THE CLINICAL PICTURE OF LATERAL AMYOTROPHIC SCLEROSIS IN RESUSCITATED PATIENTS

The authors provide the data on lateral amyotrophic sclerosis (LAS) in 11 patients whose life was extended during respiratory failure by respiratory resuscitation and intensive care. The disease was established to run a cyclic course. The following clinical disease periods were distinguished: period of precursors, local motor disorders period, and period of initial and marked generalization. De-efferentation was the final period. The latter one was characterized by the arrest of all motor functions, namely by total paralysis with the exception of the function of oculomotor nerves. Motoneurons of the truncus cerebri nuclei disintegrated much more slowly than those of the spinal cord. The phase of de-efferentation lasted 8 years whereas the total disease period reached 23 years. Intellectual functions were preserved whatever the disease periods. Slow progress of motoneuronal disintegration enables using the respiratory systems both inpatiently and at home. Respiratory resuscitation and intensive care can be employed, if the patient and his relatives give their consent.