Dysphagia in patients with the post-polio syndrome

Article Abstract:

Post-polio syndrome can occur 25 to 30 years after an original attack of poliomyelitis. Slowly progressive weakness in the muscles that were originally affected, and sometimes in those that were not, creates new problems with the performance of daily activities. In addition to weakness affecting the limbs, there are reports of weakness involving the muscles of the oropharynx (bulbar muscles, in the mouth and throat). Weakness of these muscles may cause potentially fatal choking or aspiration pneumonia, caused by food accidentally going down the trachea (windpipe) and lodging in the lung. Among patients who have had polio, the motor neurons (nerves) are chronically overstressed, and eventually can no longer support the repaired muscle fibers. The process is detectable by electromyography (measurement of the electrical activity of a muscle) or muscle biopsy. However, these tests cannot be used for the bulbar muscles, and the first indication that something is wrong may be a bout of choking or the loss of the ability to speak. To find a method of determining whether the muscles were affected, the mouth and throat musculature of 32 patients with post-polio muscular atrophy was examined. Fourteen had trouble swallowing, the other 18 did not. The objective was to identify cases of bulbar involvement so that steps could be taken to avoid potentially life-threatening choking or aspiration of food or drink. Ultrasonography and videofluoroscopy showed that patients, whether or not they had symptoms, often had evidence of abnormalities when swallowing, even if they had no bulbar involvement when they originally had polio. Aspiration of food into the trachea was rare for all patients. In limb muscles the new weakness cannot be measured accurately, but bulbar weakness can now be defined and scored objectively. For those who had trouble swallowing, training in position, changing the diet, eating soft-to-medium consistency foods, and learning compensatory techniques can help them to avoid problems. (Consumer Summary produced by Reliance Medical Information, Inc.)

Poliomyelitis

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Swallowing dysfunction in nephropathic cystinosis

Article Abstract:

Nephropathic cystinosis is an error of metabolism that prevents the normal biochemical breakdown of the amino acid cystine. In patients who suffer from this condition, there is a gradual accumulation of cystine in virtually all tissues of the body. The accumulation in the kidney produces generalized renal failure, usually by about the 10th year of life. Recently it has been found that treatment with cysteamine (a related compound) can be used to deplete the cellular concentration of cystine and prevent or slow the progress of the disease. Before this treatment was available, approximately 70 patients in the United States underwent a kidney transplant for the condition. Because the kidney contains cells from the donor, who has normal cystine metabolism, the transplanted kidneys are free of the damage caused by the accumulation of cystine. A group of 43 patients with cystinosis were included in this study of age-related swallowing dysfunction. Twenty-four of this group had received a new kidney by transplantation. The ability of the patients to swallow was assessed by several techniques that allowed the swallowing process to be observed. Approximately half of the patients were found to eat slowly, a condition which may related to an underlying pathology. Seven of the nine patients in the 21- to 31-year-old age group were found to have detectable difficulties with all three phases of swallowing (oral, pharyngeal and esophageal), apparently because of reduced muscular abilities. The degree of swallowing difficulties appears to be related to length of time that the patient has had the disease, indicating that swallowing dysfunction is a late consequence of the disease. Various strategies can be used to aid patients with swallowing problems, including changing the consistency of food and learning new strategies, such as placing the food to be swallowed far back in the mouth. Long-term cysteamine therapy may also be useful for patients with cystinosis who have swallowing difficulties. (Consumer Summary produced by Reliance Medical Information, Inc.)

Kidneys, Kidney transplantation

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Improved renal function in children with cystinosis treated with cysteamine

Article Abstract:

Diagnosis and treatment of cystinosis before age two appears to improve kidney function. Cystinosis is an inherited disease caused by the impaired transport of the amino acid cystine. Cell lysosomes store cystine, causing crystal formation and tissue damage. Typically, people with cystinosis die before age 10 unless they receive dialysis or a kidney transplant. Of 76 children with cystinosis, 17 received adequate treatment with cystamine and were diagnosed before age two, 32 received partial treatment with cystamine and 27 never received treatment with cystamine. During follow-up, none of the children who had been adequately treated had impending kidney failure compared with 66% of the children who had been partially treated and 59% of the children who had never been treated. Using data from urine analysis, kidney failure was predicted to occur at age 10 for children with untreated cystinosis, at age 20 for children with partially treated cystinosis and at age 74 for children with adequately treated cystinosis.

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