Introduction: Cerebral angiography (DSA) is commonly used to characterize small vessel vasculopathy, yet it is difficult to establish etiology by angiographic patterns alone.
Hypothesis: Responsiveness to intra-arterial verapamil (IA-V) can help differentiate etiology of small vessel vasculopathy by characterizing it as reversible or non-reversible.
Methods: We performed a retrospective analysis from 2013-15 of patients referred for DSA to evaluate cerebral vasculopathy. We characterized symptoms, radiographic findings, angiographic pattern and responsiveness to IA-V, if given, as well as biopsy and follow-up angiography.
Results: Thirty-three patients were identified (70% female; mean age 47.7). Presenting symptoms were headache (48%), focal neurologic deficit (45%), cognitive impairment (30%) and seizure (18%). Three patients presented in the post-partum period. Radiographic findings included infarct (42%), cortical subarachnoid hemorrhage (33%) and cerebral edema (3%). Arteriopathy was present on DSA in 19 of 33 patients (58%). Twelve patients had smooth segmental narrowing, 6 had irregular narrowing, 1 had diffuse narrowing. IA-V was administered in 12 of the 19 patients with arteriopathy (63%), including 9 of 12 with smooth segmental, 2 of 6 with irregular, and the patient with diffuse narrowing. Of 9 patients with smooth segment narrowing that received IA-V, 8 exhibited a vasodilatory response (89%). In 1 of these patients, superficial temporal artery biopsy was normal. Follow-up DSA was performed in 4 of the 8 patients with verapamil-responsive arteriopathy at a median time of 86 days. All 4 had angiographic resolution of their arteriopathy, consistent with reversible cerebral vasoconstriction syndrome. The 3 patients with irregular or diffuse narrowing who received IA-V did not exhibit vasodilatory response or have follow-up angiography. In 3 patients with irregular narrowing, brain biopsy was performed; 1 revealed CNS vasculitis, 2 were normal, consistent with atherosclerosis.
Conclusion: Our series is consistent with reports of patients with reversible vasculopathy exhibiting a response to IA vasodilators. Response to IA-V may better characterize small vessel vasculopathy than angiographic pattern alone.