Isolated inferior division oculomotor nerve palsy (ONP) is rare. Acquired cases have been associated with neurologic or systemic disease. To our knowledge, congenital inferior division ONP is previously unreported. We present a case of congenital inferior division ONP in which magnetic resonance imaging demonstrated the structural neuropathy.A 21-year-old woman presented with lifelong largeangle exotropia and diminished vision acuity in her right eye (OD). She denied diplopia, periocular pain, cephalgia, migraine, head trauma, or prior surgery. Birth history was unremarkable. She had no history of diabetes mellitus, hypertension, systemic vasculopathy, or thyroid disease. She has three unaffected brothers and four unaffected sisters. General physical and neurologic examinations were normal. Cranial nerves were normal except for ocular motility. Cycloplegic refraction was ϩ4.00 ϩ 2.75 ϫ 150 OD and ϩ0.50 ϩ 0.50 ϫ 90 left eye (OS). Corrected visual acuity was 20/80 OD and 20/25 OS. Ptosis was absent. Both pupils were 7 mm in diameter with slight peaking toward 9:00 OD and were normally reactive to light and accommodation. The ocular versions are shown in Figure 1. At near with correction, there was 100 ⌬ exotropia with 10 ⌬ right hypertropia. Head tilt did not appreciably alter the hypertropia, but dissociated vertical deviation was present bilaterally. Abducting and vertical saccades were normal for both eyes (OU), while adducting saccades were bilaterally slow. Convergence was absent. There was bilateral optic disk pallor with cup-to-disk ratio of 0.5 OD and 0.3 OS. There was incyclotorsion OU on fundus examination.High-resolution magnetic resonance imaging (MRI) performed with orbital surface coils identified a smaller inferior division of the oculomotor nerve (OMN) OD than OS, with no identifiable motor nerve to the right medial (MR) and inferior rectus (IR) muscles, both of whose deep portions exhibited marked atrophy and abnormal internal bright signal on T1 imaging (Figure 2). The abducens nerve was readily demonstrable OU. The inferior oblique muscle was present bilaterally, but both lateral rectus muscle paths were superiorly displaced relative to the MR muscles ( Figure 2). Just posterior to the globe, right and left optic nerve diameters were 3.07 and 3.05 mm, respectively (Figure 2), both subnormal compared with published values. 1 Imaging with a head coil demonstrated bilateral OMN hypoplasia in the skull base region, with the right OMN too small to resolve in any of the 1 mm thickness contiguous image planes obtained in the plane of its path (Figure 3). These anatomic findings indicate bilateral but asymmetrical OMN abnormality.
DiscussionThe OMN divides into superior and inferior divisions just before entering the superior orbital fissure. Classical signs of inferior division OMP were evident here, including large exotropia, hypertropia, incyclotropia, and discoria. Although pupillary light responses were spared, Good and coworkers have postulated that early embryologic damage could partially spare th...