Showing papers on "Amyotrophic lateral sclerosis published in 1973"
TL;DR: A high proportion of diluted serums of patients with amyotrophic lateral sclerosis were toxic to the anterior horn cells of the mouse in tissue culture, and Serums from other degenerative neurological diseases were inactive.
Abstract: A high proportion of diluted serums of patients with amyotrophic lateral sclerosis were toxic to the anterior horn cells of the mouse in tissue culture. This is not a general cytotoxicity, since apparently only the neurons were killed. Serums from other degenerative neurological diseases were inactive.
87 citations
TL;DR: The motor-neuron diseases as considered here include the clinical syndromes that have been shown to be due to primary abnormalities of anterior-horn cells and motor cranial-nerve nuclei.
Abstract: THE motor-neuron diseases as considered here include the clinical syndromes that have been shown to be due to primary abnormalities of anterior-horn cells and motor cranial-nerve nuclei. The term motor-neuron disease is used in a more restricted sense for the syndrome of amyotrophic lateral sclerosis and the clinical components, progressive muscular atrophy and progressive bulbar palsy. Although these disorders have a similar pathologic substrate and clinical expression, they do not necessarily share the same causal factors. Some are clearly hereditary, and amyotrophic lateral sclerosis is sporadic in 95 per cent of cases. In recent years, the distinctions between the major . . .
79 citations
TL;DR: Although the clinical and histological findings are variable, the disease seems to be a distinct entity, closely allied to amyotrophic lateral sclerosis, but also having features in common with Guamanian amyotropolitan sclerosis, and amyotroph lateral sclerosis-dementia complex of sporadic occurrence.
Abstract: In familial amyotrophic lateral sclerosis associated with dementia there was symmetrical involvement of the amygdala, insula, and fronto-temporal cortex. The cortical lesions were most severe in the superior frontal gyri and the infero-lateral temporal lobes, while the adjacent cingulate gyri and hippocampi were spared. One patient with dementia and another with slight mental abnormality had cerebral lesions which varied in extent, but were of the same type and pattern. Although the clinical and histological findings are variable, the disease seems to be a distinct entity, closely allied to amyotrophic lateral sclerosis, but also having features in common with Guamanian amyotrophic lateral sclerosis, and amyotrophic lateral sclerosis-dementia complex of sporadic occurrence.
44 citations
TL;DR: It is suggested that lesions of the type which produce Parkinson disease increase the susceptibility to levodopa-induced dyskinesias, but that apparently denervation supersensitivity alone does not account for this phenomenon.
Abstract: The incidence of levodopa-induced dyskinesias in 20 Guamanian patients with parkinsonism-dementia (PD) was compared with that found in 14 Guamanians with amyotrophic lateral sclerosis (ALS), who were matched for dose and duration of drug exposure. While 55% of the PD patients developed abnormal involuntary movements during the first six months of treatment, none of the ALS patients manifested this complication. In PD patients no definite correlation could be documented between pretreatment parkinsonian severity or degree of response to levodopa and the appearance of dyskinesias. The results suggest that lesions of the type which produce Parkinson disease increase the susceptibility to levodopa-induced dyskinesias, but that apparently denervation supersensitivity alone does not account for this phenomenon.
40 citations
TL;DR: Cell cultures established from tissues of nine patients with amyotrophic lateral sclerosis and maintained for several months to over a year yielded no clue as to a possible viral cause of the disease.
Abstract: Cell cultures established from tissues of nine patients with amyotrophic lateral sclerosis (ALS) and maintained for several months to over a year yielded no clue as to a possible viral cause of the disease. Serum antibody titers to 15 viral agents in 34 patients with ALS did not differ from those of matched controls.
32 citations
TL;DR: There was an overlap of pathological reactions in muscles from the dystrophies and the neurogenic atrophies, and it appears that the concept of muscular dystrophy as a primary muscle disease needs to be re-examined.
