Showing papers on "Amyotrophic lateral sclerosis published in 1983"

Morphometric and biochemical studies of peripheral nerves in amyotrophic lateral sclerosis

Abstract: Phrenic nerves of 11 patients with amyotrophic lateral sclerosis studied postmortem contained only 33% of the normal number of large myelinated fibers (9 controls; p less than 0.001). In the phrenic nerves of these patients, there were 18% fewer large myelinated fibers in the distal segment than in the proximal segment (p less than 0.025). The ratio of axonal circumference to myelin lamellae in large myelinated fibers in the distal segment was 34% greater than that in control fibers (p less than 0.002). The proportion of acute axonal degeneration was the same at all levels (48.0 +/- 13.7%). Sural nerves of 21 patients with amyotrophic lateral sclerosis had more acute axonal degeneration and 30% fewer myelinated fibers (p less than 0.05) than controls; evidence of degeneration also extended to unmyelinated fibers. The amount of axonal transport of acetylcholinesterase in 9 sural nerves determined in vitro was reduced by 24% (p less than 0.05) and the apparent transport rate was reduced by 44% (p less than 0.01) compared with 4 controls. These findings show that in amyotrophic lateral sclerosis a small degree of dying-back change and of distal axonal atrophy is superimposed on the degeneration of motor neuron cell bodies, and that the disease effects spread beyond the motor neurons.

Amyotrophic lateral sclerosis: Alterations in neurotransmitter receptors

Abstract: Loss of motor neurons is the primary pathological hallmark of amyotrophic lateral sclerosis. Drug and neurotransmitter receptors are neuronal markers and can be indicators of neuronal connectivity. Knowledge of alterations in receptors in amyotrophic lateral sclerosis should contribute to our understanding of normal spinal cord neurotransmitter systems as well as of the pathophysiology of amyotrophic lateral sclerosis. We therefore used a sensitive, light microscopic in vitro labeling receptor autoradiographic technique to map and quantitate muscarinic cholinergic, glycinergic, and benzodiazepine receptors in three levels of spinal cord from six patients with amyotrophic lateral sclerosis and six age- and sex-matched control patients. In control tissues, the receptor distributions were similar in the three levels of spinal cord and also similar to those found in previous studies with animals. In amyotrophic lateral sclerosis, major reductions in receptor densities were noted in Rexed layer IX, the region containing motor neurons. Reductions were noted in other laminae as well, particularly for muscarinic receptors. The changes in muscarinic receptors were caused solely by changes in high-affinity agonist sites. Reductions in glycine and muscarinic receptors were highly correlated with the degree of motor neuron loss found in the amyotrophic lateral sclerosis patients. The findings in this study point out the usefulness of this receptor mapping technique in understanding the changes in neuronal populations that occur in the degenerative neurological diseases.

Syndromes of amyotrophic lateral sclerosis and dementia: Relation to transmissible Creutzfeldt-Jakob disease

Abstract: A review of over 2,000 cases of Creutzfeldt-Jakob disease and related disorders in the literature and our own files yielded 231 cases of dementia with early lower motor neuron signs. The clinical-pathological profiles of the 231 cases were distinctly different from those of cases of transmissible Creutzfeldt-Jakob disease: the patients had a longer illness, and their brains lacked the typical spongiform change. Brain tissue from 33 of these patients has been inoculated intracerebrally into nonhuman primates, but only 2 atypical cases transmitted a spongiform encephalopathy; 23 have been incubating from three to twelve years and can be considered negative transmission experiments. The findings suggest that most cases of dementia associated with early amyotrophy are more closely related to classic amyotrophic lateral sclerosis than to transmissible Creutzfeldt-Jakob disease and do not deserve the label of "amyotrophic Creutzfeldt-Jakob disease." When lower motor neuron involvement occurs in transmissible Creutzfeldt-Jakob disease, it is usually late and accompanied by signs of a more fulminant cerebral and cerebellar involvement.

