[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.
Peña-Sagredo
J L
JL
Servicio de Reumatología, Hospital Universitario Marqués de Valdecilla, Facultad de Medicina, Santander.
Miró
J
J
Aguayo
F
F
Alvarez
C
C
Berciano
J
J
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