Search PubMed⌕ Search

Biomedical subjects

B M Patten

Publications and source records attributed to B M Patten.

At least 37 records · Page 2Linked to original sources
PubMed1984

Severe neurological disease associated with hyperparathyroidism.

Two patients, one with ataxia, internuclear ophthalmoplegia, muscle weakness, atrophy, fasciculations, and bilateral Babinski's signs, the other with dysarthria, dysphagia, muscle weakness, atrophy, fasciculations, and hyperreflexia, had elevated serum calcium and parathyroid hormone levels, establishing the diagnosis of primary hyperparathyroidism (HPT). Removal of a parathyroid adenoma in one patient and three hyperplastic parathyroid glands in the other resulted in remission of the hyperparathyroidism but left both patients with residual neurological damage. Postmortem examination of the second patient showed typical features of amyotrophic lateral sclerosis. The findings in these patients show that hyperparathyroidism may be associated with signs of severe central nervous system disease and that patients with unexplained neurological signs or symptoms should be checked for hyperparathyroidism.

Adenoma↗
PubMed1984

Creutzfeldt-Jakob Disease. Possible transmission to humans by consumption of wild animal brains.

Although the natural mode of spread of the agent responsible for Creutzfeldt-Jakob disease is unknown, several reports suggest oral transmission through consumption of contaminated food or brain. This report summarizes four cases of Creutzfeldt-Jakob disease in which a history of eating the brains of wild goat or squirrel was obtained. These cases support the hypothesis of possible acquisition of Creutzfeldt-Jakob disease by ingestion of the agent from a presumptive reservoir in the central nervous system of wild animals.

Aged↗
PubMed1984

Neuropathy and motor neuron syndromes associated with plasma cell disease.

Increasing numbers of patients are being recognized with neurological abnormalities associated with the immunochemical changes of plasma cell disease. To illustrate the wide spectrum of clinical disorders that can be found, I discuss in detail 5 patients: 2 with neuropathy, 3 with amyotrophic lateral sclerosis (ALS), all of whom had serum monoclonal paraproteinemia. In addition, I report in tabular form 6 patients with paraproteinemia and the following clinical presentations: 1) systemic lupus with polyneuropathy and severe cerebritis, 2) myasthenia gravis with thymoma, 3) polymyositis, 4) polymyositis, arthritis and Grave's disease, 5) relapsing polyneuritis (one of the original patients diagnosed by Austin) and 6) ALS, dystonia and parkinsonism. Major improvements in clinical condition occurred sometimes, but not always, coincident with reductions in the levels of the paraprotein using prednisone, cyclophosphamide, chlorambucil and plasma exchange treatments even in some of the patients who had the clinical appearance of ALS. Patients with neuromuscular diseases should be routinely screened with serum immunoelectrophoresis for monoclonal gammopathy. If a monoclonal gammopathy is found and if the disease is serious, then those patients should be treated as if they had an autoimmune disorder.

Adult↗
PubMed1983

Clinical trials of vasoactive and antiserotonin drugs in Duchenne muscular dystrophy.

In clinical trials of vasoactive and antiserotonin agents in Duchenne muscular dystrophy, we found that methysergide, 8 mg daily, was associated with an average 44% improvement in Gower Time (i.e., time to arise from supine) and an average 16% improvement in ward walking time. We then did a double blind crossover study of methysergide and found an average improvement of 33% in head holding time with methysergide compared to placebo. In other tests, including grip strength, ward walking time, and Gower Time, methysergide average scores always were better than placebo average scores, but never by more than 12%, and at no time did the differences reach statistical significance. There was no clinical or laboratory evidence of toxicity of methysergide. We conclude, that under the conditions of testing, there was no statistically significant evidence for short-term benefit from methysergide in Duchenne muscular dystrophy.

Child↗
PubMed1982

Morphologic and histochemical characteristics of laryngeal muscle.

Laryngeal muscle (LM) is highly specialized for phonation and sphincter activity. We queried whether this specialization is reflected in the structure of LM. We examined, using histochemical techniques, the structure of five LM from three men who died suddenly and who had no evidence of laryngeal disease. Compared with nonlaryngeal skeletal muscle, our specimens demonstrated moderate fibrosis, rounding of fibers, basophilia, and ragged red fibers that were shown to be mitochondria. In general, LM fibers are smaller, have more variability in size, and contain a greater percentage of histochemically type 1 fibers than limb skeletal muscles. These differences suggest that theories of motor control derived from studies of limb skeletal muscles may not apply to LM.

Adenosine Triphosphatases↗
PubMed1982

Free amino acid concentrations in spinal tissue from patients dying of motor neuron disease.

Because some investigators have reported abnormal concentrations of amino acids (AA) in fluids and tissues of patients with motor neuron disease (MND), we examined the AA content of frozen anterior horn spinal cord tissue taken from seven patients dying of MND and compared the results with those found in 12 control patients. Ammonia (21 +/- 8.1 vs. 12.7 +/- 6.9 mumol/g, P = 0.036) and ornithine (0.41 +/- 0.3 vs. 0.16 +/- 0.09, P = 0.036) were elevated in spinal tissue of motor neuron disease patients. Correlation analysis showed ammonia levels inversely related to duration of illness (r = -0.714, P = 0.036). We concluded that metabolic abnormalities exist in MND. Ammonia and ornithine may be adversely affecting motor neuron function, or alternatively they could be metabolic markers of a more generalized energy-deficient state in motor neuron disease.

