Introduction: Syphilis is called “the great imitator” and it is a rare cause of stroke, mainly in immunocompromised subjects. Treponema pallidum infection has been recognised as possible cause of Moya-Moya arteriopathy, (MA) although there are only few isolated reports.
Herein we describe a patient affected by syphilis developing a uniltateral MA.
Case report: A 59-years old patient was admitted to our Stroke Unit because of the sudden onset of left-sided hemiparesis and dysarthria. Neuroimaging (CT and MRI) with angiographic sequences showed a right frontal-parietal ischemia with a tight ispilateral ICA terminus stenosis and circular contrast enhancement (CE) in the wall of the stenotic artery. VDRL in serum and CSF was positive at high title and a diagnosis of luetic intracranial arteritis was made. After prolonged antibiotic treatment VDRL in CSF turned negative, but at neuroimaging follow-up the ICA terminus stenosis increased with persistent CE and slow development of a moyamoya-like collateral circulation on the right side, well evident in digital subtraction angiography. Other typical sites of vascular luetic involvement were excluded (i.e. luetic aortitis). The negative autoimmunity tests as well as the lack of CSF oligoclonal band did not clearly support the diagnosis of primary vasculitis. Moreover, CE of ICA wall was only partially responsive to steroid treatment.
Discussion: Immunocompetent subjects are rarely infected by treponema and a persistent large intracranial vessel arteritis has never been described at our knowledge. We suggest that diagnostic pathway of patients with symptomatic intracranial stenosis should include blood and liquoral test for syphilis.