Central nervous system involvement is rarely observed in primary Sjogren's syndrome (PSS) and aseptic meningoencephalitis occurs exceptionally.
We report the case of a 25-year-old woman who presented with weakness of the four limbs, vision loss and concentration deficit. Neurological examination revealed a mental confusion, cerebellar involvement and spastic tetra paresis. Cerebrospinal fluid analysis revealed elevation of cell counts (162/mm3) and protein content (3 g/l). MRI showed increased T2 signals in the white matter. No evidence for an infectious etiology, especially tuberculosis, was obtained in this patient. PSS was diagnosed on the basis of ocular dryness, lachrymal hyposecretion, aspects of labial gland biopsy witch revealed destruction of the ducts with periductal lymphocytic infiltration and positive anti-SSB antibodies. Oral corticosteroids (1 mg/kg/day) allowed an improvement of the neurological manifestations.
Aseptic meningoencephalitis can be the first manifestation of primary Sjogren's syndrome. Infectious causes, particularly tuberculosis which is frequent in our context must be ruled out.