[The clinico-pathological findings of the patients grouped as respiratory motor paralysis preceded type of ALS].
We reported five autopsied patients grouped as respiratory motor paralysis preceded type of ALS (R-ALS) classified as one clinical type of ALS from the patients living beyond respiratory failure reported in the previous paper, in which ALS the ontogenetically new motor functions in the "first motor system" proposed by Holstege involved first. The neuropathological changes of five patients were not limited in the degeneration of conventional "motor system" such as corticospinal tract and spinal and cranial motoneurons, but extended into the realm of "non-motor system", which could show the anterolateral myelin pallor in spinal cord by myelin staining, and detect the fibrillary gliosis of the anterior commissural area, the spinal grey matter of intemediate zone and anterior horn in all patients, and of medullary reticular formation in three one by Holzer staining. These pathological lesions could be included in the "first motor system". Considered from this study of the clinico-pathological findings of R-ALS based on "a new view of ALS", which respiratory failure in ALS is not terminus in ALS and total course of ALS might be TLS, it might be concluded that the clinico-pathologically involved initial lesions of ALS might be in the "first motor system", and include not only degenerations of "motor system", but also those of "non-motor system", which is the basically pathological lesions in ALS. Furthermore, the hitherto clinico-pathological ALS findings, which respiratory failure in ALS is terminus in ALS, could be included in this group, and showed as a part of ALS discussed from this study. Total clinico-pathological on ALS from the initial ones (R-type) to the advanced ones (TLS) including ALS-dementia should be considered further based on "the second and the third motor systems in addition to the first one".
Hayashi
H
H
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.
Kato
S
S
Oda
M
M
D000328:Adult; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology* / Q000503:physiopathology; D005260:Female; D005911:Gliosis / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009046:Motor Neurons / Q000473:pathology; D012133:Respiratory Paralysis / Q000209:etiology / Q000473:pathology*; D012154:Reticular Formation / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology