IMPAIRED QUANTITATIVE CEREBRAL BLOOD-FLOW IN SCLERODERMA PATIENTS

Systemic Sclerosis (SSc) is a multisystem disease characterised by pro liferation of vascular tissue, obliterative microvascular lesions and diffuse organ fibrosis. Despite widespread vascular disease, Central N ervous System complaints are only infrequently reported and it is unce rtain whether they merely derive from systemic complications or whethe r they may be also caused by a primary vascular process within the bra in. Regional cerebral blood flow (rCBF) was quantitatively measured by the (133)Xenon clearance technique in twenty-seven consecutive SSc pa tients without relevant systemic complications and with different seve rity of vascular involvement, as staged by nailfold capillary videomic roscopy (NCV). Absolute, percent, and asymmetry rCBF values were compa red (z-statistics) with age-and sex-matched healthy controls. Cerebral MRT and Mini-Mental State Examination (MMSE) were also performed. Dop pler sonography of neck vessels and Transcranial Doppler sonography (T CD) were performed in patients presenting rCBF reduction. Cerebral hyp operfusion was found in the 52% of patients, i.e.: in 33% of patients with the 'early' NCV pattern, in 56% of patients with the 'active' pat tern, and in 67% of patients with the 'late' NCV pattern. Thirty perce nt were the MRIs showing focal and/or diffuse signal abnormalities in the white matter of both hemispheres with the highest rate (44%) in th e 'late' NCV pattern. MMSE disclosed mild dementia in one patient in t he 'late' NCV group and some mistakes in 6 more patients, in the 'acti ve' or 'late' NCV groups, whereas TCD failed to find significant steno sis of Willis' arteries. Cerebral hypoperfusion is shown for the first time in a substantial part of SSc patients without either neurologica l symptoms or relevant systemic complications. It is suggested that th e rCBF reduction might be related to the systemic scleroderma microang iopathy although, probably due to the paucity of connective tissue in cerebral vessels, the vast majority of patients remains in a subclinic al phase. (C) 1997 Elsevier Science B.V.