Page 383 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

Ophthalmologic and Neuro-Ophthalmologic Aspects of Vasculitides 357

The diagnosis of PACNS [185], like IACNS [183] relies upon the presence of the classic
angiographic features of beading in cerebral angiographic studies or the histopathologic
features of angiitis in brain and meningeal vessels in the absence of systemic vasculitis or
another cause for the observed findings. Affected patients with PACNS present with
headache of gradual onset accompanied by either focal neurological signs or diffuse cognitive
changes, so noted respectively in those with involvement of either large named arteries or
small meningeal and distal terminal cortical vessels. The clinical course can be rapidly
progressive over days to weeks, or insidiously over weeks to months, with seemingly
prolonged periods of stabilization.

Younger and colleagues [188] described symptoms and signs in four patients with GAB
alone or in association with concomitant neurosarcoidosis, varicella zoster virus (VZV)
infection, and Hodgkin lymphoma, and reviewed the clinical and laboratory findings in 74
additionally-proven cases. Mental change, headache, fever, focal weakness, and visual
changes, were the commonest symptoms and signs at onset respectively in 58%, 54%, 21%,
15%, 9% at presentation, and in 78%, 54%, 21%, 42%, and 12% during the course of the
illness. Visual symptoms included diplopia, amaurosis fugax, blurring of vision, and
contiguous involvement of the eye in those with V1 dermatomal VZV lesions. Hemiparesis,
quadriparesis, and lethargy were associated with a poor prognosis and mandated the need for
combined meningeal and brain biopsy to establish the diagnosis followed by combination
chemotherapy employing corticosteroids and cyclophosphamide.

Cupps and colleagues [183] noted neuro-ophthalmological involvement in two of four
patients with IACNS. Patient 1 had transient hemifield visual loss accompanied by headaches
before the angiographic diagnosis of IACNS with involvement of named cerebral vessels,
followed several months later after commencement of combination immunosuppressant
therapy by a one-week period of altered VA. Patient 3 had a unilateral fundus Roth spot, with
markedly decreased VA and normal pupillary response before diagnostic cerebral
angiography of IACNS showed narrowing of named cerebral vessels, followed months later
after commencement of combination immunosuppressant therapy by occipital headaches,
transient decreased visual acuity and a starburst image in the central visual field.

Calabrese and Mallek [185] reported eye signs in 15% of literature cases with
angiographically or pathologically defined PACNS but in none of 8 Cleveland Clinic patients.
Among 70 patients with angiographically-defined and 31 patients with pathologically-verified
PCNSV described by Salvarani and colleagues [186], hemiparesis, unilateral numbness,
blurred vision and decreased acuity were most common in those with angiographically-
defined PCNSV, although persistent neurological deficit or stroke and headache were the
commonest initial findings overall in 68% of patients. A granulomatous pattern of
inflammation was seen most often in those with altered cognition and at an older age.
Lanthier and coworkers [184], who described histologically-proven IACNS in two children,
had one child (Case 2) with bilateral optic disk swelling and persistent conjugated
gaze-evoked nystagmus at presentation. Among four children with angiographically-negative
childhood PACNS described by Benseler and colleagues [187] one child (Patient 3) was
noted to have gaze-evoked nystagmus. (Figure 2)

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