Abstract

Wolves in the brain - A rare case of neuropsychiatric lupus

21 year old female with no significant past history presented with altered mental status as noted by her family members. Two days prior to presentation, she started becoming increasingly restless, tearful, loud and irritable. Social or functional status has been reported as well. Of note, she was evaluated for a skin rash involving her mouth, palms and soles two month prior to presentation and was diagnosed with possible hand-foot-mouth disease. On admission, patient vital signs were notable for. A Petechial rash was noted in the mouth, palms and soles bilaterally. Mental status examination was notable for severe agitation, labile mood, tangential thought process and delusions. Initial laboratory studies were significant for white blood count of 2100/mm3, red cell count of 3.24/mm3 and platelets of 77000/mm3. EKG confirmed sinus tachycardia. Urine drug screen was negative. Lumbar puncture showed normal CSF cell count was normal with normal protein. Patient was started on quetiapine and Haldol. Further laboratory testing revealed erythrocyte sedimentation rate of 24 mm/h, positive ANA with titer of 320, Anti dsDNA of >300, low C3 and C4 complement level of 38 mg/dl and 3 mg/dl respectively. MRI and MRA were normal and EEG revealed mild diffuse background slowing indicating mild diffuse cerebral dysfunction. Rheumatology was consulted due to concern for lupus cerebritis based on high ANA titers, evidence of vasculitis on skin examination, abnormal EEG and exclusion of other more common etiologies. Patient was started on 1 gram methylprednisolone after which she showed considerable improvement in mentation with normalization of her thought content and process. Our case also illustrates the importance of maintaining a high degree of clinical suspicion of NPSLE even with a paucity of evidence of clinical systemic activity.

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