Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The immunohistochemical and ultrastructural characteristics of spinal cord neurofibrillary tangles (NFTs) were examined in Guamanian amyotrophic lateral sclerosis and in parkinisonism-dementia complex on Guam. The spinal cord NFTs reacted with antibodies to tau protein (tau-2), ubiquitin and paired helical filaments (PHFs). Ultrastructurally, the components of the NFTs were seen as randomly arranged fibrils which were often associated with osmiophilic granules; small bundle-like arrangements were also occasionally observed. Individual NFT fibrils appeared as straight fibrils with a diameter of approximately 15 nm and constricted fibrils with a periodicity of approximately 80 nm. Ultrastructural microscopic examination of specimens stained by the modified Bielschowsky method and with the antibodies revealed silver particles and the products of the tau, ubiquitin and PHF immunoreactions on the NFT fibrils. This is the first demonstration of the fine structure of the spinal cord NFTs.
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PMID:Ultrastructural identification of neurofibrillary tangles in the spinal cords in Guamanian amyotrophic lateral sclerosis and parkinsonism-dementia complex on Guam. 155 58

The aberrant pyramidal tracts in the pontine medial lemnisci were studied, using standard Sudan III stain, in six cases of chronic pyramidal tract degeneration. Three of the six cases had bilateral or unilateral cerebral destructive lesions, one cervical hematomyelia with rare retrograde pyramidal tract degeneration, one classical amyotrophic lateral sclerosis, and one atypical motor neuron disease with striatonigral degeneration. Except for the latter two cases the aberrant pyramidal tract degeneration was confirmed bilaterally or on the side ipsilateral to the pyramidal tract degeneration in the pontine base. This degeneration could also be found, on careful examination, with other stains, i.e., H & E, Luxol fast blue-periodic acid Schiff and modified Bielschowsky. Significant change was not observed in the medullary medial lemniscus in any case. The different results observed in the aberrant pyramidal tract between the destructive and degenerative disorders might be pathogenetically important. Reservation, however, may be required since the number of the cases of degenerative disorders in this study was limited. A possible factor for this difference is the survival length which might have erased degradation products altogether. Another factor is the sensitivity of Sudan III in comparison with the Marchi's method which might demonstrate more subtle evidence of degeneration but with its intrinsic capricious staining characteristics. The physiological role of the aberrant pyramidal tract, which has been neglected in the recent textbooks of neuroanatomy, may become of clinical interest with high-quality MRI in cases such as isolated cranial motor nerve palsy without concomitant paralysis of the extremities.
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PMID:[Aberrant pyramidal tract: a study with Sudan III stain]. 280 32