Showing papers on "Amyotrophic lateral sclerosis published in 1981"
TL;DR: It is presented that each of these disorders is due to lack of a disorder‐specific neurotrophic hormone, and central nervous system tissue culture provides a convenient system in which to assay these neurotrophic hormones and should permit a test of the hypothesis.
Abstract: The causes of amyotrophic lateral sclerosis, Parkinson disease, and Alzheimer disease are unknown. Furthermore, treatment for two of these conditions is almost totally lacking. The thesis is presented that each of these disorders is due to lack of a disorder-specific neurotrophic hormone. The hormone would be elaborated or stored in the target of the affected neurons. It would be released by the postsynaptic cell and then exert its effects in a retrograde fashion after being taken up by the presynaptic terminal. In the lower motor neuron syndromes of amyotrophic lateral sclerosis, failure of muscle cells to release the appropriate motor neurotrophic hormone would result in impaired function of anterior horn cells. In Parkinson disease, the neurotrophic failure would be characterized by inability of striatal cells to provide the required dopamine neurotrophic hormone with resulting impairment of substantia nigra cells. In Alzheimer disease, the abnormalities would lie in failure of the hippocampus and cortical cells to supply the relevant cholinergic neurotrophic hormone with resulting impairment of medial septal and nucleus basalis neurons. Central nervous system tissue culture provides a convenient system in which to assay these neurotrophic hormones and should permit a test of the hypothesis.
696 citations
373 citations
TL;DR: In histograms prepared from seven cases of amyotrophic lateral sclerosis (ALS), the Cl and Al peaks were decreased selectively and severely, providing evidence for alpha, but not gamma, motor neuron vulnerability.
Abstract: The diameter histograms of cell bodies (cytons) in motor neuron columns at the L5 segment of the spinal cord of adult man reproducibly yield three peaks of increasing height: small (Cs), intermediate (Ci), and large (Cl). Histograms of L5 myelinated axons obtained from the ventral root have two peaks of increasing height: intermediate (Ai) and large (Al). In histograms prepared from seven cases of amyotrophic lateral sclerosis (ALS), the Cl and Al peaks were decreased selectively and severely. This provides evidence for alpha, but not gamma, motor neuron vulnerability. The Cl peak of spinal ganglion neurons and the Al peak of dorsal roots were significantly reduced in number, without a concomitant increase in Ci, Cs, and Ai peaks. This, plus earlier reports of abnormal cutaneous sensation thresholds, abnormal rates of fiber degeneration in cutaneous nerves, and dorsal column demyelination, provides evidence that large afferent neurons are affected in ALS, but to a lesser degree than alpha motor neurons.
206 citations
TL;DR: The present study suggested that the site of the primary lesion seems to be in the α-motoneuron fibers in motor neuron diseases, such as ALS or SPMA, and the marked discrepancy in the pathologic change in theα-mot oneuron fibersIn the VSR and the nerve roots innervating the external ocular muscles was noteworthy.
Abstract: Amyotrophic lateral sclerosis (ALS) and adult onset X-linked recessive bulbospinal muscular atrophy (SPMA), constituting the category of adult onset form of motor neuron disease, were analyzed on motor nerve roots. The results of morphometric analysis on ventral spinal roots (VSR) of all spinal segments from ALS and SPMA revealed the following three findings: (1) the large-myelinated α-motoneuron fibers were markedly decreased in number throughout all segments; (2) thin-myelinated autonomic preganglionic fibers were almost completely preserved; (3) small-intermediate-myelinated fibers which are considered to correspond to γ-motoneuron fibers were generally well preserved in ALS, but decreased by one-half to one-third in SPMA. However, all the components of the nerve roots of the oculomotor, trochlear, and abducent nerves were completely preserved in both ALS and SPMA. Moreover, the teasedfiber study showed that the regenerating-sprouting process rarely occurred in the VSR of ALS and SPMA. The present study suggested that the site of the primary lesion seems to be in the α-motoneuron fibers in motor neuron diseases, such as ALS or SPMA. However, the marked discrepancy in the pathologic change in the α-motoneuron fibers in the VSR and the nerve roots innervating the external ocular muscles was noteworthy.
