Showing papers on "Amyotrophic lateral sclerosis published in 1989"
TL;DR: The amyotrophic lateral sclerosis severity scale has been shown to have an average estimated reliability coefficient of 0.95 between examiners and speech ratings were correlated greater than 0.80 for objective speech measures.
Abstract: The amyotrophic lateral sclerosis (ALS) severity scale has been developed to provide an ordinal staging system and a means of rapid functional assessment for patients with ALS. The scale allows an exa
200 citations
TL;DR: Successive observation of the 4 cases of amyotrophic lateral sclerosis in the totally locked-in state confirmed that ontogenetically old motor functions, including oculomotor functions, were preserved in the advanced stage, and that the main lesions in the oculumotor system were supranuclear.
181 citations
TL;DR: Central motor conduction to abductor digiti minimi was evaluated in 22 patients with motor neurone disease using magnetic stimulation of the motor cortex and electrical stimulation at the C7/T1 interspace, revealing subclinical upper motor neur one involvement and document central motor pathway dysfunction in MND.
114 citations
TL;DR: The general patterns of progression of bulbar ALS are outlined and the development of symptoms are correlated with specific treatment recommendations to aid the clinician in devising an orderly plan of management for this progressive disease.
Abstract: Patients with bulbar amyotrophic lateral sclerosis (ALS) are often referred to the otolaryngologist/head and neck surgeon and speech pathologist for evaluation and management of dysphagia and dysarthria. These patients comprise an unusual group because of the progressive and multi-system nature of their illness. The neuromuscular disabilities associated with bulbar ALS cause a myriad of related symptoms associated with swallowing, speech, and respiration. Although the rate of progression cannot be predicted, a general pattern of progression is noted. Bulbar disease accounts for the majority of the worst symptoms of ALS. The loss of the ability to swallow changes eating from a pleasurable task to a burden of survival. Loss of communication effectively imprisons the patient in a state of isolation. The progressive weakness of respiration, predominantly a spinal rather than bulbar manifestation, is the cause of death for nearly all ALS patients and is also discussed. The general patterns of progression of bulbar ALS are outlined in this paper. The development of symptoms are correlated with specific treatment recommendations to aid the clinician in devising an orderly plan of management for this progressive disease.
103 citations
TL;DR: There is a strong relationship between serum antiganglioside antibodies and patterns of clinical involvement in ALS, and there is further evidence of ongoing autoimmune processes in ALS patients.
Abstract: We studied the incidence and clinical correlates of serum antibodies to GM1 and GD1a gangliosides in patients with classical amyotrophic lateral sclerosis (ALS) and other "motor nerve" syndromes. Serum antibodies to GM1 and GD1a gangliosides were measured using enzyme-linked immunosorbent assays. Our results showed that polyclonal immunoglobulin M (IgM) antibodies to the GM1 or GD1a ganglioside or both were present at serum dilutions of 1:25 to 1:4,000 in 78% (57/73) of patients with ALS. Only 8% of normal controls had similar antibodies. The pattern of serum antibody reactivity correlated with the pattern of clinical involvement in our patients. Selective reactivity to GD1a ganglioside was common when upper motor neuron signs were prominent. IgM reactivity to GM1 ganglioside was common in ALS patients with prominent lower motor neuron signs. Most patients with motor neuropathies had serum reactivity to both GM1 and GD1a gangliosides. These results provide further evidence of ongoing autoimmune processes in ALS patients. There is a strong relationship between serum antiganglioside antibodies and patterns of clinical involvement in ALS.
101 citations
TL;DR: This longitudinal study of 194 patients with sporadic ALS demonstrated that it is possible for physicians to predict the approximate survival time for an individual ALS patient given: the age of the patient, the duration of his weakness and an estimate of his clinical disability (ALS Score).
Abstract: This longitudinal study of 194 patients with sporadic ALS demonstrated that it is possible for physicians to predict the approximate survival time for an individual ALS patient given: (1) the age of the patient, (2) the duration of his weakness and (3) an estimate of his clinical disability (ALS Score). This information is of value in the clinical management of ALS patients, and it should facilitate construction of experimental therapeutic trials in ALS.
101 citations
Journal Article•
TL;DR: In this paper, the authors studied the prevalence and extent of lymphocytic infiltration, previously reported as a rare finding in the ALS spinal cord, and showed that such infiltrates are entirely secondary to atrophy of the cord.
