Case Presentation: A 58-year-old right-handed, nonsmoker, otherwise healthy male presented with episodes of vertigo and slurred speech. Vital signs were within normal range and his initial labs were noncontributory. Imaging revealed multifocal acute and subacute infarcts in the postero-occipital areas. Extensive workup did not reveal any arrhythmias, valvular abnormalities, patent foramen ovale, aortic or carotid plaques. Furthermore, hypercoagulable workup was also unremarkable. CTA head and neck revealed a beading pattern consistent with PACNS while CSF tested positive for VZV Antibodies. He was treated with pulse steroids and IV Acyclovir for possible post infectious vasculitis. Despite therapy, he continued to suffer new infarcts and it was felt that he is in the subset of rapidly progressing PACNS. He underwent a brain biopsy, which confirmed the diagnosis. He was then placed on Rituximab in addition to a steroid taper. Therapy led to the partial neurological recovery and prevented any further episode of strokes.
Discussion: Vasculitis is defined as inflammation of the blood vessels with or without necrosis of the vessel wall. Leptomeningeal and/or parenchymal vessel wall inflammation of the brain is seen in cerebral vasculitis. PACNS is the most frequent vasculitis involving the brain and spinal cord. It is a rare cause of stroke that poses a diagnostic and therapeutic dilemma. An annual incidence of 2.4 cases per million patient per year. The most common underlying pathophysiology is non-infectious granulomatous angiitis affecting the nervous system. Diagnosis is based on clinical exam findings, disease progression, the age of onset and multiple technical examinations including laboratory tests and multiple imaging modalities. Ultimately, a biopsy should be encouraged to confirm the diagnosis of PACNS as there are many mimickers of the condition, which can present with similar angiographic findings. A combination of steroids and pulse cyclophosphamide is recommended. Rituximab has also been studied as a less toxic alternative to cyclophosphamide.
Conclusions: The diagnosis of PACNS is often delayed due to the wide spectrum of neurological signs and symptoms including headaches, cranial nerve involvement, encephalopathy, seizures, psychosis, myelitis, intracranial hemorrhage, and aseptic meningoencephalitis. It is important to consider this diagnosis in cryptogenic multifocal strokes as prompt treatment can significantly reduce the morbidity of this disease.
To cite this abstract:Upadhrasta, S; Nauman, F; Abuali, I. A CASE OF PRIMARY ANGIITIS OF THE CNS. Abstract published at Hospital Medicine 2019, March 24-27, National Harbor, Md. Abstract 1048. https://www.shmabstracts.com/abstract/a-case-of-primary-angiitis-of-the-cns/. Accessed July 15, 2019.