[Skip to Navigation]
August 1985

Primary Aberrant Regeneration of the Oculomotor Nerve: Occurrence in a Patient With Abetalipoproteinemia

Author Affiliations

From the Neuro-ophthalmology Service (Drs Bosley, Savino, Sergott, and Schatz), Wills Eye Hospital, and the Departments of Neurlogy (Drs Cohen, Bosley, Savino, and Schatz) and Opthalmology (Drs Bosley, Savino, Sergott, and Schatz), Hospital of the University of Pennsylvania, Philadelphia.

Arch Neurol. 1985;42(8):821-823. doi:10.1001/archneur.1985.04210090089025

Primary aberrant regeneration of the oculomotor nerve is a clinical syndrome involving eyelid, pupil, and extraocular movement abnormalities following damage to the peripheral portion of the oculomotor nerve. It has been described only with meningiomas and internal carotid artery aneurysms within the cavernous sinus.1-6 We describe herein a patient with abetalipoproteinemia (Bassen-Kornzweig syndrome [BKS]) who has bilateral primary aberrant regeneration of the oculomotor nerve.

REPORT OF A CASE  A 31-year-old right-handed man was diagnosed as having BKS at age 9 years because of acanthocytosis and serum β lipoprotein levels less than 4% of normal7. Visual fields and funduscopic examination findings were normal, and visual acuity was 6/15 - 1 OD and 6/15 + 1 OS uncorrected. Extraocular movements and pupillary reaction were normal at that time, but he had moderate bilateral ptosis, greater on the right. He required assistance to walk, and his gait was broad based and ataxic. At age

Add or change institution