Abstract: Amyotrophic lateral sclerosis (ALS) is a devastating systemic atrophy affecting the upper and lower motor neurons. The etiology is unknown, but one theory of pathogenesis supposes that the motor system is affected by abnormal immune responses. We have studied the prevalence and extent of lymphocytic infiltration, previously reported as a rare finding in the ALS spinal cord. Application of monoclonal antibodies against macrophages, T- and B-cells to spinal cords from 48 ALS patients disclosed a cellular mononuclear infiltrate in 38 specimens (79%), intense enough to be revealed by routine neuropathological techniques in 6 of them (12.5%); the remaining 10 cords (21%) exhibited no infiltrates. Since duration and clinical signs of the preceeding illness were the same in cases with and without infiltrates, we consider it unlikely that such infiltrates are entirely secondary to atrophy of the cord. As Wallerian degeneration is not accompanied by infiltrates of lymphocytes, their presence in the cord tracts of our material throws doubt on the conventional view that tract degeneration in ALS is exclusively Wallerian.
97 citations
TL;DR: It is shown that some patients with motor neuron disease, mainly those with symptoms due to respiratory muscle weakness in the absence of severe bulbar impairment, derive symptomatic benefit from supported ventilation.
Abstract: Although respiratory insufficiency is common in the advanced stages of motor neuron disease, some patients may develop distressing respiratory symptoms early in the course of the disease or even present with respiratory failure or arrest. We describe 14 patients with motor neuron disease who were considered for respiratory support; 11 received such support and all derived significant symptomatic improvement without distressing prolongation of life. Of the 8 patients with typical features of amyotrophic lateral sclerosis, 7 had predominant diaphragm weakness and 1 generalized respiratory muscle weakness; 7 received negative pressure ventilation by cuirass which improved both the quality of sleep and exercise tolerance. Three patients with predominantly bulbar disease had nocturnal apnoea or hypoventilation. Two received no support. One, who also developed diaphragm weakness, was treated by a cuirass, continuous positive airway pressure (CPAP), and later nocturnal intermittent positive pressure ventilation (IPPV). Three patients with progressive muscular atrophy had predominant diaphragm weakness or nocturnal apnoea. These patients received nocturnal CPAP, cuirass or IPPV with symptomatic benefit. This series shows that some patients with motor neuron disease, mainly those with symptoms due to respiratory muscle weakness in the absence of severe bulbar impairment, derive symptomatic benefit from supported ventilation.
71 citations
Journal Article•
TL;DR: Although the diagnosis of monomelic muscular atrophy is based on neurologic and neurophysiologic data, MR provides confirmatory evidence as well as useful information contributing to an understanding of this disease.
Abstract: We report the MR studies of the cervical cord in seven patients presenting juvenile muscular atrophy of distal upper extremity. This illness, also known as monomelic amyotrophy or benign focal amyotrophy, is distinct from the other motor neuron diseases. Seen in young males, it is characterized by muscular atrophy of the hand, and usually of the forearm, most often unilateral. The underlying process, of unknown origin, affects the anterior horn cells in the lower cervical cord. The gradual onset of purely motor disturbances may mimic early amyotrophic lateral sclerosis. This latter diagnosis may be excluded because of clinical stabilization and lack of pyramidal tract involvement. In our series, five MR studies were positive. In three cases we were able to demonstrate focal and unilateral atrophy in the lower cervical cord limited to the anterior horn region. Morphologic MR findings correlated with clinical and electromyographic features. In two other cases the MR-clinical correlation was more complex. No pathologic MR signal was detected on either T1- or T2-weighted images. Although the diagnosis of monomelic muscular atrophy is based on neurologic and neurophysiologic data, MR provides confirmatory evidence as well as useful information contributing to an understanding of this disease.
70 citations
TL;DR: A working hypothesis is proposed, suggesting how antibodies might be related to the disease process, and attempting to account for a pathogenic role of antibodies directed against the carbohydrate components of glycolipids.
Abstract: The possibility of an autoimmune mechanism of pathogenesis in amyotrophic lateral sclerosis has long been considered, but the evidence to support a conventional autoimmune process, reviewed here, is inconclusive. However, antibodies that react in vitro with gangliosides have recently been found in sera of a large majority of patients with classical amyotrophic lateral sclerosis and other motor neuron syndromes. A working hypothesis is proposed, suggesting how antibodies might be related to the disease process. The hypothesis attempts to account for (1) a pathogenic role of antibodies directed against the carbohydrate components of glycolipids, (2) the selectivity of the process for motor neurons, (3) an antibody-mediated mechanism that could result in apparently degenerative neuropathological changes without signs of inflammation, and (4) a type of autoimmune response that is extremely difficult to suppress by conventional means. Although the evidence for this hypothesis is by no means complete, its critical features are all testable.