Adult↗
PubMed1981

Repeat study of antecedent events in motor neuron disease.

Recently we applied case control methods to the investigation of motor neuron disease (MND) with surprising results: MND patients have greater exposure to lead and mercury and drink more milk than either diseased or normal age- and sex-matched control patients. Because of the implications of these findings as possible environmental influences in the pathogenesis of MND, we repeated the earlier study using a larger population and a more standardized interview technique. This time the MND patients reported more exposure to mercury or to the combination mercury or lead than either control group. As adults and at age 18, more MND patients drank in excess of three glasses of milk daily. We conclude that exposure to lead or mercury and excessive milk ingestion are possible antecedent events predisposing to MND.

Animals↗
PubMed1980

Muscle metals in motor neuron disease.

To investigate the role of metallic elements in motor neuron disease (MND), we used a photon-excited, energy-dispersive x-ray analytical system to measure the metal content of muscle biopsies from 21 patients with MND and compared the results with those obtained from biopsies from neurologically diseased control patients matched for age, sex, and muscle biopsied. By t test analysis, the MND patients did not differ significantly from controls in the average muscle biopsy concentrations of lead, mercury, arsenic, manganese, aluminum, or any of the other ten elements measured. Subgroup analysis showed that women with MND had less barium (p < 0.05) and iron (p < 0.01) in their muscles than controls, a finding probably an artifact of the multiple statistical analyses done on the data. Metallic content of muscle, for unknown reasons, tends to decrease with age in MND, but increases with age in controls. The evidence indicates that the muscle content of heavy metals in patients with MND does not differ from that in matched controls and that in MND, muscle does not store toxic metals for retrograde transport to anterior horn cells, as has been postulated by others.

Adolescent↗
PubMed1980

Limb preservation for reimplantation. a review.

Although reimplantation of severed limbs and other parts of the body has become prevalent in recent years, the questions of how best to preserve limbs for reimplantation and how to determine if a transected part is viable have not been fully answered. The problem of preservation involves combating direct anoxic damage to tissue as well as combating the changes in the vascular system that lead to the "no reflow phenomenon." Current information concerning kidney preservation as well as experimental and clinical reports on limb preservation are reviewed in this article, and suggestions are made for further investigations.

Animals↗
PubMed1980

Meige's syndrome.

We present two women with Meige's syndrome, a condition in which the clinical presentation differs from tardive dyskinesia by the lack of exposure to neuroleptic drgus, greater severity of blepharospasms, and more prolonged dystonic contractions of oromandibular muscles. In this condition we used triaxial accelerometry to detect dystonia, which may also appear in limb and respiratory muscles. Although psychologic factors may affect the symptoms, the basic pathogenesis of this syndrome does not seem to be psychogenic. We think that biochemical abnormalities in the basal ganglia are responsible for the dyskinesias and submit data suggesting a reduction of dopamine turnover in the central nervous system of one patient. Both patients have evidence of autoimmune diseases, and one patient's dystonic movements responded to immunosuppressive therapy, suggesting that autoimmune processes contribute to the pathogenic mechanism of Meige's syndrome in some instances.

Aged↗
PubMed1980

Disruption of the purine nucleotide cycle. A potential explanation for muscle dysfunction in myoadenylate deaminase deficiency.

A patient with symptoms of easy fatigability, postexercise myalgias, and delayed recovery of muscle strength after activity is described. Skeletal muscle from this patient had <1.0% normal myoadenylate deaminase activity and NH(3) was not released from muscle after ischemic exercise. In association with this enzyme deficiency, exercise led to a >90% reduction in muscle content of adenine nucleotides. No inosine monophosphate accumulated after exercise and total purine content of the muscle fell to 21% of control. Repletion of the adenine nucleotide pool in this patient was delayed compared to controls, and ATP content had only returned to 68% of control at 165 min after exercise. These studies demonstrate that disruption of the purine nucleotide cycle as a consequence of myoadenylate deaminase deficiency results in marked alterations in ATP content of muscle, and potentially, these changes in ATP content could account for muscle dysfunction in this patient.

AMP Deaminase↗
PubMed1979

Prednisone use in concurrent autoimmune diseases.

A 40-year-old woman with clinical and laboratory features of myasthenia gravis, hyperthyroidism, and polymyositis responded to treatment with prednisone alone. Symptoms of myasthenia gravis appeared first followed by hyperthyroid symptoms. Triiodothyronine, thyroxine, and thyroid uptake were elevated as were serum levels of CPK, SGOT, SGPT, and LDH. Muscle biopsy specimen showed mild type II fiber atrophy and a small focus of inflammatory cells. Two weeks after initiation of prednisone, 100 mg every other day, the ESR declined from 44 to 12 mm/hr, serum enzyme values became normal, and the weakness improved. Over the ensuing four months, the thyroid function values returned to normal and the patient no longer needed any anticholinesterase drug. At present, she is functionally normal except for mild defects in eye movement and she takes no medication. Physicians should consider treating patients who have several concurrent autoimmune diseases with prednisone to see if all conditions can be brought under control with one simple therapy.

Adult↗