Page 61 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Overview of Primary and Secondary Vasculitides 37

gold curve test, and leptomeningeal biopsy that showed lymphocytic infiltration, focal
fibrosis, and chronic perivasculitis consistent with meningovascular syphilis [154].

Neither headache nor confirmatory pathological evidence of CNS vasculitis was
mentioned in a patient with basilar artery stenosis and serologically confirmed syphilis in the
CSF presumed to be due to meningovascular syphilis [155] so suggested by a positive string
sign of the mid-basilar artery at cerebral angiography.

Lyme Neuroborreliosis

The term Lyme neuroborreliosis was introduced by Veenendaal-Hilbers and colleagues
[156] in 1988 to emphasize that CNS involvement due to B. burgdorferi infection, the
causative agent of Lyme disease. Among 20 patients described in the literature with
neurovascular clinical syndromes ascribed to CNS vasculitis in which detailed information
was available including documentation of positive CSF Lyme serology, two patients [157,
158] who presented with headache were ultimately noted to have histopathologically
confirmed vasculitis on brain biopsy. Patient 3 of Oksi and colleagues [157] was an 11-year-
old boy with headache and hyperactivity syndrome who developed gait difficulty
concomitantly with a stroke visualized on brain MRI. Subsequent craniotomy and biopsy of
the area of enhancement disclosed lymphocytic vasculitis of small vessels without fibrinoid
necrosis, and CSF B. burgdorferi serology was positive. Headache and the MRI improved
with intravenous antimicrobial therapy. Patient 2 of Topakian and coworkers [158] presented
with headache, fatigue, malaise, nausea and vomiting first considered migrainous then
psychosomatic until subsequent MRI disclosed ischemic brain infarctions, MRA was
compatible with diffuse vasculitis, and CSF showed lymphocytic pleocytosis with positive
oligoclonal bands, and diagnostic CSF and serum B. burgdorferi serology. Brain biopsy
showed vasculitis involving leptomeningeal arteries comprised of lymphoplasmacytic vessel
wall infiltration with focal necrosis. Epithelioid cells were beaded in multiple granuloma-like
formations in the leptomeninges. There was symptomatic improved after a course of
intravenous antimicrobial therapy. A third patient reported by Miklossy and colleagues [159],
a 50-year-old man with leg spasticity and CSF pleocytosis for 15 months who progressed to
hemiparesis and ventilatory support, was later found to have diagnostic B. burgdorferi
serology in serum and CSF. Postmortem examination showed perivascular lymphocytic
inflammation of leptomeningeal vessels, some of which displayed infiltration of the vessel
walls, duplication of the elastic lamina, narrowing of lumina, and complete obstruction of
some leptomeningeal vessels by organized thrombi. Seventeen other patients with presumed
CNS vasculitis due to B. burgdorferi infection of the CNS were reported, so suggested by
complaints of headache [27, 28, 156, 162-164], while unmentioned in the case report of eight
others [165-168], none of whom had histologically-proven CNS vasculitis.

Varicella Zoster Virus Infection

VZV is the cause of childhood chickenpox and most children manifest only mild
neurologic sequela. However, after it resolves, the virus becomes latent in neurons of cranial
and spinal ganglia of nearly all individuals, and has the propensity to reactivate in elderly

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