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A case of anti‐N‐methyl‐D‐aspartate receptor encephalitis with systemic sclerosis

Sir, Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is characterized by the presence of antibodies against NR1–NR2 heteromers of the NMDAR (anti-NMDAR antibodies) with ovarian teratoma [1]. However, recently, it has become clear that anti-NMDAR encephalitis is not always paraneoplastic [2]. We present a female patient affected with anti-NMDAR encephalitis and systemic sclerosis (SSc), the disease activity of which synchronized with each other. A 70-year-old female patient had Raynaud s phenomenon, weight loss, easy fatigability, muscle weakness, and skin sclerosis. Six months later, she presented with cognitive impairment and gait disturbance. Physical examination showed skin sclerosis and ulcers on the hands, face, and feet. Neurological examination revealed dementia (15/30 in the MiniMental State Examination), resting tremor, ataxia, rigidity, and muscle weakness in the extremities. Pathological reflexes were bilaterally positive. Brain magnetic resonance imaging (MRI) showed high intensities in bilateral basal ganglia and thalamus on fluid-attenuated inversion recovery (FLAIR) images (Fig. 1). Brain magnetic resonance angiography showed normal. Initial cerebrospinal fluid (CSF) examination showed normal cell count, elevated total protein (112 mg/dl) and IL-6 (5.8 pg/ml). Oligoclonal IgG Bands were negative. Chest computed tomography (CT) showed mild interstitial pneumonitis (Fig. 1). Serological analysis showed increased serum antinuclear antibody titer (1:80 with diffuse speckled pattern). Elevation of tumor markers (CEA, NSE, Pro-GRP, and AFP) or paraneoplastic antineuronal antibodies were not revealed. Anti-NMDAR anti-