[A case of meningitis due to anti-centromere antibody-positive Sjögren syndrome].

Abstract

A 79-year-old woman was diagnosed with Sjögren's syndrome (SjS) at the age of 73 years by lip biopsy, gum test, and salivary gland scintigraphy with positive antinuclear (ANA), positive anti-centromere (ACA), and negative anti-Ro/SS-A antibodies. Seven days before admission, the patient developed gait disturbance, which progressed to difficulty in walking two days before admission. She was hospitalized because of subacute gait disturbance. Neurological examination showed gait disturbances characterized by small steps, wide-based gait, left upper limb clumsiness, and frontal lobe dysfunction. Laboratory tests confirmed positive ANA and ACA and negative anti-Ro/SS-A and anti-La/SS-B antibodies. Cerebrospinal fluid analysis showed mildly elevated protein levels and increased monocyte count. Brain MRI showed hyperintensity in the bilateral frontal regions on fluid-attenuated inversion recovery (FLAIR)/contrast-enhanced T₁-weighted imaging, and N-isopropyl-p-¹²³I-iodoamphetamine single-photon emission computed tomography (¹²³I-IMP SPECT) showed decreased accumulation in the frontal region. After excluding cancerous, infectious, and immune-mediated causes, ACA-single-positive SjS-related meningitis was diagnosed. The patient responded well to steroid therapy. The patient was able to walk short distances and showed improvement in frontal lobe function. Follow-up MRI and ¹²³I-IMP SPECT demonstrated the resolution of previous abnormalities. Knowingly, there have been no reported cases of SjS-related meningitis with positive ACA. Considering that anti-Ro/SS-A antibodies are involved in the pathogenesis of meningitis, this case is extremely rare. We propose that ACA-positive SjS be considered in the differential diagnosis of aseptic meningitis.