Syphilitic Vasculitis: a mysterious and dangerous cause of stroke in the young
By: Dr Inna Lutsenko, Department of Online Education, Kyrgyz State Medical Academy, Bishkek, Kyrgyzstan, Bishkek Hospital 1, Vascular Neurology Department.
Introduction
Among the rare stroke etiologies, vasculitis caused by infection is important as it is potentially modifiable. Multiple bacteria, viruses, fungi, and protozoa have been reported as causes of central nervous system vasculitis, particularly in patients who are immunocompromised [1-3]. In contrast to a decrease of bacterial infection, HIV and syphilis have become more common infectious causes in stroke. Here we are sharing with you a case of syphilitic vasculitis in a young patient.
Case
A 37-year-old patient was admitted to the neurological department with speech impairment, acute weakness in the right limbs, and generalized epileptic seizures. These symptoms were preceded by a two to three week history of headache. The patient is a smoker and periodically abuses alcohol but otherwise has no hypertension or diabetes. Brain MRI revealed a large acute infarct of the left middle cerebral artery territory. Patient received antiplatelet and symptomatic antiepileptic treatment (carbamazepine 400 mg per day). Unfortunately, he continued to have seizure attacks in the month following discharge. What should we do?
Yes, we should first determine the aetiology of his stroke.
He did have abnormal bloods with elevated erythrocyte sedimentation rate, platelet count, and elevated fibrinogen A and B. He also had a heart murmur on examination and subsequent echo showed thickening of the aortic wall. Therefore infectious vasculitis was suspected.
The patient went on to have screening for syphilis but his Rapid Plasma Reagin test (RPR) was negative. However, we found highly positive fluorescent treponemal antibody absorption (FTAABS), Treponema pallidum hemagglutinin (TPHA) and Venereal Disease Research Laboratory (VDRL). Cerebrospinal fluid (CSF) findings also showed low glucose level, elevated protein and positive TPHA and VDRL.
So this is a case of syphilitic vasculitis causing ischemic stroke in a young patient! The patient was then treated with i.v. Benzylpenicillin for 21 days and he recovered well.
Syphilis of the heart and blood vessels accounts for 80% of all syphilitic visceropathies [1,2]. Pathological findings of neurosyphilis include acute syphilitic meningitis and meningovascular neurosyphilis which can lead to stroke and parenchymatous neurosyphilis [1]. Two types of arteritis have been described: endarteritis obliterans of the medium and large arteries (ie, Heubner arteritis) and Nissl–Alzheimer arteritis affecting small arterial vessels [4]. A specific compaction of the ascending aorta can also develop very early on in syphilis and can be asymptomatic for a long time. Moreover, thrombotic masses in the left atrium, ventricle, and valvular apparatus of the heart, as well as those that form in the walls of the main extracranial arteries, are an embologous substrate, upon separation from which the embolus is transferred to the arterial system of the brain [4].
Conclusion
The useful take home message is that the diagnostic confirmation of neurosyphilis is often associated with a positive FTA test plus a positive CSF VDRL test accompanied by CSF pleocytosis and elevated CSF protein levels [3]. It is worth remembering that in many patients RPR test can be negative and one should not stop there if syphilis is high on the differential list.
So to conclude, although rare, given that the diagnosis has potential impact on effective treatment, clinicians should not forget about syphilis as a cause for stroke, particularly in young and male patients with elevated infectious markers [4].
References:
- Simon RP (1985) Neurosyphilis. Arch Neurol 42:606–613
- Coyle P Dattwyler R. // Spirochetal infection of the central nervous system. Infect. Dis. Clon. North. Am. — 1999- V4, N4, p.731-746.
- Gaa J, Weidauer S, Sitzer M, Lanfermann H, Zanella FE. Cerebral vasculitis due to Treponema pallidum infection: MRI and MRA finding. Eur Radiol (2004) 14:746–747 DOI 10.1007/s00330-003-2015-4
- Bauerle J, Zitzmann A, Karl E, Mecke S et al. The Great Imitator—Still Today! A Case of Meningovascular Syphilis Affecting the Posterior Circulation. Journal of Stroke and Cerebrovascular Diseases, Vol. 24, No. 1 (January), 2015: pp e1-e3