57 citations
TL;DR: In ALS, glycinergic receptor binding was greatly reduced in the anterior gray matter, and this finding may be attributed to loss of large neurons in the posteriorgray matter, known to be characteristic of ALS.
Abstract: Transmitter receptor binding was estimated in the spinal cord of 6 subjects with amyotrophic lateral sclerosis (ALS) and 4 control subjects in assays using 3H-quinuclidinyl benzilate for muscarinic cholinergic receptors, 3H-strychnine for glycinergic receptors, 3H-spiroperidol for dopaminergic receptors, 3H-muscimol for GABAergic receptors, and 3H-dihydroalprenolol for beta-adrenergic receptors. In ALS, glycinergic receptor binding was greatly reduced in the anterior gray matter. This finding may be attributed to loss of large neurons in the anterior gray matter, known to be characteristic of ALS.
54 citations
TL;DR: The normal values from the Creutzfeldt-Jakob cases imply that neuronal destruction per se need not lead to accumulation of aluminum in the brain, and the significance of high aluminum values is not clear.
Abstract: Graphite furnace atomic-absorption spectroscopy was used to measure aluminum concentrations in brain samples from 33 patients dying from a variety of neurologic diseases. Four samples from patients dying of nonneurologic causes also were studied. Nine samples (one from each of nine patients) of Creutzfeldt-Jakob disease brain contained normal amounts of aluminum. Aluminum was increased in 9 of 18 brain specimens with seven different pathologic processes. This included three of seven Alzheimer disease, two of three Huntington disease, two of two Parkinson disease, one of one progressive supranuclear palsy, one of one acoustic neuroma, one of two cerebrovascular disease, and one of two Guamanian amyotrophic lateral sclerosis (ALS). Aluminum was normal in the remaining samples (four normal, two ALS, one multiple sclerosis, one Pick disease, and two Guamanian parkinsonism-dementia). The significance of high aluminum values is not clear, but the normal values from the Creutzfeldt-Jakob cases imply that neuronal destruction per se need not lead to accumulation of aluminum in the brain.
47 citations
TL;DR: No difference in RNA content was observed between the ALS group and controls in the nucleus dorsalis, which suggests that the reduction of RNA is restricted to the motor system in ALS.
Abstract: The content of RNA and volume of individual neurons isolated from the nucleus dorsalis and from the ventrolateral portion of the lumbar swelling were determined in eight cases of amyotrophic lateral sclerosis (ALS) and eight controls whose spinal cords were obtained at autopsy. The mean content of RNA in the lumbar motor neurons of the controls was 557 pg, compared to only 386 pg in the ALS group. This represents approximately a 31% reduction and is highly significant, p less than 0.01. No difference in RNA content was observed between the ALS group and controls in the nucleus dorsalis, which suggests that the reduction of RNA is restricted to the motor system in ALS. The volume of individual motor neurons of the lumbar intumescence was not significantly different between the controls and ALS.
43 citations
TL;DR: When the disease starts before the age of 50, the prognosis is often less poor than usual and the forms with spinal, and especially cervical, onset appear to be less rapid than bulbar forms; there may be some unknown factor that increases the resistance of some subjects to the disease.
Abstract: The clinical features and course of amyotrophic lateral sclerosis are discussed. The data on a series of 116 patients are compared with those of the literature. The following points emerge:
39 citations
TL;DR: The content of RNA and the volume of motor neurons isolated from the lateral portion of the cervical swelling were examined in six control and six amyotrophic lateral sclerosis cases obtained at autopsy and the reduction in RNA content in the ALS group is statistically significant.
Abstract: The content of RNA and the volume of motor neurons isolated from the lateral portion of the cervical swelling were examined in six control and six amyotrophic lateral sclerosis (ALS) cases obtained at autopsy. The mean volume of motor neurons in the ALS group did not differ significantly from the values obtained in the controls. However, in two cases of ALS with extensive neuronal loss and relatively long duration of disease most of the remaining cells were atrophic. The content of RNA in motor neurons averaged 300 micromilligram in the ALS group compared to 513 micromilligram in the control cases. The reduction in RNA content in the ALS group is approximately 42% and is statistically significant.
