Sjogren's syndrome (SS) is an important but poorly recognized cause of peri
pheral neuropathy. Several forms of peripheral nerve dysfunction occur, inc
luding trigeminal sensory neuropathy, mononeuropathy multiplex, distal sens
orimotor polyneuropathy and pure sensory neuronopathy. The pathological fin
dings vary and the definite treatment is not known. Here we present 4 cases
of acute ataxic sensory polyneuropathy with SS, and the experience of trea
tment with plasmapheresis (PP). The 4 patients were all females; ages range
d from 30 to 58 years. All had prominent loss of kinesthetic and propriocep
tive sensation. The course ranged from acute to subacute onset. Patients we
re treated with 5-9 sessions of PP. Two patients with initiation of treatme
nt within 2 weeks of onset showed dramatic and sustained responses after PP
, while the other 2 had no detectable effects. Our experience showed that P
P should be considered in patients who present with sensory neuropathy asso
ciated with SS, and the treatment should be given as early as possible. Cop
yright (C) 2001 S. Karger AG, Basel.