The amyotrophic lateral sclerosis (ALS) severity scale has been developed to provide an ordinal staging system and a means of rapid functional assessment for patients with ALS. The scale allows an examiner to evaluate the symptoms of ALS numerically in four categories that describe speech, swallowing, lower extremity, and upper extremity abilities. These scores, combined with a vital capacity measured on a hand-held respirometer, provide a rapid, accurate assessment of a patient''s disease status and can be used for treatment planning. The ALS severity scale has been shown to have an average estimated reliability coefficient of 0.95 between examiners. Speech ratings were correlated >0.80 for objective speech measures. Rates of progression of the total score in a small group of patients ranged from –3.4 to –24.0 points/year with a mean of –11.3 points/year.

No abstract

No abstract

The natural progression of amyotrophic lateral sclerosis (ALS) leads to respiratory failure and death. Artificial ventilation can prolong the course, leading to extreme degrees of weakness and dependence. Little specific information is available to counsel ALS patients about making the decision for artificial ventilation. In order to gain more information, we visited four ventilator-dependent ALS patients and their primary caregivers. We determined the neurologic state and level of function of the patients and interviewed their primary caregivers to assess medical care and management needs (both social and financial) and how they were being met. We also administrated questionnaires to assess the psychological well-being of both patient and primary caregiver and how the relationship between the patient and primary caregiver changed under these circumstances.

No abstract