Unilateral Hyperkinetic Choreiform Movements due to Calcification of the Putamen and Caudate from an Underlying Developmental Venous Anomaly

Abstract

This index case report describes a patient who presented with unilateral hyperkinetic choreiform movements of the left neck, arm, and leg caused by right-sided putamen and caudate calcification as the product of an underlying developmental venous anomaly (DVA). No underlying metabolic disorder or other calcium-related disorder was present. Calcification of the putamen and caudate has been described in relation to an underlying DVA which results in localized venous hypertension and other changes, and tends to spare the anterior limb of the internal capsule. This resulting unilateral choreiform movement disorder has not been described in the literature and represents the need for greater recognition of this entity in the differential for lateralizing hyperkinetic disorders.

Hyperkinetic Choreiform Movements Secondary to Basal Ganglia Calcification and Underlying Developmental Venous Anomaly

“…We found only one other reported case of similar movements associated with cerebral calcifications and DVA [3]. After a review of the success of clonazepam therapy in that reported case and associated literature, it was deemed appropriate to also initiate a trial in our patient, with almost near-resolution of symptoms observed.…”

“…Symptomatic unilateral chorea has prior only been reported in the setting of acute stroke. Although basal ganglia calcifications associated with DVA have been reported in the literature, there is only one other report of a concurrent chorea-like movement disorder [3].…”

“…Vascular causes have been presented, and chorea has been seen as one of the most common movement disorders presenting after strokes, particularly when the subthalamic nucleus is involved [1]. Unilateral chorea, in particular, has been associated with vascular causes, such as arteriovenous malformations and subdural hematomas, in addition to strokes [2,3].…”

“…The basal ganglia play a crucial role in the development of hyperkinetic movements through an imbalance of direct and indirect pathways [1]. Unilateral hyperkinetic movements associated with underlying pathology of the basal ganglia are primarily reported in conjunction with prior strokes [3]. Acute onset without any of the underlying risk factors mentioned above has not been thoroughly established.…”

“…Prior case reports primarily associate them with strokes, with only one prior report of an associated movement disorder. It has been hypothesized that hypertension in the venous network in the areas drained by DVA's can contribute to encephalomalacia and gliosis, leading to eventual calcification [3].…”