Faculty Bibliography

The most common cause of death in patients with rapidly progressive neuromuscular disease has been respiratory failure. The medical community and society as a whole have not yet recognized the possibilities for markedly extended life expectancy and prolonged productivity in severely disabled neuromuscular patients with proper medical management and rehabilitation. In a population of 120 patients with early childhood onset, rapidly progressive muscular dystrophy, 29 were in the most advanced stage requiring mechanical ventilation 24 hr/day and had vital capacities of less than 10% predicted for age and height. Of these patients with severely weakened bulbar innervated muscles, 10 have been receiving positive pressure ventilation via indwelling tracheostomy tubes for an average of 3 years. The average age of the 29 patients is 27 years with a range of 15 to 54 years. Of the 29 patients, 24 live in the community. Three are married and have a total of 5 children. Some patients are in professional careers, or active in political and community organizations. The management of and potential for improved quality of life and increased longevity in these patients are discussed.

A 96-trial tachistoscope recognition task was given to 15 left-hemisphere-damaged, 12 right-hemisphere-damaged, and 30 non-brain-damaged subjects. Procedure called for a first stimulus presented for 250 msec, a 1-sec. pause, and a second stimulus for 250 msec. The stimuli were 12 animal drawings used in repeated series according to a random schedule. Six animals were familiar and six were novel. For half of the trials, the two pictures were of different animals. Size and position of the animal picture were controlled. Both reaction time and accuracy were independently scored. Judgments of same and different appear to function as if they are governed by different processes. For the control group, measures of same and different judgments do not correlate highly despite high internal consistency of subtests. Recognition of same becomes impaired with brain damage, but more so if the damage is rightsided. Recognition of different judgments shows considerably less sensitivity to the effects of unilateral brain damage

A man with a completely transected spinal cord and a spastic neurogenic bladder was conditioned to void upon the presentation of an external stimulus. A classical conditioning paradigm was employed in which strong abdominal shock (unconditioned stimulus) was paired with an initially neutral mild electrical stimulation of the thigh (conditioned stimulus; CS). After the pairing trials, a reliable conditioned response of urination was elicited by the CS alone. The conditioned response did not extinguish over time, and the procedure left the bladder with clinically safe residual amounts of urine. Practical implications of the conditioning technique for the treatment of spastic neurogenic bladder conditions in spinally injured patients are discussed, as is the theoretical significance of conditioning at the reflex level in the absence of cortical involvement.