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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a retrospective study, we reviewed sections from the spinal cords from eight patients, aged 36 to 61 years, who had had poliomyelitis and who died of nonneurologic diseases nine months to 44 years (mean, 20.7 years) after the acute poliomyelitis infection. Five patients had stable postpoliomyelitis deficits without new symptoms, and three patients had new slowly progressive muscle weakness defined as postpoliomyelitis progressive muscular atrophy (PPMA). Representative spinal cord sections matched the patients' clinical involvement in both groups. Control tissues from ten patients with amyotrophic lateral sclerosis and five with spinocerebellar degeneration were examined simultaneously. The spinal cord segments from all patients who had had poliomyelitis showed loss or atrophy of motor neurons, severe reactive gliosis (disproportional to the neuronal loss), and a surprising mild to moderate perivascular and interparenchymal inflammation. There was no difference in these pathologic changes between the patients with stable postpoliomyelitis deficits and those with PPMA. Additional findings were axonal spheroids (dystrophic axons) and occasional chromatolytic neurons in the spinal cord of patients with PPMA. Corticospinal tracts were spared.
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PMID:Long-term changes in the spinal cords of patients with old poliomyelitis. Signs of continuous disease activity. 335 1

Neuropathological features of a case of ataxia-telangiectasia are reported. The main findings were the presence of Lewy bodies, cytoplasmic inclusions and axonal spheroids in the brainstem nuclei; pathological changes of spinal cord closely resembled those reported in the familial form of amyotrophic lateral sclerosis. In immunocytochemical studies, filamentous inclusions and axonal spheroids strongly reacted with monoclonal antibodies against neurofilament subunits. The results show that disorganization and accumulation of neurofilament proteins occur in ataxia-telangiectasia.
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PMID:Cytoskeletal pathology in ataxia-telangiectasia. 337 Aug 63

Analysis of the superficial peroneal nerve sampled in 9 cases of classical amyotrophic lateral sclerosis (classical ALS, Charcot disease) and compared with 8 age-matched controls showed a very significant reduction of all myelinated fibres (P less than 0.001), affecting small-diameter (P less than 0.01) and large-diameter (P less than 0.02) fibres. Moreover, the small-diameter unmyelinated fibres were very significantly reduced (P less than 0.001) and the large-diameter fibres were highly increased (P less than 0.01). These results suggest a phenomenon of chronic axonal degeneration. Analysis of the same nerve in 7 patients suffering from juvenile ALS and compared with 4 age-matched controls showed a significant reduction (P less than 0.05) of myelinated fibres. The small-diameter and overall unmyelinated fibres were not significantly reduced while the large-diameter fibres, were significantly increased (P less than 0.01). The same analysis of 4 patients presenting an early-onset ALS compared with 3 controls showed lesions of a severity half-way between that of the classical and the juvenile form. Our study showed that the lesions of the sensory nerve are of the same type in classical ALS and in juvenile ALS, but of differing severity. The nosologic place of juvenile ALS compared with classical ALS and with heredodegenerative diseases of the nervous system is discussed.
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PMID:Morphometric study of the sensory nerve in classical (or Charcot disease) and juvenile amyotrophic lateral sclerosis. 358 5

Amyotrophic lateral sclerosis (ALS), Parkinson's disease (PD), and Alzheimer's disease (AD) are human neurological disorders which occur in middle and late life. These three diseases share certain features: they are slowly progressive; transmitter-specific groups of neurons are selectively affected by disease processes; and affected nerve cells exhibit cytoskeletal pathology. The causes and mechanisms of cell injury are unknown, and there are no treatments which directly affect the disease process. Dysfunction and death of these specific cell groups account for different clinical syndromes. In ALS, patients become paralyzed, at-risk cholinergic motor neurons in the spinal cord develop neurofilamentous swellings of proximal axons, and distal axons atrophy. In PD, affected individuals show slowed movements, tremor, and rigidity. These clinical findings are attributed to regeneration of dopaminergic neurons of the substantia nigra, a cell group showing abnormal accumulations of neurofilament antigens in the form of Lewy bodies. In AD, patients develop dementia (a syndrome of cognitive and memory impairment), and cholinergic neurons of the basal forebrain and certain other populations of nerve cells develop abnormalities of the cytoskeleton. These include perikaryal neurofibrillary tangles and enlarged distal axons which appear as neurites in senile plaques. Certain features of ALS, PD, and AD are recapitulated in three animal models described in this review. Hereditary Canine Spinal Muscular Atrophy (HCSMA), a dominantly inherited motor neuron disease, shows many clinical and pathological features in common with ALS. Affected dogs are clinically weak, have denervation atrophy of muscles, and develop neurofilamentous swellings of proximal axons, atrophy of distal axons, and degeneration of motor neurons. These abnormalities of axonal caliber are associated with impaired transport of the neurofilament triplet proteins and a maldistribution of phosphorylated neurofilaments. Intoxication of macaques with 1-methyl-4-]henyl-1,2,3,6,tetrahydropyridine (MPTP) produces a Parkinsonian syndrome due to selective injury of dopaminergic neurons in the substantia nigra and associated denervation of the striatum. Finally, aged rhesus monkeys (older than 23 years of age) show cognitive and memory deficits and exhibit senile plaques whose neurites are derived from cholinergic and other transmitter systems. Although these macaques do not have AD, they do provide a model for examining the relationships between age-associated cognitive deficits and pathological changes occurring in certain transmitter systems of primates.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Animal models of degenerative neurological disease. 360 87