27 citations
01 Jan 1981
TL;DR: The rarity of the association of a pallido-luyso-nigral atrophy and an ALS, the occurrence of an ALS at such a young age and the fact that her grandmother died of Parkinson disease at age 30 suggest that this association may represent more than a coincidental occurrence.
Abstract: Clinical and neuropathological studies of a case of pallido-luyso-nigral atrophy and amyotrophic lateral sclerosis (ALS) in a young woman with a strong likelyhood of a similar familial past medical history have been presented. Microscopic examination revealed neuronal loss and gliosis of globus pallidus, corpus luysii and substantia nigra. Pallor of the pyramidal tracts and neuronal loss in hypoglossal nuclei and anterior horns with gliosis were present. The rarity of the association of a pallido-luyso-nigral atrophy and an ALS, the occurrence of an ALS at such a young age and the fact that her grandmother died of Parkinson disease at age 30 suggest that this association may represent more than a coincidental occurrence.
26 citations
TL;DR: Treatment with cerebrospinal fluid from patients with amyotrophic lateral sclerosis and other neuromuscular disorders had no effect on neuron-specific enolase, which was measured in cultured embryonic rat motor neurons as an index of neuronal health.
Abstract: Immunoreactivity of neuron-specific enolase was measured in cultured embryonic rat motor neurons as an index of neuronal health. Treatment with cerebrospinal fluid from patients with amyotrophic lateral sclerosis and other neuromuscular disorders had no effect on neuron-specific enolase.
TL;DR: Serum immunoglobulin (Ig) levels did not correlate with the duration of either disease, and Immunodeficient ALS and PD patients had higher IgM and lower IgA levels than the other ALS andPD patients.
Abstract: Among Guamanian natives, serum IgA and IgG levels were found to be higher than normal in amyotrophic lateral sclerosis (ALS); serum IgA was higher and IgM lower than normal in parkinsonism-dementia (PD). IgA levels increased with age in ALS, PD, and normal subjects; IgG increased with age in ALS and IgM decreased with age in PD. Serum immunoglobulin (Ig) levels did not correlate with the duration of either disease. Immunodeficient ALS and PD patients had higher IgM and lower IgA levels than the other ALS and PD patients.
Neither differences in viral antibody titers nor the presence of autoantibodies or circulating immune complexes could account for the variations in serum Ig levels between patients and controls. We conclude that differences in serum Ig levels in ALS and PD patients are probably due to repeated infections and abnormal immunoregulation accompanying immunodeficiency during the course of ALS and PD, rather than to a specific antiviral or autoimmune response.
Journal Article•
TL;DR: A neuropsychiatric survey of three generations of a family with adult-onset ALS with dementia and psychosis in each generation found possible genetic links due to regional inbreeding and a consanguineous marriage.
TL;DR: A Spanish family transmits, as an autosomal dominant trait, a form of amyotrophic lateral sclerosis characterized by an unusually prolonged evolution of the disease in all affected members.
TL;DR: The clinical and pathologic features of Amyotrophic lateral sclerosis, a progressive disease characterized by selective loss of upper and lower motor neurons, equals or exceeds multiple sclerosis in incidence, are discussed.
Abstract: Amyotrophic lateral sclerosis, a progressive disease characterized by selective loss of upper and lower motor neurons, equals or exceeds multiple sclerosis in incidence. The clinical and pathologic features are discussed, as well as practical management strategies. Also included is an examination of the extensive investigations under way to determine the syndrome's elusive etiology.
TL;DR: A 57-year-old female patient who had been treated for 7 years with cortisone because of myelophthisis, developed amyotrophic lateral sclerosis (ALS) 4 months after a herpes zoster infection.
Abstract: A 57-year-old female patient who had been treated for 7 years with cortisone because of myelophthisis, developed amyotrophic lateral sclerosis (ALS) 4 months after a herpes zoster infection Cellular and humoral immunodeficiency were observed Possible connections of herpes zoster infection and ALS are discussed
Journal Article•
TL;DR: The global involvement of the cerebral cortex correlated well with the loss of higher mental function which was present in the patients, and the lesions show a characteristic topographical distribution.
