Bernard M. Patten

Neuromuscular Disease in Primary Hyperparathyroidism

Abstract Fourteen of 16 patients with primary hyperparathyroidism studied prospectively had weakness, easy fatiguability, and atrophy of muscles—particularly of the lower extremities. Electromyogra...

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.

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.

Hyperparathyroidism: Recent Studies

Abstract A review of 57 cases of primary hyperparathyroidism has delineated the changing clinical complex of the disease; its apparent prevalence increases with greater clinical awareness and more ...

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.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.

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.

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.

Neuropathy and motor neuron syndromes associated with plasma cell disease.

Abstract– 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 Graves disease, 5) relapsing polyneuritis (one of the original patients diagnosed by Austin) and 6) ALS, dystonia and parkinsonism.

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.

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 μmol/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.