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Pendular nystagmus in patients with peroxisomal assembly disorder. Arch Neurol 1998 Apr;55(4):554-8

Date

04/30/1998

Pubmed ID

9561985

DOI

10.1001/archneur.55.4.554

Scopus ID

2-s2.0-0031896987   16 Citations

Abstract

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 degrees to 80 degrees 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.

Author List

Kori AA, Robin NH, Jacobs JB, Erchul DM, Zaidat OO, Remler BF, Averbuch-Heller L, Dell'Osso LF, Leigh RJ, Zinn AB

Author

Bernd F. Remler MD Professor in the Neurology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adolescent
Child
Child, Preschool
Eye Movements
Female
Genes, Recessive
Humans
Male
Nystagmus, Pathologic
Optic Atrophy
Peroxisomal Disorders
Vision Screening