70 citations
TL;DR: The study revealed the existence of two types of filaments in lower motor neurons of patients with amyotrophic lateral sclerosis: ubiquitin-positive, granule-associated filaments, approximately 15 nm in diameter, that form Lewy body-like inclusions; and 12 nm coated filaments that may be a candidate for another ubiquitIn-positive structure and possibly a precursor of Bunina bodies.
Abstract: Neuronal inclusions in lower motor neurons in 23 cases of adult-onset sporadic amyotrophic lateral sclerosis were studied immunocytochemically and ultrastructurally. Monoclonal and polyclonal antiubiquitin antibodies recognized four structures in the neuronal perikarya: (1) all Lewy body-like inclusions in 6 cases with a relatively short clinical course, (2) a small percentage of Bunina bodies in 4 cases with abundant Bunina bodies, (3) ill-defined structures closely associated with Bunina bodies (Bunina body-related structures) in 15 cases, and (4) a focally aggregated meshwork of fine filamentous structures not associated with Bunina bodies in all cases. These four structures were not recognized by the antibodies raised against cytoskeletal proteins (neurofilament, tubulin, microtubule-associated protein 2, and phosphorylated tau). Electron microscopy revealed Lewy body-like inclusions to be accumulations of randomly oriented filaments, approximately 15 nm in diameter, covered by fine granules. Bundles of coated filaments 12 nm in diameter that sometimes formed Bunina body-like structures were also observed in the perikarya. Immunoelectron microscopy showed the reaction product with antiubiquitin to be on the filaments, 15 nm in diameter, of Lewy body-like inclusions. Our study revealed the existence of two types of filaments in lower motor neurons of patients with amyotrophic lateral sclerosis: (1) ubiquitin-positive, granule-associated filaments, approximately 15 nm in diameter, that form Lewy body-like inclusions; and (2) 12 nm coated filaments that may be a candidate for another ubiquitin-positive structure and possibly a precursor of Bunina bodies. These two types of filaments may represent early pathological changes of lower motor neurons in amyotrophic lateral sclerosis.
TL;DR: Autonomic function mediating cardiovascular regulation was evaluated in amyotrophic lateral sclerosis (ALS) in comparison with Shy-Drager syndrome and indicated subclinical sympathetic hyperfunction and parasympathetic (vagal) hypofunction, probably resulting in cardiovascular dysfunction.
Abstract: Autonomic function mediating cardiovascular regulation was evaluated in amyotrophic lateral sclerosis (ALS) in comparison with Shy-Drager syndrome. The subjects were 14 normal controls and 9 patients each with ALS and Shy-Drager syndrome. To evaluate the autonomic function in detail, a new series of quantitative autonomic function tests was conducted, in conjunction with conventional tests. In patients with ALS, the data indicated subclinical sympathetic hyperfunction and parasympathetic (vagal) hypofunction, probably resulting in cardiovascular dysfunction.
TL;DR: Histological sections of cerebral motor cortex, brainstem, and spinal cord from 10 cases of clinically diagnosed motor neurone disease and 10 control cases were examined by conventional histology and immunocytochemical methods to localise ubiquitin inclusions, finding similar inclusions to those reported in earlier studies.
TL;DR: None of the 38 animal models described in this review provides an exact animal copy of a specific human motor neuron disease, but they reproduce certain structural or physiological aspects of their human counterparts.
TL;DR: Though this dosage had no major toxic effect, Cronassial treatment did not significantly benefit ALS patients and was not effective in any of the outpatients.
Abstract: We performed a 3-month, double-blind, placebo-controlled trial of 300 mg of gangliosides (Cronassial) in 40 outpatients with amyotrophic lateral sclerosis (ALS). We evaluated drug effect through physical examinations and symptom scales. Though this dosage had no major toxic effect, Cronassial treatment did not significantly benefit ALS patients.
TL;DR: The failure of BCAA in the treatment of the patients could be due to different disorders with unpredictable outcome included under the diagnosis of ALS.
