Background. Anti-neurotrophil cytoplasmic antibody (ANCA) and positive small vessel vasculitides (SVV) are systemic diseases characterized by inflammation and necrosis of multiple organ small blood vessels and include Wegener's granulomatosis (WG) Churg-Strauss syndrome (CSS), and microscopic polyangiitis (MP). The development of a rapid onset dementia paralleling the systemic disease has been anecdotically reported, although other possible causes of cognitive deterioration were not ruled out so that the real involvement of the CNS small vessels in SVV is still unknown. The aim of our study was to detect the subclinical neuropsychological involvement of SVV patients due to the disease itself. Subjects. Within a large population of 65 patients in whom ANCA+ SVV was diagnosed, we selected and submitted to a detailed general neuropsychological test battery 43 subjects (36 WG, 5 CSS, 2 MP; 20 males, 23 females mean age 54.5 years, mean education 8 years). A score in the pathological range in 2 or more tests was considered a criterion for cognitive impairment. The inclusion criteria were as follow: (I) age23; (3) absence of focal neurological deficits either at clinical examination or brain imaging; and (4) absence of systemic diseases other than SVV, possibly causing dementia. A subgroup of 11 patients were submitted to MRI. Results. The results show that, taken as a group, SVV patients performed within normal controls' score, although 30% of the patients proved to have a subclinical neuropsychological impairment: 18.6% of the patients obtained pathological scores in 2 tests and 11.6% of the patients in 3 or more tests. The test scores more frequently impaired were Rey's figure recall, WCST, digit symbol association, digit span, reaction time without pre-alarm and with pre-alarm. MRI findings were normal in 4 patients (3 cognitively normal and 1 with 2 pathological tests). Only 1 patient (2 tests impaired) had a high TLV (11.8 ml) and 6 patients showed multiple hyperintense white matter lesions, mainly periventricular or subcortical (1 with 3, 3 with 2 and 1 without patological tests scores). Conclusions. We conclude that, even in clinically nondemented patients, SVV can cause a neuropsychological impairment. Although no clear correlation with a specific MRI pattern of lesions was found, a general tests to show white matter lesions was noticed in patients with some pathological findings at testing.
|Issue number||4 SUPPL.|
|Publication status||Published - 2000|
ASJC Scopus subject areas
- Clinical Neurology