Augusta Alba

PULMONARY DYSFUNCTION AND SLEEP DISORDERED BREATHING AS POST-POLIO SEQUELAE: EVALUATION AND MANAGEMENT

Post-polio sequelae can include sleep disordered breathing and chronic alveolar hypoventilation (CAH). Both conditions develop insidiously and can render the post-polio survivor susceptible to cardiopulmonary morbidity and mortality when not treated in a timely and appropriate manner. These conditions can be diagnosed by a combination of spirometry, noninvasive blood gas monitoring, and ambulatory polysomnography. Sleep disordered breathing is most frequently managed by nasal continuous positive airway pressure, while tracheostomy ventilation is the most common treatment for ventilatory failure. We report the more effective and comfortable techniques recently made available for managing sleep disordered breathing and the use of noninvasive treatment options for CAH, respiratory failure, and impaired airway clearance mechanisms. One hundred forty-three cases are reviewed.

Survival of individuals with spinal muscular atrophy on ventilatory support

Six ventilator-assisted individuals (VAIs) with spinal muscular atrophy (SMA) are described. All six survived by using intermittent positive pressure ventilation via an indwelling tracheostomy for a mean of 11.7 +/- 17.7 yr despite frequent episodes of mucus plugging and pneumonia. Four of the VAIs also received all nutrition via indwelling gastrostomy tubes because of severe bulbar muscle weakness. Four VAIs used tracheostomy intermittent positive pressure ventilation with their tracheostomy cuffs deflated and could communicate verbally. Five of the six VAIs remained institutionalized from the onset of ventilatory use. Two SMA VAIs survived for 15 and 4 yr, respectively, despite need for ventilatory support since early infancy. All four SMA VAIs who could communicate remained socially active and one, gainfully employed. We conclude that for patients with advanced SMA markedly prolonged survival is possible with ventilatory assistance despite severe respiratory and bulbar muscle dysfunction.

A clinical disability rating for Parkinson patients

Abstract A clinical rating scale for the motor aspects of Parkinson disability has been developed. This scale makes it possible to relate the functional disability of the patient with the variables of general mobility at rest, rigidity, contractures and deformities, and tremor. The interrelationship of the variables can also be studied. The scale permits quick comparison of the ratings of an individual patient with the composite ratings of a group of patients. Differences in laterality of function can be assessed. The importance of psychotropic drugs and methocarbamol in the control of tremor is supported by this scale. No statistical difference in the scores of the patients on high doses of anti-Parkinson medications as compared to low doses could be found. Pulmonary functions showed distinct correlations with the total score. Relationships with the individual variables of the score proved of greater importance in determining how pulmonary functions are affected by the Parkinsonians disability.

Influence of age on rehabilitation outcomes and survival in post-acute inpatient cardiac rehabilitation.

PURPOSE To investigate the relationship of increasing age to clinical characteristics, rehabilitation outcomes, and long-term survival in a post-acute inpatient cardiac rehabilitation program. METHODS The study population consisted of all 364 consecutive cardiac rehabilitation patients admitted over a 4-year period to an inpatient cardiac rehabilitation program in a long-term acute care hospital.Admission and discharge comparisons were made between 3 age cohorts: 65 years (n = 117), 65 to 74 years (n = 127), and ≥ 75 years (n = 120). Patients were followed through January, 2010 for survival. RESULTS The 3 cohorts on admission differed significantly in Functional Independence Measure, estimated Glomerular Filtration Rate, smoking and hypertension histories, body mass index, and cardiac diagnoses (all P < .05) but not in Simplified Acute Physiology Score II, Cumulative Illness Rating Scale for Geriatrics, or left ventricular ejection fraction. There were no cohort differences in rehabilitation outcomes of physical function, inpatient days, and discharge disposition. Survival was longest in the youngest cohort whereas the 2 older cohorts had similar survivals (P < .01; log-rank test). All 3 cohorts had at least 40% survival at 8 years. Cox regression analyses showed that the comorbidity burden as quantified by the Cumulative Illness Rating Scale for Geriatrics was the only predictor of death in all cohorts (all P ≤ .002). CONCLUSIONS This study provided evidence that post-acute inpatient cardiac rehabilitation programs equally benefited both elderly patients and younger patients. These programs are valuable in the continuum of care for elderly patients who are not yet ready for discharge to home following a serious cardiac event.

Chronic ventilator use in osteogenesis imperfecta congenita with basilar impression: A case report

Osteogenesis imperfecta, a rare connective tissue disorder, is known to be associated sometimes with the invagination of the basilar skull. This deformity may disturb respiratory function secondary to brain stem compression and hydrocephalus. In addition, the deformed thoracic cage and fragile ribs make pulmonary care more complicated. A case of 24-year-old man is presented with brain stem compression syndrome secondary to osteogenesis imperfecta congenita with basilar impression. He developed respiratory failure and became tracheostomy positive-pressure ventilator dependent at the age of 21 years. He also suffered multiple skeletal abnormalities and mental retardation, and following the brain stem compression, severe quadriparesis. The patients condition is stable since he has been using the ventilator and he is currently living in the community.

Conditioned responding of the neurogenic bladder.

&NA; Classical conditioning techniques were employed to condition responding of the spastic neurogenic bladder in a man with quadriparesis and urinary incontinence secondary to spinal cord injury at the cervical level. A neutral stimulus of mild electrical stimulation to the thigh was paired temporally with an unconditioned stimulus (UCS) of stronger electrical stimulation of the lower abdomen, and then was presented alone as a conditioned stimulus (CS) to elicit the conditioned response (CR) of voiding. The previously neutral CS reliably elicited large amounts of urine and left little residual urine in the subjects bladder. Following the experimental sessions, the subject self‐applied the CS on a predetermined schedule during his daily routine outside of the laboratory. The CS initially was successful, but after several days the CR exhibited extinction. Additional CS‐UCS pairing sessions did not reinstate the responses satisfactorily. Aspects of the experimental procedure and the results are discussed as well as the feasibility of conditioning the human spinal cord in the absence of an intact central nervous system

RECOGNITION OF MATCHING TASKS AND STIMULUS NOVELTY AS A FUNCTION OF UNILATERAL BRAIN DAMAGE

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.

The primary role of the Erb's point-axilla segment in median and ulnar motor nerve conduction determinations in alcoholic neuropathy

Motor nerve conduction velocity (MNCV) was explored in the Erbs point-axilla (N-A) nerve segment of median and ulnar nerves, bilaterally, in 10 patients with a history of prolonged heavy drinking but in whom no other predisposing factors to peripheral neuropathy were found. For comparison, MNCV was determined also in the axilla-elbow (A-E), elbow-wrist (E-W) nerve segments, as well as the motor terminal latency (MTL) of the same nerves. A total of 140 nerve segments were tested tested but only 133 results were obtained. Abnormal MNCV or MTL was found in 36 or 27% of all tested nerve segments. From the latter, 38.9% were in the N-A nerve segments. Of N-A nerve segments tested, reduction in MNCV was found in 46.7%. Our results are statistically significant.