Pendular nystagmus in patients with peroxisomal assembly disorder. Arch Neurol 1998 Apr;55(4):554-8
Date
04/30/1998Pubmed ID
9561985DOI
10.1001/archneur.55.4.554Scopus ID
2-s2.0-0031896987 (requires institutional sign-in at Scopus site) 19 CitationsAbstract
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 ABAuthor
Bernd F. Remler MD Professor in the Neurology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdolescentChild
Child, Preschool
Eye Movements
Female
Genes, Recessive
Humans
Male
Nystagmus, Pathologic
Optic Atrophy
Peroxisomal Disorders
Vision Screening
