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Biomedical subjects

B M Patten

Publications and source records attributed to B M Patten.

At least 19 recordsLinked to original sources
PubMed1992

Structure and specificities of anti-ganglioside autoantibodies associated with motor neuropathies.

Autoantibodies that bind to GM1 ganglioside and asialo GM1 (GA1) have been implicated in the pathogenesis of motor neuropathies. To investigate the structure and specificity of these autoantibodies, peripheral blood B cells from patients with motor neuron diseases and from normal individuals were immortalized by EBV, and B cells secreting anti-GM1 or GA1 antibodies were cloned. We report an analysis of the structure and specificities of eight autoantibodies from patients with motor neuropathy, and two from normal individuals. Four antibodies were IgM, six were IgG, and all bound predominantly to GA1. The sequences of V domains of H and L chains were determined by a reverse transcription-polymerase chain reaction procedure. A variety of V genes were used to encode these antibodies: four VH1, two VH3, three VH4, one VH5, two V kappa I, two V kappa II, three V kappa III, and two V lambda II. Most V genes (13/19) exhibited less than 95% similarity to known germ-line genes, which suggests that somatic mutation was required to generate these autoantibodies, or that the relevant germ-line genes have not been identified. The average length of the H chain CDR3 was 16 amino acids, and in three antibodies this segment contained more than 20 amino acids. It was not possible to identify amino acid sequences that were encoded by germ-line D segments by conventional alignment of sequences. Partial analogies could be identified by introducing gaps, allowing mismatches and searching for D-D fusions and inversions. These results indicate that anti-GA1 antibodies can be encoded by a variety of VH-VL pairs, that the antibodies exhibit extensive somatic mutation, and that the CDR3 segments are generated by a number of nonconventional mechanisms.

Adult↗
PubMed1992

Lightning and electrical injuries.

Lightning and electrical injuries are similar in that both produce immediate tissue injury from burn and trauma induced by fall and both can arrest the heart and respiratory center. Immediate support of circulation and respiration is life-saving. Subsequently the nervous system may show signs of injury, and seizures, cerebral edema, and muscle and nerve lesions should be handled as the indications arise. Prevention of the injury is more effective than any postinjury treatment. Outdoors hikers and campers must take shelter to minimize their exposure; indoors properly installed equipment and attention to the relation of the equipment user to the electrical ground are the key elements in avoiding electrocution.

Electric Injuries↗
PubMed1992

Neurology of microgravity and space travel.

Exposure to microgravity and space travel produce several neurologic changes, including SAS, ataxia, postural disturbances, perceptual illusions, neuromuscular weakness, and fatigue. Inflight SAS, perceptual illusions, and ocular changes are of more importance. After landing, however, ataxia, perceptual illusions, neuromuscular weakness, and fatigue play greater roles in astronaut health and readaptation to a terrestrial environment. Cardiovascular adjustments to microgravity, bone demineralization, and possible decompression sickness and excessive radiation exposure contribute further to medical problems of astronauts in space. A better understanding of the mechanisms by which microgravity adversely affects the nervous system and more effective treatments will provide healthier, happier, and longer stays in space on the space station Freedom and during the mission to Mars.

Adaptation, Physiological↗
PubMed1991

Clinical correlations of anti-GM1 antibodies in amyotrophic lateral sclerosis and neuropathies.

Clinical correlations of antiganglioside GM1 antibodies are important because high titers of these antibodies may have therapeutic significance. To further evaluate this significance, we reviewed our experience with 78 patients who had the following diagnoses: amyotrophic lateral sclerosis (ALS), ALS syndromes in patients with gammopathy or thyroid abnormalities, cervical spondylosis simulating ALS, motor neuropathies, and chronic inflammatory demyelinating polyneuropathies (CIDP). Antiganglioside antibody titers were measured "blind" by ELISA assay at the neuromuscular clinical laboratory, Johns Hopkins School of Medicine. We conclude that anti-GM1 antibodies are found in a wide variety of neuromuscular conditions. Patients with classical ALS had a mean anti-GM1 antibody titer significantly lower than patients with CIDP or motor neuropathy. Patients with ALS associated with gammopathy or thyroid disorders had higher anti-GM1 titers than seen in classical ALS. The highest mean titer occurred in patients with CIDP, a treatable neuropathy.

