[Primary Sjogren syndrome with involvement of the nervous system. Description of 4 cases].
Four patients, 2 males and 2 females, with primary Sjögren's syndrome (PSS) and neurologic involvement are described. The first patient presented a progressive axonal polyneuropathy due to endo-epineural vasculitis. The second patient developed an acute and focal lymphocytic meningoencephalitis with increased IgG synthesis and excellent response to corticosteroids. The remaining 2 patients had previously been diagnosed as having definite multiple sclerosis (MS), but after a standardized study both fulfilled PSS diagnostic criteria. These cases illustrate the polymorphism of the neurological manifestations in PSS. Pathogenetic relationships between PSS and MS are briefly reviewed.
Servicio de Reumatología, Hospital Universitario Marqués de Valdecilla, Facultad de Medicina, Santander.
J L
JL
Peña-Sagredo
J
J
Miró
F
F
Aguayo
C
C
Alvarez
J
J
Berciano
D000293:Adolescent; D000328:Adult; D001921:Brain / Q000000981:diagnostic imaging / Q000276:immunology / Q000503:physiopathology*; D003937:Diagnosis, Differential; D005071:Evoked Potentials; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000276:immunology; D007074:Immunoglobulin G / Q000276:immunology; D007075:Immunoglobulin M / Q000276:immunology; D008279:Magnetic Resonance Imaging; D008297:Male; D008590:Meningoencephalitis / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D008875:Middle Aged; D009103:Multiple Sclerosis / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology; D011859:Radiography; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000276:immunology / Q000503:physiopathology
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M