Our prospective study consisted of a general and neurological evaluati
on in 48 patients (41 females, 7 males; mean age: 58.2 years) with pri
mary Sjogren's syndrome (PSS). We performed serologic studies and cran
ial magnetic resonance imaging (MRI). Main extraglandular features wer
e arthralgias and non-erosive arthritis (37.5%), Raynaud (21%) and pul
monary fibrosis (12.5%). Antinuclear antibodies were positive in 42.5
% and anti-SS-h (Re) in 20%. Migraine (52%), neuropsychiatric disease
(29%) and a past history of focal acute neurological deficits (23%), w
ere the central nervous system (CNS) manifestations more frequently ob
served. Cranial MRI examination detected hyperintense small subcortica
l lesions in 51.3% of patients and in 36.6% of age and sex matched con
trols (P < 0.001). CNS disease was not serious concerning vital progno
sis, but produced significant morbidity in some patients. Late onset a
migraine-like,, episodes with prolonged sensoromotor deficits and coe
xisting neuropsychiatric disease emerged as a characteristic clinical
spectrum in those patients diagnosed in a neurological setting. Crania
l MRI was frequently abnormal, but findings were not specific. Neurolo
gic manifestations reminiscent of multiple sclerosis were rarely seen.