Abstract: Thirty-two patients affected by amyotrophic lateral sclerosis (ALS) were included in a controlled, open therapeutic trial with branched chain amino acids (BCAA). Patients with bulbar muscle involvement were evaluated separately. No statistically significant differences were found in the clinical outcome between the patients treated and the control groups. Blood L-glutamate levels measured in eight patients were normal. The failure of BCAA in the treatment of the patients could be due to different disorders with unpredictable outcome included under the diagnosis of ALS.
TL;DR: Activities of choline acetyltransferase (ChAT) were microassayed in individual cell bodies of motor neurons, isolated from freeze‐dried sections after autopsy of lumbar spinal cords from four patients with sporadic amyotrophic lateral sclerosis and four control patients with nonneurological diseases.
Abstract: Activities of choline acetyltransferase (ChAT) were microassayed in individual cell bodies of motor neurons, isolated from freeze-dried sections after autopsy of lumbar spinal cords from four patients with sporadic amyotrophic lateral sclerosis (ALS) and four control patients with nonneurological diseases. Numerous large neurons were found in the anterior horn at the early degeneration stage of ALS, but the cell bodies atrophied and decreased in number at the late advanced stage. The small, atrophied neurons were very fragile and were easily destroyed during the isolation procedure with a microknife. The average activity, expressed on a dry weight basis, of 58 ALS neurons was lower than that of 67 control neurons. The large, well-preserved neurons at the early nonadvanced stage had markedly lower ChAT activities than control neurons. The specific activity gradually increased with the progress of atrophy but did not return to the control level.
TL;DR: The finding indicates that the primary degeneration may occur in the anterior horn cells and the neurons in the intermediae zone degenerate sequentially in the spinal gray matter in ALS.
Abstract: To elucidate the degenerating mechanism of the neurons in the intermediate zone of the spinal cord in classical amyotrophic lateral sclerosis (ALS), the spinal neurons in a patient with ALS, whose muscular strength was fairly well preserved up to death, were examined quantitatively and topographically, and compared with the data of advanced ALS patients and age-matched control subjects reported previously. In advanced ALS patients, anterior horn cells completely disappeared and the medium-sized (nuclear area; 71–150 μm2) and large (nuclear area; greater than 151 μm2) neurons in the intermediate zone were severely reduced. In the present case, however, the loss of anterior horn cells was severe but the degree was not equal to that of advanced ALS patients, and the neurons in the intermediate zone were quite well preserved. The finding indicates that the primary degeneration may occur in the anterior horn cells and the neurons in the intermediae zone degenerate sequentially in the spinal gray matter in ALS.
TL;DR: The protein was absent or markedly decreased in 8 early stage patients with Duchenne muscular dystrophy and in mdx mice, the absence or marked deficiency of dystrophin was also noted; however, the decrease of orthogonal arrays was not as severe as in DMD, which might relate to the milder clinical features in m dx mice as compared with those in D MD.
TL;DR: The observations show that ALS may be distinguished from SMA by the presence of abnormal dermal collagen, and suggest that comparable clinical and pathological skin analysis is the most important diagnostic tool in differentiating between ALS and SMA.
TL;DR: A correlation between the severity of the disease and the degree of impaired fixation could be observed and patients suffering from ALS for longer periods showed more pronounced impairment of 123I-IMP fixation.
Abstract: N-Isopropyl-p-123I-amphetamine (123I-IMP) single photon emission computer tomography studies were performed on 17 patients suffering from amyotrophic lateral sclerosis (ALS). All patients displayed varying degrees of impaired fixation of 123I-IMP in the whole brain. These findings were not related to the clinical variants of ALS and the age of the patients. A correlation between the severity of the disease and the degree of impaired fixation could be observed. In addition, patients suffering from ALS for longer periods showed more pronounced impairment of 123I-IMP fixation.
Journal Article•
TL;DR: In HCSMA, nerve fiber shape, i.e., circularity, was reduced, and the relative thickness of the myelin sheath as a function of axonal caliber was decreased, so changes in axonal size in motor nerves are associated with both growth arrest and axonal atrophy.
TL;DR: The role of hope as a mode of coping in patients with amyotrophic lateral sclerosis is emphasized and an analysis of tasks involved in the hoping process can be used as guidelines for nursing intervention with ALS patients and their families.
Abstract: The purpose of this article is to emphasize the role of hope as a mode of coping in patients with amyotrophic lateral sclerosis (ALS). When taking care of patients with ALS, a rapidly progressing fatal disease which has no known cure or treatment and obscure etiology, the nurse's primary aim is to assist the patient to live as fully as possible within limitations imposed by the disease. An analysis of tasks involved in the hoping process can be used as guidelines for nursing intervention with ALS patients and their families.