Mercury intoxication simulating amyotrophic lateral sclerosis.

Abstract: A 54-year-old man had a syndrome resembling amyotrophic lateral sclerosis after a brief but intense exposure to elemental mercury. The syndrome resolved as his urinary mercury levels fell. Mercury toxicity must be considered not only in individuals with recent anterior horn-cell dysfunction but also with otherwise unexplained peripheral neuropathy, tremor, ataxia, and a gamut of psychiatric symptoms including confusion and depression. (JAMA1983;250:642-643)

Amyotrophic lateral sclerosis in Middle-Finland: an epidemiological study.

Abstract: Amyotrophic lateral sclerosis (ALS) was diagnosed in 36 patients in Middle-Finland Central Hospital District during 1976–1981. The annual incidence of ALS was 2.4 per 100,000 population and the prevalence rate was 6.4 per 100,000 population. The age-specific incidences of ALS were similar for men and women with a maximum of 14/100,000/year in the age group 60–69 years. The initial symptoms originated in 37% of the patients from bulbar and in 63% from spinal levels. Bulbar onset was more common in patients aged 60 years or more compared with younger patients. Patients with bulbar onset had a significantly poorer prognosis than those with spinal onset, which explained the poorer prognosis of older patients. 4 matched controls were chosen for each ALS patient from the files of the Central Hospital. There was no difference between the patients and the controls with respect to previous injuries, surgical operations, malignant neoplasms, or exposure to domestic animals. An earlier observation that evacuees from Karelia ceded to USSR after World War II should have a prevalence twice that of the remaining population was not substantiated.

Nutritional considerations in the management of patients with amyotrophic lateral sclerosis (ALS).

Abstract: This study examines the nutritional status of patients with ALS based on data derived from a dietary history, anthropometric measurements, and biochemical assessment. Twenty patients, 11 men and 9 women, were studied to determine nutritional status in order to differentiate appropriate therapy and monitor prognosis. The study suggests that nutritional support of these patients may help allay weight loss and retard muscle atrophy. Nutritional management involves (a) early detection and correction of inadequate nutrient intake, particularly of kilocalories; (b) concurrent modification in consistency of food intake with development of bulbar involvement; and (c) determination of optimal time to institute alternate feeding routes in cases of bulbar involvement.

Plasmapheresis with immunosuppression in amyotrophic lateral sclerosis.

Abstract: • Four patients with amyotrophic lateral sclerosis and one with progressive muscular atrophy were treated with large-volume plasmapheresis combined with immunosuppression. Assessment of muscle strength and functional ability was performed during a period of time ranging from 6.2 to 13 months. Patients were compared with a clinically matched control group. Both treated and untreated patients continued to show similar deterioration. The results of this study failed to show any therapeutic benefit of plasmapheresis with immunosuppression in patients with amyotrophic lateral sclerosis.

Eye movements in amyotrophic lateral sclerosis.

Abstract: It has generally been assumed that the oculomotor system is not involved in amyotrophic lateral sclerosis (ALS). However, reports from the literature and recent experience with patients indicate that there are oculomotor abnormalities in some ALS patients. These appear to fall into two main categories. One group of patients has signs that reflect damage or degeneration in cortico-oculomotor pathways. These patients have problems in generating voluntary saccades, convergence and pursuit eye movements. It should be possible to corroborate deficits in ocular pursuit in these patients with deficits in visual suppression of the vestibulo-ocular reflex (VOR) and in optokinetic nystagmus (OKN). A second group of patients, much less common than the first, has a more global type of ophthalmoplegia, probably reflecting a loss of neurons in and around the ocular motor nuclei. There are probably also patients with mixed types of involvement. Testing the vestibulo-ocular reflex (VOR) in conjunction with other tests of visual-oculomotor function should help in identifying the deficits. The relative incidence of the various types of deficits is not known and is a subject for study. It would also be of interest to correlate the changes that are present in the oculomotor system of patients with ALS with deficits in other parts of the somatic motor system.