The aim of this work was to study the neuropathological picture of the sural nerve in the pseudopolyneuropathic form of amyotrophic lateral sclerosis (ALS). Five patients were considered: in all cases the clinical and electromyographic follow-up excluded other diseases. EMG-studies were repeatedly performed: they showed the progressive evolution of the spinal anterior horn cell pathology from lower spinal to cervical levels. The sural nerve was removed and immediately fixed in phosphate-buffered 2.5% glutaraldehyde and processed according to the procedure used in our laboratory for light and ultrastructural microscopy. Quantitative analysis of myelinated fiber density was carried out on photographic enlargements of 1 micron semithin sections and recorded on histograms. The light and ultrastructural findings revealed a severe loss of myelinated fibers, the decrease affecting all types of fibers, but predominantly the largest ones. In the teased fibers, Wallerian-like degeneration was observed. In the axons there was an increase of mitochondria, dilatation of the small vesicles, and an increase in the number of neurofilaments. It can be assumed from the histopathologic data that the neuropathologic pattern in the pseudopolyneuropathic form of ALS shows an axonal degeneration. It is our opinion that the histopathologic data obtained in the sural nerve biopsy in this form of ALS reveals a clear involvement of the sensory neurons of the spinal ganglia, and the results can be useful for the study of precocious lesions in ALS.
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PMID:Morphological studies of sural nerve biopsies in the pseudopolyneuropathic form of amyotrophic lateral sclerosis. 372 69

Morphometric evaluation was performed on myelinated fibers of the corticospinal tract at the seventh thoracic spinal cord segment from three patients with Shy-Drager syndrome (SDS), six patients with amyotrophic lateral sclerosis (ALS), and five patients with nonneurologic symptoms. In SDS, small-sized myelinated fibers were nearly completely depleted, while large-sized myelinated fibers were considerably well preserved. In ALS, on the contrary, large myelinated fibers were predominantly decreased. These results suggested that selective vulnerability of axonal loss depends on fiber size and should be considered in interpretation of pathology of corticospinal tracts.
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PMID:Size-dependent myelinated fiber loss in the corticospinal tract in Shy-Drager syndrome and amyotrophic lateral sclerosis. 382 53

We report the clinical and pathological findings of the unusual combination of two idiopathic central nervous system diseases, multiple sclerosis and amyotrophic lateral sclerosis in a 56 year old physician with a twenty-seven year history of a disease initially characterized by relapses and remissions, followed by an eight year quiescent period. During the last year of life there was rapid deterioration with development of generalized weakness, atrophy, weight loss and fasciculations of body and tongue, and associated difficulty with swallowing and sudden respiratory failure. The autopsy confirmed characteristic "burned out" plaques of multiple sclerosis and anterior horn cell and axonal degeneration of amyotrophic lateral sclerosis.
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PMID:The concurrence of multiple sclerosis and amyotrophic lateral sclerosis. 395 55

A 59-year-old man presented with dyspnoea, hypersomnia followed by acute respiratory failure necessitating mechanical ventilation. There were no signs of cardiopulmonary disease and on the first few days, extubation was impossible. Further neurological evaluation supported the diagnosis of amyotrophic lateral sclerosis. The neuropathological examination corroborated this diagnosis, and showed a preponderance of lesions in the phrenic nuclei and axonal alterations associated with a fast evolution.
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PMID:Amyotrophic lateral sclerosis presenting with respiratory insufficiency as the primary complaint. Clinicopathological study of a case. 397 16

Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of the motor system in adults that occurs in sporadic, familial, and Western Pacific forms. Involvement of non-motor pathways has been increasingly recognized, both clinically and pathologically. Although the usual course is relentlessly progressive with death in half the cases within three years from onset, it can sometimes be protracted. Degeneration and loss of large motor neurons in the cerebral cortex, brainstem, and cervical and lumbar spinal cord are characteristic. Marked reduction in the number of large myelinated fibers is notable in the cervical and lumbar ventral roots. Peripheral nerves show reduced numbers of large myelinated fibers, acute axonal degeneration at all levels, and distal axonal atrophy. Motor end-plates reveal small or absent nerve terminals. Subclinical non-motor system involvement includes neuronal loss in Clarke's nucleus and dorsal root ganglia, degeneration of non-motor tracts in the spinal cord, loss of receptors in the dorsal horns of the spinal cord, and myelinated fiber loss with segmental demyelination in sensory and mixed nerves. The serious implications of the diagnosis of ALS make it mandatory to exclude similar potentially treatable disorders. Management should be multidisciplinary, and discussions with the patient and family members should be frank and frequent. Discussions about ventilatory support should take place early in the disease so that death from respiratory failure can be prevented, when that is desired, and conversely to obviate the discontent and anger that accompany involuntary life on a ventilator.
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PMID:Amyotrophic lateral sclerosis: Part 1. Clinical features, pathology, and ethical issues in management. 405 56

Responses of single muscle fibres to electrical stimulation of the tibial nerve trunk or of the intramuscular nerve twigs were detected in young volunteers without evidence of neurological disease. With suitably adjusted amplitude of the stimulus, clear-cut double distribution of the response latencies was obtained in some fibres. Experiments with two stimulating cathodes and with recordings from more than one muscle fibre in the same motor unit suggest that axon reflexes were involved. The results indicate that axonal branching normally occurs not only in the intramuscular course of the nerve but also outside the muscle, in some cases even rather high in the nerve trunk. The possibility is discussed that axon reflexes may underlie fasciculations evoked by neostigmine and those seen in some other conditions, such as amyotrophic lateral sclerosis, as well as muscle cramps in normal subjects.
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PMID:Demonstration of axon reflexes in human motor nerve fibres. 424 70


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