Page 54 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

30 David S. Younger

material, especially of the CNS, leads to recognizable clinical sequela due to vascular and
parenchymal damage.

The ACR delineated criteria for the diagnosis of SLE [114]. The recognition of
neuropsychiatric lupus (NPSLE) was noted in 6.4% of a cohort of 1,253 SLE patients defined
by the ACR [115]. According to Tomic-Lucic and colleagues [116] those with late-onset SLE
due to development of disease after age 50 year, had a frequency of NPSLE of 6.6%
compared to 36.6% in early-onset disease despite less major organ involvement and more
benign course. Once thought to be an important cause of CNS or cerebral lupus, true
vasculitis was present in only 12% of postmortem examinations in the series of Johnson and
colleagues [117], and in 26.7% of late-onset SLE patients compared to 16.6% of those with
early-onset SLE. A comparison of the cumulative incidence of clinical manifestations in the
two latter groups showed that seizures were more common in early-onset patients compared
to later-onset patients (6.6% versus 0%), similarly for multiple cerebrovascular attacks
(23.3% versus 3.3%), cranial and peripheral neuropathy (6.6% versus 3.3%). Nonetheless,
there was no mention of CNS vasculitis among 150 patients with SLE described by Estes and
Christian [118], despite the finding of neurological complications in 30% of patients that
included focal deficits, seizures, dementia, stupor, and coma. Devinsky and colleagues [119]
noted a prevalence of 3.5% of psychiatric involvement that included organic affective,
delusional and hallucinatory syndromes in a cohort of 50 patients with SLE, one-half of
whom had CNS lesions. Feinglass and colleagues [120] noted neuropsychiatric
manifestations at onset of SLE among 3% of 140 patients compared to 37% in the course of
the illness; however headache was not specifically tabulated. Cerebral dysfunction in SLE can
be caused by large vessel or small vessel involvement or both. In the series by Fienglass and
colleagues [120] vasculitis was noted overall in 28% of patients, as well as in 46% of those
with neuropsychiatric involvement compared to 17% of patient lacking neuropsychiatric
involvement. Postmortem examination of the CNS in ten of nineteen fatalities showed two
cases of multiple large and small infarcts, which in one of them, demonstrated inflammatory
cells infiltrates in the walls of medium-sized vessels, and perivascular infiltrates around small
arterioles. Although active CNS vasculitis was absent in the brain and spinal tissue of all fifty
patients reported by Devinsky and colleagues [119], two had evidence of inactive healed CNS
vasculitis so suggested by focal disruption of the elastic lamina and mild intimal proliferation
of a single medium-sized artery, one of which had active systemic vasculitis of the PAN type,
both of whom evidenced Libman-Sacks endocarditis and embolic brain infarcts. Focal angiitis
of the CNS with cyst-like formation around affected blood vessels was noted at postmortem
in the patient described by Mintz and Fraga [121] with typical SLE rash, cutaneous vasculitis,
and active neuropsychiatric involvement. Trevor and colleagues [122] summarized the
literature of large named cerebral vessel occlusions from 1958 to 1965 noting one patient with
a middle cerebral artery (MCA) stenosis progressing to occlusion and three others with
angiographic internal carotid artery (ICA) occlusions, adding three new patients and
suggesting a relation of the occurrence to cerebral arteritis. Two women, one age 21 and the
other age 42 years, presented with headache followed by focal neurological symptoms
attributed respectively to lesions along the left MCA followed by right ICA occlusions, and a
right MCA stenosis progressing to occlusion in four months. A third patient had a left ICA
occlusion without mention of headache. Among the four literature patients, one had
angiographic occlusion of the MCA, and three others had occlusion of the ICA.

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