TL;DR: The maximal action potential amplitude of the muscle in the ALS patients was significantly smaller than that in the control group and the difference between the maximal and minimal conduction velocities in each ALS patient was not statistically different from that in each control subject.
Abstract: In 15 patients with amyotrophic lateral sclerosis (ALS) and 20 age-matched control subjects, we measured the maximal and minimal motor nerve conduction velocities of the ulnar nerve as well as the action potential amplitude of the abductor digiti minimi muscle Both maximal and minimal motor nerve conduction velocities in ALS patients were significantly lower than those in the control group However, the difference between the maximal and minimal conduction velocities in each ALS patient was not statistically different from that in each control subject The maximal action potential amplitude of the muscle in the ALS patients was significantly smaller than that in the control group
TL;DR: The high incidence focus of ALS and PD on Guam and similar, but less well‐studied, foci in West New Guinea and the Kii Peninsula of Japan represent natural paradigms of chronic degenerative disease that have provided new information and insights for understanding other neurological disorders such as classical ALS, Parkinson disease, Alzheimer disease, and early neuronal aging.
Abstract: The strikingly high incidence of amyotrophic lateral sclerosis (ALS) and parkinsonism-dementia (PD), two uniformly fatal neurodegenerative disorders which often occur in the same families and occasionally in the same individual, was recognized on Guam more than three decades ago. Since the first systematic observations began, nearly 800 Guamanian Chamorro patients have been clinically diagnosed as having either disease. The original incidence rates for ALS and PD accounted for one in five deaths among Chamorros over ago 25. During the past 30 years, however, the incidence and mortality rates have dramatically declined and today the risk to Guamanian chamorros is only several-fold higher than that for non-Chamorro residents of the continental United States. The accumulating epidemiological and genetic data strongly suggest that environmental factors are primarily involved in the etiology and pathogenesis of these disorders. The high incidence focus of ALS and PD on Guam and similar, but less well-studied, foci in West New Guinea and the Kii Peninsula of Japan represent natural paradigms of chronic degenerative disease that have provided new information and insights for understanding not only ALS and PD, but other neurological disorders such as classical ALS, Parkinson disease, Alzheimer disease, and early neuronal aging, insights that might otherwise not have been forthcoming from studies of low incidence sporadic disease in large cosmopolitan Western communities.
TL;DR: The serum from 30 patients with amyotrophic lateral sclerosis and 30 controls was tested by immunoblot with protein extracts obtained from fetal muscle, adult muscle, and denervated muscle and reactivity was confirmed by immunoperoxidase staining in frozen sections of fetal muscle.
Abstract: The serum from 30 patients with amyotrophic lateral sclerosis and 30 controls was tested by immunoblot with protein extracts obtained from fetal muscle, adult muscle, and denervated muscle. Results with adult and denervated muscle confirm previous reports that show a lack of particular reactivity of ALS serum when compared with control serum. However, we found reacting bands 5 times more frequently with ALS than with controls in the immunoblot with protein extract from rat fetal muscle; we confirmed reactivity by immunoperoxidase staining in frozen sections of fetal muscle. Our results point to the possibility of an immune reaction in ALS patients against ephemeral proteins of muscle that are present in large quantities at early stages of muscle differentiation and innervation.
TL;DR: There was a shifting age pattern of MND mortality with a higher proportion of cases in older ages for the most recent time period and there was a slight increase in total average annual age adjusted ALS/MND mortality rates over the three intervals.
Abstract: All death certificates with amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND) as the primary or underlying cause occurring among Kentucky residents between 1964 and 1984 were manually
TL;DR: Monoclonal antibody 44.1, an immunocytochemical marker for neurons, identified heterotopically located, multipolar neurons deep within the spinal cord white matter of patients with amyotrophic lateral sclerosis.
Abstract: Monoclonal antibody 44.1, an immunocytochemical marker for neurons, identified heterotopically located, multipolar neurons deep within the spinal cord white matter of patients with amyotrophic lateral sclerosis. Displaced neurons were most numerous in the ventral outflow and lateral corticospinal tract regions of all cord levels. These changes may be the result of aberrant neuronal migration during spinal cord development.
TL;DR: The findings show that the variance in phonation time accounts for nearly 50% of the variability of vital capacity, and therefore, successive measurements of phonationTime are a useful means of estimating the loss of vital Capacity.