가족력이 없는 조화운동불능 동반 선천안운동행위상실증

Abstract

Purpose: To evaluate the natural clinical course of sporadic congenital oculomotor apraxia associated with ataxia.

Methods: In a retrospective study from June 1994 to March 2004, 3 patients with sporadic congenital oculomotor apraxia associated with ataxia were evaluated. Oculomotor apraxia and ataxia were checked.

Results: The three patients with sporadic congenital oculomotor apraxia associated with ataxia showed a decrease in head thrust which can be found specifically in oculomotor apraxia, and an improvement of oculomotor apraxia and ataxia over the 10-year observation period.

Conclusions: A favorable natural clinical course can be expected in a case of sporadic congenital oculomotor apraxia associated with ataxia in the absence of any clinical evidence of inheritance or genetic abnormality.