Amyotrophic Lateral Sclerosis↗
PubMed1990

The history of memory arts.

Ancient humans, lacking devices to store large amounts of information, invented and developed a system of mnemonics which evolved and passed to modern times. The mnemonics, collectively known as the Ancient Art of Memory, were discovered in 447 BC by a Greek poet, Simonides, and were adequately described by Cicero, Quintilian, and Pliny. These arts fell into neglect after Alaric sacked Rome in 410 AD, but were subsequently revived in 1323 by Saint Thomas Aquinas, who transferred them from a division of rhetoric to ethics and used them to recall Catholic doctrine and versions of biblical history. In 1540 Saint Ignatius Loyola used mnemonic images to affirm the faith with his newly formed Society of Jesus and tried to convert the Ming dynasty in China by teaching these memory skills to Chinese nobles. Today, the ancient memory arts have applications in pilot training, gambling, mentalism and telepathy demonstrations, and may have a role in the rehabilitation of brain-damaged patients. Objective testing confirms that with the use of these memory skills, recall is increased, at least 10-fold, and the memory deficits of proactive and retroactive inhibition do not exist.

History, 19th Century↗
PubMed1989

Amyotrophic lateral sclerosis: abnormalities of the tongue on magnetic resonance imaging.

We compared the magnetic resonance images of the tongues of 16 amyotrophic lateral sclerosis (ALS) patients with those of 20 control patients and found the tongue in ALS patients is more frequently and more severely involved than suspected clinically, with major abnormalities of size, shape, position, and internal structure. The tongue size in ALS, as measured in the sagittal plane, can be reduced by as much as two-thirds of normal. The shape of the tongue in ALS tends to be rectangular or square rather than curved as is normal. As severity of the disease increases, the position of the tongue changes so that the bulk of the muscle falls away from the incisors and no longer is in contact with the hard or soft palate. The normal radial bands from the anterior floor of the mouth to the mucosal surface are often missing in ALS as are the two curvilinear bands that run parallel to the mucosal surface and intersect the radial bands. Also, there is a mottled disorganization of the internal structure of the tongue with areas of increased and decreased signal intensity.

Adolescent↗
PubMed1989

Abnormal systemic metabolism of iron, porphyrin, and calcium in Fahr's syndrome.

Striopallidodentate calcinosis (Fahr's disease) is characterized clinically by seizures, rigidity, and dementia and pathologically by mineral deposition in the basal ganglia, dentate nucleus, and cerebral cortex. Disorders of iron and calcium-phosphate metabolism are thought to play a role in its pathogenesis. We present the case of a patient with familial striopallidodentate calcinosis who had porphyria cutanea tarda, refractory anemia, and pseudohypoparathyroidism type 2. The serum level of ferritin was markedly increased, serum iron and iron-binding capacity were below normal, and at autopsy she had deposition of iron in liver, spleen, bone marrow, and brain. She showed intermittent mild hypocalcemia, increased serum values of parathyroid hormone, elevated renal tubular reabsorption of phosphate, and low serum levels of 1,25-dihydroxyvitamin D, suggesting blunted renal responsiveness to endogenous parathyroid hormone. Pseudohypoparathyroidism type 2 was confirmed by infusion of synthetic parathyroid hormone, which gave a normal urinary cyclic adenosine monophosphate response, but a blunted phosphaturic response. After splenectomy for hypersplenism and weekly phlebotomies, she showed progressive improvement in function, mental status, weight, and seizure control. The hypothesis advanced is that the underlying pathophysiology of the separate diseases contributed to the formation of the brain stones through mechanisms of defective iron transport and free radical production.

Adult↗
PubMed1987

Blepharospasm and autoimmune diseases.

We studied two patients, one with systemic lupus erythematosus and the other with myasthenia gravis, both of whom had coexistent blepharospasm. The blepharospasm occurred during exacerbations of the autoimmune diseases and improved with immunosuppressive therapy. These cases illustrate the possible association of blepharospasm and autoimmune disorders.

Adult↗
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↗
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↗