Reactive neuroaxonal dystrophy in children. Clinical pathological correlation.

The aim of the present study was to determine the frequency and 
etioloical factors of neuroaxonal dystrophy (N.D.) in brainstem and spinal cord 
of children with various non-neurological diseases. The materials 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 cases 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. 