PMC9263889_01
HSV-2 inflammatory intracranial arterial vasculopathy (Patient 7); DWI (A) demonstrates an acute infarct involving the right occipital and medial temporal lobes. ToF MRA (B) shows occlusion of the P1 segment of the right PCA (dashed arrow) and narrowing right MCA with a 'beaded' appearance (arrow), as well as diffuse narrowing of the intracranial right ICA. VWI (C) wall thickening and enhancement of the right MCA (double arrow), right P1 segment (dashed arrow) and right intracranial ICA (not shown). ASL (D) shows reduced perfusion in the area of infarction (arrow) and extensive ATAs over the cortical territory of the right MCA (arrowheads), indicating a relative delay in blood arrival. Follow-up imaging after treatment (E-H) shows the right temporo-occipital infarct maturation, with no evidence of new ischaemia on DWI (E). ToF MRA (F) demonstrates partial recanalization of the right PCA (dashed arrow), improvement in the calibre of the right MCA (arrow) and reduced narrowing of the right intracranial ICA. VWI (G) shows near resolution of the wall thickening and enhancement of the right MCA (double white arrows), right P1 segment (dashed arrow) and right intracranial ICA (not shown). ASL (H) shows normalization of perfusion in the corresponding cortical territories of the right MCA and PCA.
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In the absence of definitive evidence for the management of presumed inflammatory intracranial arterial vasculopathy, treatment choices were practical, individualized after discussion and drawn from the experience of managing large vessel systemic vasculitis (e.g. giant cell and Takayasu Arteritis) with use of drugs such as methotrexate, cyclophosphamide, mycophenolate mofetil and Tocilizumab (Supplementary Fig. 1).