
neuroaxonal dystrophy in congenital
biliary atresia
neuroaxonal dystrophy has been described in 8 patients with congenital
biliary atresia.  the characteristic changes include axonal dystrophy, a mild
loss of nerve cells and a variable degree of astrocytic reaction, mainly in the
gracile, cuneate and trigeminal nerve nuclei in the medulla, and rarely in
other parts of the central nervous system.
the neuroaxonal dystrophy in these patients is remarkably similar to that
in patients with prolonged mucoviscidosis and in experimental vitamin
e-deficient rats.
it is strongly suggested that the neuroaxonal dystrophy in these patients
with congenital biliary atresia is a manifestation of prolonged vitamin e
deficiency.
the neuroaxonal dystrophy in these patients is also similar to that seen
in aging and bears resemblance to the axonal change encountered in a group
of heredo-degenerative diseases, the various forms of "hallervorden-spatz
disease".  the pathological similarity between these diverse conditions
warrants further investigation as to the possible metabolic
relationship between them.