Abstract: The Guam amyotrophic lateral sclerosis and Parkinsonian-dementia (ALS and PD) complex still presents a challenge to research Previous papers have highlighted the lesions in the hippocampus and basal nuclei to explain the bizarre clinical syndrome In our study of 20 to 62 cases of PD with or without ALS, significant widespread and often severe lesions were found throughout the entire cerebral cortex These changes included variable degrees of nerve cell loss, spongy change, gliosis, increased lipofuscin content of nerve cells, the presence of granulovacuolar bodies and neurofibrillary tangles within neurones and, less frequently, Hirano bodies The lesions show a characteristic topographical distribution The global involvement of the cerebral cortex correlated well with the loss of higher mental function which was present in the patients
TL;DR: The patient has had progressive involvement of the lower and upper extremities and trunk, preserving his ability to walk and swim by maintaining a schedule of exercise and rest.
Abstract: To the Editor.— Amyotrophic lateral sclerosis (ALS) is a progressive disease of unknown etiology in which there is destruction of the anterior horn cell, with the clinical expression of weakness and atrophy, fasciculations, and hyperreflexia. An electrolyte disturbance has not consistently been observed in this illness, yet we have recently noted persistent hypokalemia in one patient. The diagnosis of ALS had been established in June 1979. Since then the patient has had progressive involvement of the lower and upper extremities and trunk, preserving his ability to walk and swim by maintaining a schedule of exercise and rest. There were no other medical illnesses. Medications were diazepam, 10 mg at bedtime, and 50 to 100 g of lecithin daily, which was begun in April 1980. For the first four months this was taken as granules of animal lecithin mixed as a milkshake and for the past six months as soybean lecithin
TL;DR: Figure Pol vgraphlic (top) atid oscil/loscopic (/ottotI) recoridings of.s.s ssitll te7 reappearance of miuscle totie.
Abstract: Figure Pol vgraphlic (top) atid oscil/loscopic (/ottotI) recoridings of. (A) .seiLl-ie di.,ring rest. ninvoc/loic jerk.s. Tue oscil/oscopic recording s/iows. the siiu/ltaneoius occurrenCe of.spike (117d1 inYloC/iic Ierk.s. (B) sci--ire drlitig posture wit/il out-stretch/ec/ artiiu drllop of the lieaid ailcl iii.s. Tlhe oscilloscopic recordiig s110.S1 tIle coinicidenice of /ofs o tolle withi the .slow comiponenlt o/ t/he spike-and-wave comiip/ex; the spike is sync/hronou.s ssitll te7 reappearance of miuscle totie.
TL;DR: Action potential analyses were carried out in 62 skeletal muscles of 16 patients with the Guillain‐Barré syndrome, finding changes in duration in the early stage of a poor prognosis muscles were similar to those of acute beriberi neuropathy and the changes in the late stage were similarto those of amyotrophic lateral sclerosis.
Abstract: Action potential analyses were carried out in 62 skeletal muscles of 16 patients with the Guillain-Barre syndrome. The muscles of a good prognosis showed only a slight change in duration, amplitude and phase of action potentials on electromyograms. On the other hand, the muscles of a poor prognosis showed a marked increase in duration and amplitude of action potentials, especially in the chronic stage. The incidence of polyphasic potentials was more frequent in the early stage than in the chronic stage of the muscles of a poor prognosis. The changes in duration in the early stage of a poor prognosis muscles were similar to those of acute beriberi neuropathy and the changes in the later stage were similar to those of amyotrophic lateral sclerosis, both of which showed axonal degenerations. In the early stage, myopathy-like changes in electromyography were often noticed, but the muscle biopsy showed only early neurogenic changes. Coupling or satellite discharges were found most frequently six or eight months after the onset, and decreased in number in the chronic stage. Satellites with blockings appeared earlier than those without blockings. A poor prognosis might be induced by an axonal degeneration partly together with segmental demyelinations.