Reactive neuroaxonal dystrophy in children

1432-0533

Neuroaxonal dystrophy

Springer Online Journal Archives 1860-2000

Medicine

Summary The aim of the present study was to determine the frequency and etiological factors of neuroaxonal dystrophy (N.D.) in brainstem and spinal cord of children with various non-neurological diseases. The material used in this study consisted of 266 consecutive autopsies from 1974–1976 and an additional 13 cases from previous years. By far, the most common cause of N.D. in children was chemotherapeutic drugs, particularly vincristine, used for the treatment of malignant tumors. Approximately 90% of these children developed N.D. and the changes ranged from mild to severe. Approximately two-thirds of children with cystic fibrosis, congestive heart failure and chronic renal failure developed N.D. and the changes were mostly mild. Only one-third of patients with congenital biliary atresia and malnutrition developed N.D. and the changes were always mild. The frequency and severity of N.D. increased in patients who had prolonged clinical courses; no N.D. was seen in patients who died from acute causes such as trauma, acute infection, intoxication, acute renal failure or prematurity. This type of N.D. may be considered as reactive to a wide variety of injurious factors such as drug toxicity, malnutrition, chronic hypoxia and chronic renal failure, either alone or in combination. In contrast to previous reports of a low incidence of N.D. in children, there has been a sharp increase in recent years due to the advent of chemotherapy.

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