Intracranial Atherosclerosis Versus Primary Angiitis of the Central Nervous System: a Case Report

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare disease with various clinical presentations. It is the preferred name for vasculitis that is confined to the central nervous system (CNS) and is often considered a diagnosis of exclusion in vascular or inflammatory CNS diseases. This case describes a 46-year-old right-handed female with a past medical history of hypertension (HTN), hyperlipidemia (HLD), diabetes mellitus type two (DM2), obesity, and hemorrhagic stroke who was transferred from an outside facility after a one-month hospitalization to be evaluated for CNS vasculitis. Emergency medical personnel who brought the patient to the receiving hospital endorsed dysarthria and fluctuating level of cognition. Before she was transferred, the patient underwent a series of computed tomography (CT) and magnetic resonance imaging and angiographies (MRI/MRA) as well as four-vessel angiography. The studies revealed multiple bilateral striatal and cortical infarcts, scattered narrowing and occlusion of major cerebral vasculature, as well as other signs initially more suggestive of intracranial atherosclerosis than CNS vasculitis. Before she was transferred, imaging demonstrated a new cortical stroke. Hypercoagulable studies were positive for protein C deficiency although ensuing echocardiograms with normal ejection fractions were negative for a source of cardioembolism. Having undergone extensive rheumatological, radiological, and neurosurgical evaluation in the receiving facility, recommendations were made for the patient to undergo leptomeningeal biopsy to confirm the diagnosis of vasculopathy and to specifically rule out intracranial atherosclerosis and reversible cerebral vasoconstriction syndrome (RCVS).

Keywords: cerebral angiography; cns vasculitis; intracranial atherosclerosis; magnetic resonance angiography; primary angiitis of the central nervous system; reversible cerebral vasoconstriction disorder; vasculitis.

Figure 1. T2-weighted MR (A), FLAIR (B) and susceptibility weighted images (C) showing bilateral supratentorial white matter lesions, along with micro- and macro-hemorrhages.

Ref. [5] This figure was used from Folia Neuropathologica with consent.

Figure 2. Venous phase of left internal carotid artery digital subtraction angiogram shows irregularities and indistinct pattern of deep veins suggestive of vasculitis.

Ref. [5] This figure was used from Folia Neuropathologica with consent.

Figure 3. Photomicrographs showing (A) transmural granulomatous inflammation involving small and medium-sized leptomeningeal arteries (H&E, 200×). High magnification view (B) shows lymphocytes, epithelioid histiocytes, and giant cells (H&E, 400×). GFAP stain (C) highlights reactive astrocytosis (IHC, 400×). Similar granulomatous vasculitis seen in a small cortical artery (D; H&E, 200×) in case 6, along with transmural fibrinoid necrosis (E) in an adjacent cortical arteriole (H&E, 400×). Surrounding cortex (F) shows ischemic neurons (H&E, 400×).

Ref. [5] This figure was used from Folia Neuropathologica with consent.

Figure 4. Photomicrographs showing (A) lymphocytic infiltrates within the wall of a leptomeningeal artery; there is no significant inflammatory infiltrate in the adjacent cortex (H&E, 100×). High power view (B) showing complete destruction of the vessel wall (H&E, 200×). VVG stain (C) demonstrates loss of internal elastic lamina (VVG, 400×). Cortical blood vessels (D) showed similar features (H&E, 200×). The inflammatory cells are predominantly CD3+ T cells (E) with only few CD20+ B cells (F) (IHC, 400×).

Ref. [5] This figure was used from Folia Neuropathologica with consent.