Primary Central Nervous System Vasculitis as an Unusual Cause of Intracerebral Hemorrhage: A Case Report

Abstract

A 64-year-old male with a history of transverse myelitis presented to the hospital with a decreased level of consciousness of one day's duration. CT of the head revealed intracranial hemorrhage measuring 2 x 1.2 cm in the right temporal lobe and multiple small hemorrhages in the left hemisphere, suggestive of vasculitis. Initial vasculitis workup was negative for antinuclear antibody (ANA), complement component 3 (C3), and antineutrophil cytoplasmic antibodies: P-ANCA, C-ANCA. Syphilis, hepatitis B and C, West Nile virus antibody [immunoglobulin G (IgG) and immunoglobulin M (IgM)], herpes simplex virus (HSV) polymerase chain reaction (PCR), and HIV 1 and 2 were also negative. In view of the CT scan findings suggestive of vasculitis and the vague presentation of primary central nervous system vasculitis (PCNSV), a brain biopsy was performed. It revealed angiocentric granulomatous inflammation with focal vessel disruption and associated parenchymal hemorrhage, consistent with a diagnosis of granulomatous vasculitis. The patient received levetiracetam, multiple high doses of steroids, and six cycles of cyclophosphamide for a six-month duration. After induction, he has remained in remission without any maintenance therapy until now (eight years post-presentation).

Keywords: cns angiitis; induction therapy; intracerebral hemorrhage; primary cns vasculitis.

Figure 1. CT scan of the head without contrast

The image demonstrates small amounts of acute intraparenchymal hemorrhage in the right temporal and frontal lobes (yellow arrows) with a large area of associated edema below (white arrow) CT: computed tomography

Figure 2. Brain MRI (T2*-weighted gradient-echo sequences)

The images reveal focal intraparenchymal hemorrhage with surrounding edema in the right posterior, superior temporal lobe (yellow arrows in A-B). Additional areas of hemorrhage are present superiorly in the right frontal lobe (white arrow in C), and the left hemisphere below (white arrows in A) MRI: magnetic resonance imaging

Figure 3. Biopsy images - 1

(A, B) Transmural granulomatous inflammation of leptomeningeal arteries composed predominantly of lymphocytes and scattered histiocytes (brackets, H&E stain). (C) Vessel wall with membranous staining (immunohistochemistry staining). (D) Cerebral parenchyma showing a focal vessel with concentric perivascular inflammation consisting of chronic inflammatory cells (arrow, H&E stain) H&E: hematoxylin and eosin

Figure 4. Biopsy images - 2

(A, B) Cerebral parenchymal surface showing vessels with concentric perivascular chronic inflammatory cells (arrows, H&E stain). (C) Vessel wall inflammation with diffuse staining (arrow, immunohistochemistry staining). (D) Cerebral parenchyma showing a congested vessel with perivascular inflammation (black arrow) and adjacent red neurons (H&E stain) H&E: hematoxylin and eosin