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Clinicopathological Study of Two Respirator‐assisted Long Survival Cases of Amyotrophic Lateral Sclerosis
Author(s) -
Hashizume Yoshio,
Yoshida Mari,
Murakami Nobuyuki
Publication year - 1993
Publication title -
neuropathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.701
H-Index - 61
eISSN - 1440-1789
pISSN - 0919-6544
DOI - 10.1111/j.1440-1789.1993.tb00202.x
Subject(s) - amyotrophic lateral sclerosis , medicine , upper motor neuron , degeneration (medical) , pyramidal tracts , pathology , anatomy , physical medicine and rehabilitation , disease
This report concerns the evaluation of the clinicopathological features of amyotrophic lateral sclerosis (ALS) in two male patients who survived ten years with the assistance of artificial respirators. The patients were 54 and 52 years old at disease onset and they were placed on respirators 32 and 27 months later, respectively. The patients did not show impairments in occulomotor, sensory and rectovesical functions nor develop bedsores, with the tetrad of ALS being negative. The main histopathological changes observed include neuron loss of the upper and lower motor systems and degeneration of the pyramidal tract. By contrast, other systems such as the posterior column, spinocerebellar tracts, Clarke's nucleus, oculomotor nucleus and Onuf's nucleus remained intact. These findings on our two patients suggests that there may be cases in which the selective features of ALS are maintained throughout prolonged respiratory management.