We report the case of an infant with Menkes' disease who presented wit
h moderately abnormal neurologic findings at birth and with extraaxial
bleeding in the posterior fossa, Early cerebellar atrophy and hypomye
lination were evident on magnetic resonance imaging at 5 weeks of age.
We suggest that neurodegeneration occurs earlier than has been previo
usly reported and may be identifiable even in neonates. (C) 1997 by El
sevier Science Inc. All rights reserved.