Norberto Escobales, MD
Resident
Department of Physical Medicine & Rehabilitation Temple University Hospital
Philadelphia, Pennsylvania, United States
Eric Xu, DO
Resident
Temple University
Philadelphia, Pennsylvania, United States
Ernesto S. Cruz, MD
Professor
Lewis Katz School of Medicine at Temple University
Ambler, Pennsylvania, United States
A patient with granulomatosis with polyangiitis with a prior associated stroke presenting for a multifocal acute cerebral infarction with concomitant VZV vasculitis.
Case Description:
42-year-old female with past medical history of GPA with positive c-ANCA and PR3 complicated by previous multifocal strokes and tracheal stenosis who presented to the hospital for aphasia, dysarthria, and left facial droop and hemiplegia. MRI brain showed moderate-sized acute cortical infarction involving the left temporal lobe with evidence of acute microhemorrhage. Additionally there were acute infarcts involving the right caudate head, centrum semiovale, basal ganglia, occipital cortex, left frontal cortex and bilateral cerebellum. The patient was started on intravenous steroids and cyclophosphamide. Later she developed altered mental status, worsening verbal output and right-sided gaze preference. A CT Brain showed new right ACA and MCA infarcts that were also confirmed on MRI. Subsequently, they were found to have VZV vasculitis and acute ischemic strokes, thought to have contributed to the right frontal-parietal stroke.
Discussions:
Antineutrophilic cytoplasmic antibody (ANCA) associated vasculitides are a rare group of autoimmune conditions that is implicated in the inflammation of small caliber vessels. Classically, affecting pulmonary and renal systems, but neurologic manifestations have been documented. Within this ischemic infarction have been seen, with variable lesion involvement of mostly white matter. Strokes attributed to autoimmune vasculitis are resistant to antiplatelet therapy, recur without immunosuppression, associated hemorrhagic transformation and impose significant morbidity.
Varicella-zoster virus has been implicated in vasculopathies of the central nervous systems. VZV implicated cerebral infarction, is typically multifocal in nature with grade–white matter junction involvement.
Conclusions:
ANCA associated vasculitides have been shown to impact cerebral vasculature leading to cerebral infarction. In treating ANCA associated strokes it is important for clinicians to be mindful of the risks associated with immunosuppression, as these patients are at high risk not only for recurrent stroke but also opportunistic infections.
