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Ji Soo Kim, MD, PhD; Sung-Ho Park, MD; Kwang-Woo Lee, MD

Arch Neurol. 2003;60:1621-1624.

Background  Spasmus nutans and congenital ocular motor apraxia share clinical characteristics. However, their development in a patient with cerebellar vermian hypoplasia has not been previously described.

Objective  To report spasmus nutans and congenital ocular motor apraxia in a child with cerebellar vermian hypoplasia.

Design  Case report.

Setting  Tertiary-care hospital.

Patient  A 7-year-old boy with a history of spasmus nutans during infancy and developmental delay was referred for the evaluation of abnormal head and eye movements.

Results  The patient had impaired voluntary saccades and smooth pursuit in the horizontal plane and showed thrusting movements of the head during attempted gaze shift. Magnetic resonance imaging of the brain demonstrated cerebellar vermian hypoplasia, especially in the inferior portion.

Conclusions  Spasmus nutans and congenital ocular motor apraxia may develop in patients with cerebellar vermian hypoplasia. In patients with congenital ocular motor apraxia, a history of spasmus nutans should be sought, and careful evaluation of the cerebellar vermis is needed during brain imaging.

From the Department of Neurology, College of Medicine, Seoul National University, Seoul, South Korea.