Abstract: Muscle biopsy specimens from 179 cases of muscular dystrophies and from 140 cases of anterior horn cell disorders (from a total of 1,348 biopsied patients) were examined histologically. There were 72 cases of Duchenne type muscular dystrophy (DMD), five of Becker type MD, four girls with myopathy resembling DMD, 40 with limb-girdle, 10 with facioscapulohumeral, seven with late onset, 13 with congenital, and 28 with unclassifiable muscular dystrophies. Groups of small atrophied muscle fibres were encountered in 42 (23%) of the cases in this group, most frequently in patients with limb-girdle, facioscapulohumeral, and least frequently with DM dystrophy. In the second group there were 25 cases of infantile, 38 of juvenile, and 39 of adult spinal muscular atrophy (SMA); there were 21 patients with motor neurone disease (MND), six with poliomyelitis, and 11 with an unclassifiable type of anterior horn cell disorder. Pseudomyopathic changes were encountered in 43 (30%) of all cases in this group. They were most frequently present among patients with juvenile and adult SMA and in those with MND. The presence of group atrophy in muscular dystrophy is considered significant myopathological evidence of a denervation process. On the other hand, pseudomyopathic changes, variation in fibre size, rounding, central nuclei, and increase in connective tissue occurring in various anterior horn cell disorders are seen not to be specific `myopathic' changes. Thus there was an overlap of pathological reactions in muscles from the dystrophies and the neurogenic atrophies. Comparably atrophied fibres (much less than 2 SDs below the normal mean diameter) and hypertrophied fibres (much more than 2 SDs above the normal mean diameter) were encountered in both dystrophy and neurogenic atrophy, considering the large muscles of the limb. Likewise, the mean fibre diameters were comparable in DMD and in juvenile SMA. The fourth evidence of a neurogenic factor in muscular dystrophy was derived from an examination of SDH preparations of muscle. There was a preponderance of type I muscle fibres in dystrophic muscles compared with specimens from controls, suggesting depletion of type II fibres. It appears that the concept of muscular dystrophy as a primary muscle disease needs to be re-examined.
18 citations
TL;DR: The clinical and neuropathologic findings in a patient with a similar disorder from another family and one member of the large Dutch family that clinically displayed an ALS-dementia complex are published.
Abstract: In 1968,' we reported a slowly progressive hereditary disorder, i.e., a juvenile amyotrophic lateral sclerosis (AM)-dementia complex, in a Dutch family; 7 of the 15 siblings from healthy but consanguineous parents had this disease. In 1969,2 we published the clinical and neuropathologic findings in a patient with a similar disorder from another family (case A). This disorder was probably hereditary also due t o the extreme rarity of this syndrome, the possibility that an older sibling had suffered from a comparable disorder, and the consanguinity of the parents. Until now, this clinical syndrome had only been described by Hoffmann3 in 1895. Neuropathologic examination of case A showed diffuse demyelination of white and gray matter of the spinal cord and degeneration of the anterior horn cells and of nerve cells of the spinal ganglia. The corpus callosum was extremely thin, with active demyelination, which was also present in the white matter beneath the motor cortex and in the internal capsule. Throughout the white matter, but especially in the somewhat atrophic basal ganglia, loss of perivascular substance was found; however, the nerve cells in the basal ganglia were nor ma1 . We now have neuropathologic data f rom one member (case B) of the large Dutch family that clinically displayed an ALS-dementia complex. For the clinical details we refer t o our previous report published in 1968.l
17 citations
TL;DR: It is proposed that if neurofibril lary changes in cer ta in areas of predilection in the central nervous system (CNS) in a n o n C h a m o r r r o with ALS, who had been exposed to the Guamanian disease, it would indicate that the disease could be acquired.
Abstract: Class i c s p o r a d i c a m y o t r o p h i c l a t e ra l sclerosis (ALS) differs in important respects f rom ALS found among the C h a m o r r o s o n the island of G u a m . Brody, Hirano and S c o t t ’ postulated that if they found neurofibril lary changes in cer ta in areas of predilection in the central nervous system (CNS) in a n o n C h a m o r r o with ALS, who had been exposed t o the Guaman ian disease, it would indicate that the disease could be acquired. Th i s report dea l s with such a case.
12 citations
5 citations
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TL;DR: Findings suggest some heterogeneity of the demyelinating process in various cases of SSPE and indicate age-dependent differences in attempts to restore the structural proteins of myelin and the changes observed in ALS confirm the different nature of the pathogenesis of that disease.
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05 Nov 1973
TL;DR: There is a close relationship between the two entities in the case described, and the role of neurosyphilis as an atiological factor of ALS is discussed.
Abstract: A clinic case of amyotrophic lateral sclerosis (ALS) in a 51 years old woman is reported. The patient exhibited signs of pyramidal system and anterior motor neuron involvement. The cerebrospinal fluid examinations showed clear cut indications of parenchymatous neurosyphilis. Attention is called to the rarity of the association above mentioned. The role of neurosyphilis as an atiological factor of ALS is discussed. The authors conclude that in the case described there is a close relationship between the two entities.