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April 1998

Pendular Nystagmus in Patients With Peroxisomal Assembly Disorder

Author Affiliations

From the Departments of Neurology (Drs Kori, Zaidat, Remler, Averbuch-Heller, Dell'Osso, and Leigh), Biomedical Engineering (Messrs Jacobs and Erchul and Drs Dell'Osso and Leigh), and Genetics (Drs Robin and Zinn); the Center for Human Genetics (Drs Robin and Zinn), University Hospitals of Cleveland; and Veterans Affairs Medical Center, Case Western Reserve University, Cleveland, Ohio.

Arch Neurol. 1998;55(4):554-558. doi:10.1001/archneur.55.4.554

Background  Pendular nystagmus commonly occurs in congenital and acquired disorders of myelin.

Objective  To characterize the nystagmus in 3 siblings with an infantile form of an autosomal recessive peroxisomal assembly disorder causing leukodystrophy.

Design  We examined visual function and measured eye movements using infrared oculography. We noted changes in eye speed and frequency before and after the administration of gabapentin to 1 patient.

Results  All 3 siblings showed optic atrophy and pendular nystagmus that was predominantly horizontal, at a frequency of 3 to 6 Hz, with phase shifts of 45° to 80° between the oscillations of each eye. Gabapentin administered to 1 child caused a modest improvement of vision and the reduction of the velocity and frequency of oscillations in the eye with worse nystagmus.

Conclusion  The pendular nystagmus in these patients was due to their leukodystrophy and may have a similar pathogenesis to the oscillations seen in other disorders affecting central myelin.