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
Cohen DA, Bosley TM, Savino PJ, Sergott RC, Schatz NJ. Primary Aberrant Regeneration of the Oculomotor Nerve: Occurrence in a Patient With Abetalipoproteinemia. Arch Neurol. 1985;42(8):821–823. doi:10.1001/archneur.1985.04210090089025
Customize your JAMA Network experience by selecting one or more topics from the list below.
Create a personal account or sign in to: