A fifty-nine year old female presented with a nine-day history of low-grade fevers, headache, myalgia, weakness, and waxing and waning confusion. Collateral history from family described intermittent psychotic symptoms including auditory and visual hallucinations, as well as short-term memory deficits. Review of systems was otherwise negative. Patient had no significant past medical or social history. On exam, patient was afebrile and had normal vital signs. She was alert and oriented to person, place, and time. She had a confluent erythematous rash on bilateral cheeks sparing the nasolabial folds. She had no focal neurologic deficits, but had anomic aphasia and perseverating behavior. Remainder of the exam was normal. Routine chemistry, CBC, Respiratory viral panel, blood cultures and urinalysis were within normal limits. There was a mild elevation of transaminases on liver tests. Lumbar puncture was done and CSF showed normal cell counts, negative cultures and viral studies, and mild elevation in protein. CT head showed chronic lacunar infarcts and MRI brain showed no signal abnormalities. Further testing revealed positive ANA titer 1:320 and Anti-DS DNA of 67U/ml. She was evaluated by rheumatology and diagnosed with Systemic lupus erythematosis.
Discussion:
Systemic Lupus Erythematosis (SLE) is a systemic disease that can manifest in almost any organ system including the brain. Neuropsychiatric symptoms are present in about five percent of patients with SLE. Some patients feature psychotic symptoms including delusions and hallucinations. Patients may also present with a fluctuating delirium, poor attention span, easy distraction, misinterpretation of surroundings, agitation, and/or combative behavior. The mechanism is thought to be secondary to presence of antineuronal antibodies.
Diagnosis requires meeting 4 out of 11 of the 1997 American College of Rheumatology (ACR) criteria or the 2012 Systemic Lupus International Collaborating Clinics (SLICC). Neuropsychiatric symptoms are one criteria present in both ACR and SLICC. A psychiatric disturbance due to lupus is a diagnosis of exclusion and other organic causes of psychosis need to be ruled out. In our patient, the negative head imaging and infectious work up ruled out most common etiologies of encephalopathy. In addition to neuropsychiatric symptoms, our patient exhibited a malar rash, had positive ANA and had elevated Anti-dsDNA antibodies.
Conclusions:
Our patient did not present with the common manifestations of lupus such as, arthritis and hematologic or renal abnormalities. However, she did meet 4 /11 ACR criteria required for diagnosing lupus. Her only symptoms were a malar rash and psychosis. The psychosis was characterized with confusion, memory loss, and auditory/visual hallucinations. When dealing with cases involving psychosis, autoimmune etiologies are important to discuss on the differential. Once the diagnosis was confirmed, our patient was started on steroids and her symptoms improved.