Page 60 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

36 David S. Younger

the ICA and MCA with thickened vessel walls and narrowed lumina. The fifth case was
Patient 4 of Leher [151], a 39-year-old man who presented with confusion and stupor, was
found to have indentation of the lateral aspect of the ICA as it entered the subarachnoid space
above the anterior clinoid on cerebral angiography. Ventriculography showed a block at the
aqueduct of Sylvius. He died shortly thereafter and postmortem examination showed widely
distributed systemic tuberculosis with binding of vessels at the base of the brain that showed
arteritis, accompanied by infarction of the basal ganglia and brainstem.

Kopsachilis and colleagues [152] described a 39-year-old man without known
tuberculosis, who developed sudden visual loss in one eye. Fluorescein angiography showed
an infero-temporal branch retinal vein occlusion consisting of blockage with areas of
hemorrhage, exudation, and late leakage. This was followed by optic disc swelling and
headache. Biopsy of an enlarged cervical and submandibular lymph node revealed caseating
epithelioid ant cells confirming tuberculosis. Treatment with antituberculous treatment leads
to improved visual acuity.

Syphilis

Meningovascular syphilis comprises 39 to 61% of all symptomatic cases of neurosyphilis
and tends to occur more frequently in patients with concurrently infected HIV/AIDS. It is
characterized by obliterative endarteritis that affects blood vessels of the brain, spinal cord
and leptomeninges precipitating substantial ischemic injury.

Often referred to as Heubner arteritis, it involves medium-sized to large arteries with
lymphoplasmacytic intimal inflammation and fibrosis however there is a variant form termed
Nissl-Alzheimer arteritis that characteristically affects small vessels and produces both
adventitial and intimal thickening. Both types can lead to vascular thrombotic occlusions and
cerebral infarction, with preferential involvement of the MCA.

The search for the cause of stroke in young adults should include meningovascular
syphilis as a potential etiology. Sudden acute severe headache heralded onset of occlusion of
bilateral vertebral and proximal basilar artery documented by MRA [153] in a 35-year-old
African man. He responded to thrombectomy with restoration of blood flow but succumbed to
fatal pontine and subarachnoid hemorrhages. Postmortem examination revealed reactive
plasma reagin (RPR) and a positive Venereal Disease Research Laboratory (VDRL) test in
CSF with CNS vasculitis characterized by mural thrombi along the vertebrobasilar arteries
with well-defined lines of Zahn of alternating layers of fibrin, platelet and red blood cell
aggregates, and inflammatory cell infiltration of the arterial walls particularly in the
adventitia. Headache of two to three weeks in duration were the presenting features of two
other patients with stroke syndromes [27] one of whom had narrowing of bilateral M1
segments of the MCA, reactive CSF VDRL positive Treponema pallidum hemagglutin-assay
and fluorescent treponemal antibody-absorption (FTA-Abs) staining in the CSF, similar to
second patient who instead presented with a stroke in the territory of the posterior cerebral
artery (PCA) without focal changes on cerebral angiography, neither of whom were studied
pathologically for true vasculitis.

One patient with abrupt onset of confusion, aphasia, and hemiparesis had carotid
angiography that documented normal named cerebral vessels except for smaller than average
caliber, with an abnormal complement fixation test of the blood and CSF, positive colloidal

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