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Volume 10, Issue 2, Pages 135-142 (April 2006)


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High-Resolution Magnetic Resonance Imaging Demonstrates Abnormalities of Motor Nerves and Extraocular Muscles in Patients With Neuropathic Strabismus

Presented at the AAPOS Meeting Orlando Florida March, 2005.

Joseph L. Demer, MD, PhDabcdCorresponding Author Informationemail address, Maria Carolina Ortube, MDa, Elizabeth C. Engle, MDef, Neepa Thacker, MDa

Received 9 March 2005; accepted 18 November 2005.

Introduction: Although the ocular motility examination has been used traditionally in the diagnosis of strabismus that is a result of cranial nerve (CN) abnormalities, magnetic resonance imaging (MRI) now permits the direct imaging of lesions in CN palsies. Methods: Prospectively, nerves to extraocular muscles (EOMs) were imaged with T1 weighting in orbits of 83 orthotropic volunteers and 96 strabismic patients in quasicoronal planes using surface coils. Intraorbital resolution was 234–312 microns within 1.5- to 2.0-mm thick planes. CNs were imaged at the brainstem using head coils and T2 weighting, yielding 195 micron resolution in planes 1.0-mm thick in 6 normal volunteers and 22 patients who had oculomotor (CN3), trochlear (CN4), or abducens (CN6) palsies and Duane syndrome. Results: Oculomotor (CN3) and abducens (CN6) but not trochlear (CN4) nerves were demonstrable in the orbit and skull base in all normal subjects. Patients with congenital CN3 palsies had hypoplastic CN3s both in orbit and skull base, with hypoplasia of involved EOMs. Patients with chronic CN6 and CN4 palsies exhibited atrophy of involved EOMs. Patients with Duane syndrome exhibited absence or hypoplasia of CN6 in both orbit and brainstem regions, often with mild hypoplasia and apparent misdirection of CN3 to the lateral rectus muscle. Unlike CN6 palsy, patients with Duane syndrome exhibited no EOM hypoplasia. Patients with congenital fibrosis exhibited severe hypoplasia of CN3, moderate hypoplasia of CN6, and EOM hypoplasia, particularly severe for the superior rectus and levator muscles. Conclusion: High-resolution MRI can directly demonstrate pathology of CN3 and CN6 and affected EOM atrophy in strabismus caused by CN palsies. Direct imaging of CNs and EOMs by MRI is feasible and useful in differential diagnosis of complex strabismus.

a Department of Ophthalmology, Jules Stein Dye Institute, University of California, Los Angeles, California

b Department of Neurology, Jules Stein Dye Institute, University of California, Los Angeles, California

c Bioengineering Interdepartmental Program, Jules Stein Dye Institute, University of California, Los Angeles, California

d Neuroscience Interdepartmental Program, Jules Stein Eye Institute, University of California, Los Angeles, California

e Department of Medicine (Genetics and Genomics) and Neurology, Children’s Hospital Boston, Boston, Massachusetts

f Harvard Medical School, Boston, Massachusetts

Corresponding Author InformationReprint Requests: Joseph L. Demer MD, PhD, Jules Stein Eye Institute, 100 Stein Plaza UCLA, Los Angeles CA 90095-7002

 Supported by grants from the National Eye Institute: EY08313, EY13583, and EY00331. Also supported by an unrestricted grant from Research to Prevent Blindness. Joseph L. Demer is the Leonard Apt Professor of Ophthalmology.

PII: S1091-8531(05)00350-2

doi:10.1016/j.jaapos.2005.12.006


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