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April 1976

Ophthalmoplegia and Dissociated Nystagmus in Abetalipoproteinemia

Author Affiliations

From the Clinical Branch, National Eye Institute (Drs Yee and Cogan) and the Medical Neurology Branch, National Institute of Neurological Diseases and Stroke (Dr Zee), Bethesda, Md.

Arch Ophthalmol. 1976;94(4):571-575. doi:10.1001/archopht.1976.03910030265002

• A characteristic pattern of acquired exotropia, progressive paresis of the medial rectus muscles, and dissociated nystagmus on lateral gaze was found in three patients with abetalipoproteinemia.

Study with electronystagmography of the eye movements of one patient revealed abnormally slow voluntary saccades and slow or absent fast components of vestibular nystagmus, optokinetic nystagmus, and jerk-type, dissociated nystagmus. Defects in central nervous system centers generating saccadic eye movements are postulated.