J Cerebrovasc Endovasc Neurosurg.  2024 Sep;26(3):324-330. 10.7461/jcen.2024.E2023.04.010.

Primary Angiitis of Central Nervous System related intracranial aneurysm with spontaneous occlusion after immunomodulatory treatment

• John S
• Khan M
• Kesav P
• Raj D
• Hussain SI

• KMID: 2559444
• DOI: http://doi.org/10.7461/jcen.2024.E2023.04.010

Abstract

Primary Angiitis of the Central Nervous System (PACNS) is an uncommon disease with kaleidoscopic clinical manifestations. Ischemic strokes are commoner than their hemorrhagic counterpart. Intracranial pseudoaneurysms are rarely reported in PACNS cohorts. We hereby describe the case of a 39-year-old female, who presented for evaluation of acute onset of left middle cerebral artery (MCA) ischemic stroke, with cerebral angiogram showing multifocal stenosis and irregularities in intracranial blood vessels with an aneurysm arising from the lenticulostriate branch of the left MCA M1 segment. A diagnosis of probable PACNS was made and patient initiated on immunomodulatory treatment with corticosteroids. 12 weeks follow up neuroimaging studies revealed resolution of the previously described intracranial aneurysm, thereby postulating the possibility of a pseudoaneurysm related to the underlying angiitis.

Keyword

Primary Angiitis of CNS; Intracranial aneurysm; Pseudoaneurysm; Spontaneous occlusion

Figure

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  Fig. 1. (A, B, C) Magnetic resonance imaging (MRI) Brain diffusion-weighted imaging (DWI) sequences reveals hyperintense signal involving the left basal ganglia, internal capsule and periventricular region suggestive of acute infarcts in the left middle cerebral artery (MCA) territory (black arrows). Digital Substraction Angiogram (DSA) shows aneurysm involving the left lenticulostriate aneurysm on coronal sequences of left internal carotid artery injection (D; white arrow), with lateral sequences (E) revealing the same aneurysm (white arrow) and underlying left M1 MCA stenosis. (F) Anteroposterior view of vertebral artery injection shows left P1 posterior cerebral artery (PCA) stenosis (black arrow).

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  Fig. 2. (A) Baseline contrast enhanced magnetic resonance angiogram (MRA) of the cerebral vessels reveals left lenticulostriate aneurysm (white arrow), with underlying left middle cerebral artery (MCA) M1 and P1 posterior cerebral artery (PCA) stenosis. (B) Baseline coronal T1 post contrast sequences of high-resolution MR vessel wall imaging (VWI) study demonstrate concentric wall thickening and circumferential enhancement of the left M1 MCA (B; white arrow) and left PCA P1 segment (C; white arrow) suggestive of vasculitis. (D) Follow up MRA of cerebral vessels reveals disappearance of the aneurysm with underlying stable stenosis of left MCA M1 and PCA P1 segment. Follow up T1 post contrast coronal images of MR VWI depicts resolution of the enhancement involving the left MCA M1 (E; white arrow) and left PCA P1 segment (F; white arrow).

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