Bilateral optic neuropathy revealing Sjögren's syndrome.
BACKGROUND
The central nervous system involvement has been reported in 20% of cases of primary Sjogrën's syndrome (SS), a chronic autoimmune disease characterized by a disorder of the exocrine glands secondary to progressive lymphocyte infiltration. Classically described neurological manifestations include sensorimotor deficits, aseptic meningitis or meningoencephalitis, multiple sclerosis-like syndromes and myeolopathies.


METHODS
We report here the case of a 53-year-old woman who exhibited rapidly progressive visual loss, disclosing bilateral optic neuropathy, as an uncommon initial symptom of primary SS. Examination of CSF revealed associated aseptic meningitis. Because of the lack of efficacy of the first treatment by intravenous corticosteroids, monthly intravenous cyclophosphamide was quickly introduced. After six months, significant visual recovery was observed.


CONCLUSIONS
Optic neuropathies have been rarely reported as the initial symptom revealing primary Sjogrën syndrome, and bilateral simultaneous lesions remain exceptional.
Béjot
Y
Y
Service de neurologie, CHU de Dijon, 3, rue du Faubourg-Raines, 21000 Dijon, France. ybejot@yahoo.fr
Osseby
G-V
GV
Ben Salem
D
D
Beynat
J
J
Muller
G
G
Moreau
T
T
Giroud
M
M
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000032:analysis; D001921:Brain / Q000473:pathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008582:Meningitis, Aseptic / Q000134:cerebrospinal fluid; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D009901:Optic Nerve Diseases / Q000134:cerebrospinal fluid / Q000209:etiology / Q000473:pathology*; D012859:Sjogren's Syndrome / Q000134:cerebrospinal fluid / Q000150:complications / Q000473:pathology*
D000893:Anti-Inflammatory Agents; D000974:Antibodies, Antinuclear; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone