Objective: To better delineate the spectrum of neurological complications o
f primary Sjogren's syndrome (PSS). Methods: A detailed neurological invest
igation was prospectively performed in a group of 25 consecutive patients w
ith PSS followed in an internal medicine department between June 1996 and D
ecember 1997 (Internal Medicine group). In addition, eleven patients with n
eurological complications of PSS were identified in the Neurological Depart
ment of the same institution during the same period (Neurological group). R
esults: In the Internal Medicine group, neurological complications were dis
covered in 10/25 (40%) patients. Peripheral nervous system involvement was
present in 4/25 patients from the Internal Medicine group and in 10/11 pati
ents from the Neurological group and consisted mainly of axonal sensorimoto
r/sensory polyneuropathy. A motor neuron syndrome was identified in two pat
ients. CNS involvement occurred in 7/25 patients from the Internal Medicine
group and in 4/11 patients from the Neurological group. Three patients had
spinal cord involvement. Cognitive dysfunction was the most frequent findi
ng (5/25 in the Internal Medicine group, 3/11 in the Neurological group) ch
aracterized either by subcortical or corticosubcortical dysfunction. Cognit
ive impairment was not attributed to mood disturbance and was not associate
d with specific laboratory or radiological abnormalities. Conclusion: Neuro
logical complications of PSS are frequent since they were present in 40% (1
0/25) of patients in a consecutive series of patients from a department of
Internal Medicine. Although PNS involvement predominates, complications of
PSS affecting the brain or spinal cord are not rare, with subcortical dysfu
nction as the main finding.