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Biomedical subjects

B M Patten

Publications and source records attributed to B M Patten.

At least 55 records · Page 3Linked to original sources
PubMed1979

Histologic findings in motor neuron disease. Relation to clinically determined activity, duration, and severity of disease.

Correlation of 18 histologic variables with age and sex of 24 patients with motor neuron disease (MND), and the duration, severity, and activity of their disease, showed that high density of atrophic fibers correlated with degree of muscle weakness and the worst prognosis and that type I grouping correlated with the best prognosis. Although both type I and type II fibers are involved in the majority of patients with MND, the data suggest that involvement of type I fibers is more important in relation to activity of the disease.

Adult↗
PubMed1979

Metals in spinal cord tissue of patients dying of motor neuron disease.

To evaluate the role of toxic metals in causing motor neuron disease (MND), we used a photon-excited, energy-dispersive x-ray analytical system to measure the metal content of spinal ventral horn tissue. Specimens were taken from the cervical and lumbar enlargements of 7 patients who died of MND and the results compared with those found in 12 control patients. Anterior horn lead levels were elevated in MND patients compared to controls (mean, 40.7 micrograms/gm versus 14.6 micrograms/gm; p less than 0.05) and lead levels correlated with the duration of illness (r = +0.84, p less than 0.05). Only 2 MND patients had detectable manganese levels (72.3 and 132.2 micrograms/gm) whereas 1 control had detectable manganese (14.3 micrograms/gm). One MND patient had 244 micrograms/gm selenium, but 3 controls had levels of 180, 58, and 62. Patients with the histories of greatest environmental exposure to metals during life exhibited the highest tissue levels of metals after death; despite chelation therapy for about a year, high lead levels remained in their tissue.

Aged↗
PubMed1979

Familial recurrent rhabdomyolysis due to carnitine palmityl transferase deficiency.

Muscle carnitine palmityltransferase (CPT) activity was very low (0 to 14 per cent of controls) in two brothers with a syndrome of recurrent rhabdomyolysis and myoglobulinuria. In isolated muscle mitochondria the majority (87.5 per cent) of total measurable CPT enzyme activity could be attributed to external membrane CPT with severe deficiency of inner membrane CPT. By contrast, control mitochondria demonstrated a 1:1 distribution of external membrane CPT to inner membrane CPT. Thus, myoglobinuria may be due to a genetic defect of lipid metabolism in skeletal muscle, with inner membrane CPT deficiency presenting the same clinical features as external membrane CPT deficiency.

Acyltransferases↗
PubMed1979

Myasthenia gravis, thymectomy and serum thymic hormone activity.

Serum thymic hormone activity was measured in 36 patients with myasthenia gravis and in 10 control subjects from each age decade. In all 25 patients under 50 years of age results were within, or close to, the normal range. Activity at levels considered normal for juveniles was detected in 10 of the 11 older patients whereas levels normally decline in older subjects. One week after thymectomy, 13 of 17 patients (76 per cent) had no demonstrable serum thymic hormone activity. However, 10 months or longer after thymectomy only five patients (30 per cent) lacked thymic hormone activity in the serum. There was a significant correlation between clinial improvement and sustained lowering of serum thymic hormone activity after thymectomy.

Adolescent↗
PubMed1979

Dermatomyositis and female malignancy.

All cases of dermatomyositis-polymyositis in women seen between 1970 and 1977 at Baylor Affiliated Hospitals were reviewed. Twenty-five patients were identified with this diagnosis. Of these women, 5 were found to have a malignant tumor: 3 ovarian carcinomas, 1 cervical intraepithelial carcinoma, and 1 colon carcinoma. Pertinent histologic and clinical findings in patients with dermatomyositis-polymyositis are discussed. The high prevalence of malignancy in these patients, previously recognized, is discussed. A case report is presented.

Adult↗
PubMed1978

Free amino acid levels in amyotrophic lateral sclerosis.

To evaluate reports of abnormal levels of free amino acids (AA) in patients with amyotrophic lateral sclerosis (ALS), we studied serum, cerebrospinal fluid, and urine AA in 12 patients with ALS and 12 controls matched for age, sex, and severity of disability. ALS patients had statistically significant elevations in serum levels of tyrosine, total aromatic AA, and total basic AA. ALS patients also had statistically significant elevations in cerebrospinal fluid of total basic AA, lysine, essential AA, and leucine. The severity of ALS correlated inversely with acidic AA (glutamate and aspartate) and O-phosphoserine in cerebrospinal fluid. Activity of ALS correlated directly with serum aspartate and cerebrospinal fluid alanine. We conclude that subtle abnormalities of AA levels are present in ALS and that these are not due to age, sex, or disability. The pattern of distribution of AA levels differs from that in hepatic or renal disease and suggests defective membrane transport or poor cellular utilization of basic and essential AA in the central nervous system.

Adult↗
PubMed1978

Neuromyopathy in malignant hyperthermia.

