh plasma, were found in controls and carriers but not in Duchenne muscular dystrophy patients. Frozen stored EDTA-plasma should, therefore, not be used for diagnostic purposes.
Adolescent↗
Leukoencephalopathy in oculocraniosomatic neuromuscular disease with ragged-red fibers. Mitochondrial abnormalities demonstrated by computerized tomography.
In a group of 13 patients affected by oculocraniosomatic neuromuscular disease with mitochondrial abnormalities and ragged-red fibers, a major, diffuse leukoencephalopathic process was recognized by computerized tomography (CT) in three cases. Clinically, these three patients were the most severely affected. The areas involved by the leukoencephalopathic process did not show enhancement after contrast medium injection. In several of the remaining ten cases, various forms and grades of CSF cavity dilation were encountered. Four of the 13 patients had very high CSF protein levels, three had CT-demonstrated leukoencephalopathy, and the fourth had substantial sulcal dilation. The CT findings in this group of patients corresponded well to the previously reported histopathological observations and indicated the clinical usefulness of CT in identifying brain involvement in this syndrome.
Adolescent↗
Muscle fiber hypotrophy with intact neuromuscular junctions. A study of a patient with congenital neuromuscular disease and ophthalmoplegia.
An infant born with severe but nonprogressive somatic and cranial muscle weakness including bilateral external ophthalmoplegia was studied with a motor-point muscle biopsy. There was a strinking generalized decrease in the size of muscle fibers (hypotrophy), most marked in the type I fibers. Many of the small fibers were immature, resembling myotubes. Neuromuscular junctions on severely hypotrophic fibers were normal with esterase staining and by ultrastructural criteria. Although these are unusual clinical and biopsy characteristics, this infant's condition bears a resemblance to two other congenital nonprogressive neuromuscular diseases:myotubular myopathy and congenital fiber type disproportion. In these conditions and in our patient, there is no primary degenerative process affecting nerve or muscle but, rather, an apparent lack of maturation of fetal muscle fibers, indicating a defective normal trophic interaction between nerve and muscle.
Biopsy↗
Inside-of-the-Body test drawings performed by patients with neuromuscular diseases: a preliminary report.
Inside-of-the-Body test drawings were obtained from 50 individuals with various neuromuscular diseases. A mean of 18.0 +/- 5.1 body parts were identified in the drawings. Diseased body structures were emphasized by most patients; for example, thymus was only drawn by individuals with myasthenia gravis, while muscle was only identified by individuals with polymyositis. In contrast, drawings by individuals with neuropathic atrophy omitted the atrophic extremities.
Adult↗
Bicycle ergometry and gas exchange measurements in neuromuscular diseases.
Using bicycle ergometry with computerized respiratory gas exchange measurements, we compared exercise capacities in patients with various neuromuscular diseases to those in normal controls. As expected, male and female patients had significantly reduced maximum work capacities (kilopond-meters per minute per kilogram of body weight) and maximal oxygen consumptions. The oxygen cost of exercise was normal in the majority of patients, although some appeared to have abnormally high oxygen consumptions during exercise. Breathing patterns during exercise, particularly in regard to onset of hyperventilation, were similar in patients and controls.
Adolescent↗