Developmental Venous Anomaly Associated with Hemi-Parkinson’s Syndrome

Abstract

Objective and Importance: Developmental venous anomalies (DVAs) are common congenital anomalies of intracranial venous drainage. In general, it is felt that DVAs replace the normal venous drainage system and, unless associated with another lesion such as a cavernous malformation, should not produce symptoms. The present case raises important questions regarding the potential for DVAs to become symptomatic and the role of venous hypertension in this process.Clinical Presentation: We present the case of a 23-year-old woman who presented with rapidly progressive hemi-parkinsonism consisting of unilateral tremor, micrographia, gait difficulty, and cogwheel rigidity. MRI demonstrated a large DVA draining the region of the contralateral basal ganglia with associated high signal intensity on T2-weighted MR imaging and gadolinium enhancement. Arteriography demonstrated only the DVA with the trunk of the angioma draining forward toward the inferior petrosal sinus instead of into the deep venous system. A focal stricture of the main venous trunk was noted. Retrograde venography confirmed a tight stricture at the entrance of the venous trunk into the cavernous sinus.Conclusions: Although DVAs are generally considered benign variants of normal venous drainage, it is possible that in select cases, venous outflow restriction may result in symptomatic venous hypertension. This may be particularly problematic in the setting of a large DVA when multiple radicular veins drain into a single large venous channel.