OBJECTIVE: To study the clinical course, response to therapy, and longterm outcome of @entity16 ( @entity16 ) in a series of @entity1 with primary @entity244 ( @entity244 ) followed prospectively in our referral centers. METHODS: We studied 15 @entity1 (13 @entity1 , 2 @entity1 ) with primary @entity244 and @entity16 . All @entity1 fulfilled 4 or more of the European diagnostic criteria. RESULTS: At diagnosis of @entity16 , clinical manifestations included @entity1183 and @entity809 (11 @entity1 ), @entity16 (6 @entity1 ), and @entity36898 (4 @entity1 ). In 7 @entity1 , @entity16 was diagnosed prior to @entity244 , in 5 the diagnoses were made simultaneously, and in the remaining 3 @entity1 @entity16 was diagnosed after the appearance of @entity244 symptomatology. The mean duration of the prospective @entity16 followup was 10 years (range 1-20). The progression of @entity16 was acute in 1 @entity1 (producing severe @entity409 in less than 1 month), subacute in 3 @entity1 , and in the remaining 11, the symptoms progressed slowly over the ensuing years to other extremities. @entity1 were treated with @entity288 (n = 13), @entity786 (n = 4), and intravenous immunoglobulins (n = 1), and 2 @entity1 received no treatment. In spite of treatment, most @entity1 showed an indolent and insidious longterm @entity16 course. CONCLUSION: We found 3 differentiated clinical courses of the @entity16 in @entity1 with primary @entity244 : subacute progression in less than 1 month (7%), late acceleration of @entity16 2-4 years after an initial indolent onset (20%), and a very longterm insidious, chronic evolution (73%). Prospective analysis of the longterm course of @entity16 shows a chronic and insidious evolution in most @entity1 with @entity16 and @entity244 , with a poor response to treatment, although stabilization of symptomatology for long periods is often observed.

Pure XXXX in primary @entity244 . Longterm prospective followup and review of the literature.

@entity1 :: ('9606', 'Species') :: ['women', 'Patients', 'patients', 'patient', 'men'] @entity1183 :: ('MESH:D006987', 'Disease') :: ['numbness'] @entity36898 :: ('MESH:D015845', 'Disease') :: ["Adie's pupil syndrome"] @entity16 :: ('MESH:D009422', 'Disease') :: ['trigeminal neuropathy', 'pure sensory neuropathy', 'PSN'] @entity809 :: ('MESH:D010292', 'Disease') :: ['paresthesias'] @entity409 :: ('MESH:D008107', 'Disease') :: ['dysfunction'] @entity786 :: ('MESH:D003520', 'Chemical') :: ['cyclophosphamide'] @entity244 :: ('MESH:D014899', 'Disease') :: ["Sj  gren's syndrome", 'SS'] @entity288 :: ('MESH:D000305', 'Chemical') :: ['corticosteroids']
@entity16 :: (MESH:D009422,Disease) :: ['sensory neuropathy']