Search for a red cell enzyme or serum protein marker in amyotrophic lateral sclerosis and parkinsonism‐dementia of guam

Abstract: Frequency distributions were determined for 24 red cell enzyme and four serum protein systems, in an attempt to identify a genetic marker associated with either amyotrophic lateral sclerosis (ALS) or parkinsonism-dementia (PD), two progressive and fatal neurological disorders of unknown cause found with unusually high incidence among the Chamorros of Guam and the Northern Mariana Islands. No striking associations were identified between either disorder and any of the gene markers tested. Thus, no genetic cause is known for either disease; local environmental factors are most likely involved in pathogenesis.

Chronic Pain Syndrome in Amyotrophic Lateral Sclerosis

Abstract: Amyotrophic lateral sclerosis (ALS) is classically a disease of motor neurons and has clinical manifestations involving the motor system, although cramps may occur. 1 Paresthesias are frequently described, particularly at the onset of weakness and muscular atrophy. 2 Chronic pain has not been reported in this disorder, to my knowledge. Motor neuron disease may be associated with dementia, parkinsonism, or other neurologic disorders, 3 and pathologic involvement extending beyond the motor system has been reported, particularly in the posterior column pathways. 4 This report concerns a patient with clinically and electromyographically typical ALS in whom a concomitant chronic central pain syndrome developed, for which no additional cause could be found. REPORT OF A CASE Weakness, atrophy, and fasciculations of the left lower extremity developed in a 54-year-old woman in 1979. In 1980 she underwent lumbar laminectomy without relief of symptoms. Weakness, wasting, and fasciculations subsequently progressed in the left

Amyotrophic lateral sclerosis. Lack of central chromatolytic response of motor neurocytons corresponding to active axonal degeneration.

Abstract: • Ventral spinal roots and anterior horn cells in the lateral nuclear group of the fourth lumbar segment from 21 patients with amyotrophic lateral sclerosis (ALS) and 23 control patients were morphometrically analyzed. The number of large myelinated fibers was remarkably decreased, while small myelinated fibers were well preserved. The population of large myelinated fibers significantly correlated with the population of anterior horn cells. Numerous axonal degenerations were observed in the ventral spinal roots of patients with ALS, even in patients with severe loss of neurons and axons. In spite of this high frequency of active axonal degeneration, the incidence of central chromatolysis of anterior horn cells remained at the control level.

Occurrence of reduced alpha 2-macroglobulin and lowered protease inhibiting capacity in plasma of amyotrophic lateral sclerosis patients.

Abstract: Lowered levels of plasma alpha 2-macroglobulin were found in 13 patients with amyotrophic lateral sclerosis using anti-alpha 2-macroglobulin-embedded agar plates. Levels of this major protease inhibitor in ALS patients were contrasted with those in disease controls, consisting of patients with a variety of neurologic disorders, as well as with those in normal individuals. The patients' levels, in milligrams per deciliter, were 191 +/- 39 (SD). The disease controls had levels of 269 +/- 33. Local normal values compared with the reported normal values in the literature (260 +/- 70). A correlation was not made for either the form of motor neuron disease, disease severity, or age. As a measure of functional protease inhibition, trypsin inhibitory capacity of sera was estimated using a radioactive fibrinolytic assay. An increase in the half-maximal inhibitory concentration of sera was found in the amyotrophic lateral sclerosis group. These findings likely relate to the known increases in proteolytic activity in denervated muscle and collagenolytic activity in skin of patients with amyotrophic lateral sclerosis. The determination of whether altered protease inhibitory potential in such patients is primary or secondary may help in understanding the pathogenesis of this enigmatic disorder and provide suggestions for future approaches to intervention.

