Central Nervous System Vasculitis

Central nervous system vasculitis (CNSV), alternatively termed primary angiitis of the central nervous system (PACNS) or granulomatous angiitis of the central nervous system, is a rare diagnosis with an estimated incidence rate of 2.4 cases per 1 million person years ( ). The diagnosis can be challenging given the nonspecific presenting signs of headache, encephalopathy, and variable focal neurological signs. Here we outline some of the typical features and methods of distinguishing CNSV from mimics to create a framework for improving the diagnosis of this often discussed but rare disease.

Features and Diagnosis

Clinical Features

Clinical symptoms are often nonspecific, can include headache (50%–60%), progressive cognitive difficulties (50%–70%), confusion, personality changes, and vision loss, and vary depending on the central nervous system (CNS) region affected. Ischemic strokes, intracranial hemorrhage, and subarachnoid hemorrhage can be seen but are less common than the aforementioned symptoms. Although fever, weight loss, rash, night sweats, arthritis, and peripheral neuropathy have been reported in a minority of patients with CNSV, systemic features should prompt a workup to rule out secondary vasculitis due to systemic cause (Limaye, 2018). The Calabrese diagnostic criteria include the presence of unexplained neurological deficits with evidence of either classic angiographic or histopathological features of angiitis within the CNS and no evidence of a systemic vasculitis or any other condition that could elicit the angiographic or pathological features ( ).

Pathology

Features on histology have been subcategorized into granulomatous lymphocytic and necrotizing vasculitis ( ; Limaye, 2018). Amyloid beta–related angiitis (ABRA) shares clinical and physiological characteristics but represents a separate disease entity ( ).

Brain biopsy remains the “gold standard” of diagnosis for CNSV and is recommended to confirm diagnosis given the ongoing commitment for immunosuppressive therapy and potential side effects. Biopsy may also be helpful in making an alternate diagnosis ( ).

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