A 31-year old male developed malignant hyperthermia (MH) during the initial minutes of Halothane induction. CPK rose to 6120 U/ml and decreased to normal levels as the patient became afebrile over a 10 day period of cooling measures and metabolic management. Muscle weakness, predominantly proximal and depressed deep tendon reflexes were found upon examination during convalescence. Muscle biopsy showed neurogenic changes characterized by fiber type grouping and targetoid fibers. CPK was elevated in one of the patient's children. This case supports the view of underlying hereditary neuromyopathy in MH.

Adult↗
PubMed1978

Effects of guanadrel on patients with thyrotoxicosis.

Eleven patients with Graves' disease were treated with guanadrel sulfate and observed for changes in neuromuscular and cardiovascular manifestations. No notable changes in pulse rate or muscle strength were detected in either these patients during a three-day pretreatment period or in five control patients with Graves' disease receiving placebo for six days. Thyroid hormone levels were not altered by seven days of guanadrel sulfate therapy (5 to 20 mg orally every six hours), and no adverse side effects were encountered. Mean supine resting pulse fell from 102 +/- 6 (mean +/- SEM) to 90 +/- 3 beats per minute (P less than .02). The patients' proximal and distal muscle strengths were initially decreased, when compared with healthy subjects, and improved substantially with guanadrel therapy. We conclude that guanadrel sulfate may be useful in the symptomatic management of patients with thyrotoxicosis.

Adult↗
PubMed1977

Catecholamine-induced muscle weakness.

Infusions of epinephrine or levarterenol bitartrate into a rabbit nerve-muscle preparation decreased the force of the evoked twitch of anterior tibial and gastrocnemius-soleus muscles. The adverse effect of the catecholamines was not directly on skeletal muscle. The alpha-receptor blocking drug phenoxybenzamine hydrochloride prevented the adverse effect of the catecholamines if it was given prior to catecholamine infusions and unmasked a weak augmentation of twitch tension. Taken with the finding of abnormal accumulation of catecholamine in human dystrophic muscles, the production of an experimental myopathy resembling human dystrophy by the monoamine oxidase inhibitor pargyline hydrochloride, and the finding of excessive levels of catecholamines in the tissues and urine of dystrophic animals, these experiments support the hypothesis that catecholamines could play a pathogenetic role in some dystrophic diseases of muscle.

Action Potentials↗
PubMed1977

Mitochondrial abnormalities in progressive external ophthalmoplegia.

Three cases of chronic progressive external ophthalmoplegia demonstrated ragged-red fibers. Histochemical stains showed that the ragged-red fiber appearance was produced by abnormal mitochondria in the muscle. Electron microscopic studies demonstrated the ultrastructural origin of the intramitochondrial inclusions seen in ragged-red fibers. The associated abnormalities in lactic acid metabolism possibly represent an underlying generalized defect in metabolism. This evidence suggests that chronic progressive external ophthalmoplegia is a diffuse disease of the mitochondria.

Adult↗
PubMed1977

Hepatitis-associated lipid storage myopathy.

We report the case of a 23-year-old woman who developed severe myalgias in association with mild hepatitis B surface antigen-positive hepatitis. Muscle biopsy showed myriads of microvacuoles filled with neutral lipid. Prednisone therapy was associated with complete clearing of all clinical and laboratory abnormalities. Since muscle carnitine levels were normal before treatment, we conclude that infection with hepatitis virus may induce non-carnitine-deficient lipid storage myopathy in man.

Adult↗
PubMed1976

Antecedent events in amyotrophic lateral sclerosis.

To identify antecedent events contributing to the development of amyotrophic lateral sclerosis, we studied 25 amyotrophic lateral sclerosis patients in whom we tabulated the incidence of factors previously associated with motor neuron disease and compared the incidences with those found in 25 hospitalized patients and 25 normal people. More amyotrophic lateral sclerosis patients reported exposure to lead and mercury, participation in athletics, and consumption of large quantities of milk. Exposure to lead and mercury, athletic participation, and milk ingestion are possible risk factors that may predispose to the development of amyotrophic lateral sclerosis.

Adult↗
PubMed1976

Motor neuron disease: retrospective study of associated abnormalities.

In a retrospective study of the charts of 39 patients who had motor neuron disease we found that over 50 percent of patients had radiographic abnormalities of bone and over 20 percent had serum calcium concentrations out of the range observed in normal controls. Statistical analysis indicated that, in respect to serum calcium levels, patients who have motor neuron disease are a separate population. These findings, which might reflect a disturbance in bone or calcium metabolism in patients with motor neuron disease, are of interest in view of the known ability of divalent ions other than calcium, such as lead and mercury, to simulate motor neuron disease, and the discovery that patients with proven primary and secondary hyperparathyroidism may have features of amyotrophic lateral sclerosis.

Adolescent↗
PubMed1976

Mitochondrial abnormalities in progressive ophthalmoplegia.

A clinically diagnosed case of progressive external ophthalmoplegia was biopsied and ultrastructurally evaluated. The disease was found to be characterized by numerous giant mitochondria. The intramitochondrial changes consisted of either swirls of cristae or of paracrystalline structures which were in apposition with the membranes of the cristae. The internal structure of the paracrystalline units consisted of parallel bands of material which were aligned in the same plane as the membranes of the related cristae.

Humans↗