Familial congenital fourth cranial nerve palsy
W. F. Astle and A. L. Rosenbaum
Few reports of hereditary transmission patterns in congenital superior
oblique palsy have been made in the past. In this study, three families
with congenital superior oblique palsies have been identified and examined.
Two members of each family had a superior oblique palsy. After full
history, eye examination, and sensory testing, the patterns in each family
have been analyzed. Deviations varied from binocular fusion and orthophoria
to large vertical deviations, especially in adduction. Sensory patterns
were compatible with the congenital nature of the palsy. A discussion of
possible causes for a congenital superior oblique palsy is offered and the
congenital superior oblique palsies are analyzed. A definite genetic
transmission pattern could not be found, but possible explanations are
considered. Regardless of etiology, therapy for this problem is unchanged
from that for all superior oblique palsies. Occurrences of congenital
trochlear palsies in multiple family members should be considered when a
patient with this problem is diagnosed.