Ceruloplasmin and copper in the serum of patients with amyotrophic lateral sclerosis (ALS)

Abstract: The serum level of ceruloplasmin and copper were determined in 14 patients with ALS, 9 with Wilson's disease and 10 with other brain diseases. The enzyme level in 8 patients with ALS (57%) was decreased, similarly as in 8 with Wilson's disease (89%), and 2 (20%) in the control group. The mean ceruloplasmin level in the group of patients with Wilson's disease was 50% that in ALS patients. The copper level was decreased in only 1 ALS patient and 1 in the control group, while in patients with Wilson's disease it was low in 8 cases. These changes may be an effect as well as a cause of motor neuron disease.

A clinico-pathological study of chronic hereditary motor neuropathy.

Abstract: Forty cases of chronic hereditary motor neuropathy (CHMN) were divided into five categories according to the distribution of muscle atrophy; they were proximal, facioscapulohumeral, bulbospinal, distal and scapuloperoneal forms. Their clinical features and laboratory data were analysed, and muscle biopsies from 32 of them were studied by histological, histochemical and electron microscopical methods. An attempt at quantitative assessment of the histological changes was also made. All muscle biopsies showed a mixture of neurogenic and ‘myopathic’ changes in varying proportions. They showed more ‘myopathic’ changes than Werdnig-Hoffmann's disease, amyotrophic lateral sclerosis and other neurogenic atrophies except Charcot-Marie-Tooth disease. There was marked variation in the average number of atrophied muscle fibers contained in grouped atrophy. Frequently, deranged internal structure of the muscle fibres was revealed both by histochemical and electron microscopical methods. Based on the evidence of heterogeneity of CHMN in respect of genetics, clinical features and histological changes, it was speculated that CHMN represents a group of diseases which involves primarily different parts of the motor units.

Electronystagmography in amyotrophic lateral sclerosis.

Abstract: To the Editor. —We are glad that several authors 1-4 have recently reported eye-movement disorders in patients with amyotrophic lateral sclerosis (ALS), modifying the classic teaching that oculorotary functions are spared in ALS. It is possible that our 1974 report has not been cited because it appeared in The Laryngoscope , 5 although presentation was made at the Eighth International Congress of Electroencephalography and Clinical Neurophysiology in 1973 6 and the study was mentioned later in a neurology review. 7 Briefly, electronystagmography (ENG) was applied to patients with several motor neuron diseases (MND) (mainly, of course, ALS). The analysis by 1973 5,6 included 88 patients with MND, 66 of whom we considered to suffer from classic ALS. About two thirds underwent neurologic reevaluation after three months, and fewer after 12 and 24 months, so that the original diagnoses could be reviewed and the clinical courses assessed under our personal observation. There

ALS and parkinsonian syndromes among the Auyu and Jakai.

Abstract: To the Editor: The remarkable report of Gajdusek and Salazarl on the occurrence of foci of high incidence and prevalence of amyotrophic lateral sclerosis, parkinsonism-dementia, and poliomyeloradiculitis among certain primitive people of West New Guinea but not in adjacent areas with the same apparent ecology, culture, and diet is of considerable interest. This situation suggests three different possible hypotheses: (a) There is something that is present or absent in the affected areas that is not found in the unaffected areas (an environmental factor). (b) The people in the affected areas are different (a genetic factor). (c) The people in the affected areas do something that is different (a cultural factor). The authors reject genetic and cultural factors as possible explanations but their reasons for doing this were not explained fully. In particular, the social customs and mores were not given in sufficient detail to discern how much interchange of goods and people there is between different villages; what are the mating, marriage and child-rearing customs; whether there is intermarriage between people from affected and unaffected areas; what is the fate of villagers from an affected area who move to an unaffected area; is food prepared in the same way, is hygiene similar, are death rites identical, etc. Further clarification of these details would be helpful. It is also unclear why the mineral content of the water and soil of three affected villages that are within 10 kilometers of each other are compared with water and soil in the USA rather than with unaffected villages.