Abstract

The brain's action-intentional ("when") programming system helps to control when to and when not to initiate an action, when to persist at an action, and when to terminate an action. Motor perseveration is a failure to terminate an action. This disengagement disorder most often results from dysfunction of the executive frontal-subcortical networks that control the action-intentional programming system. Reports of unilateral perseveration are unusual. Here we describe a patient with a form of progressive supranuclear palsy (PSP) who exhibited continuous right-hand motor perseveration. This 68-year-old right-handed man had impaired walking and vertical gaze, consistent with PSP. He often repeated words, and on many motor tasks he showed continuous perseveration of his right but not his left hand. Unilateral motor perseveration may be a sign of PSP, the corticobasal syndrome, or a subtype of these disorders. Future studies of patients with both disorders should use tasks that assess for asymmetric hand perseveration. The mechanism of the unilateral perseveration must also be determined. Bilateral perseveration is found most often in patients with unilateral right frontal-subcortical (basal ganglia) or insula dysfunction. Because patients with PSP or corticobasal syndrome have callosal degeneration, their unilateral perseveration might result from a callosal disconnection of the right frontal lobe from the left hemisphere's premotor and motor as well as speech areas.

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