Abstract Introduction Critically ill patients with altered mental status and sepsis present significant diagnostic challenges. We present a case of steroid-responsive encephalopathy in a patient with septic shock and overlapping autoimmune and infectious markers. Case Presentation A 70-year-old male from Ghana presented with syncope and one week of flu-like symptoms. On admission, he was febrile (103 °F), tachypneic, hypotensive, leukopenic with lactic acidosis. He was admitted for septic shock and started on vancomycin and cefepime. He subsequently developed headache, photophobia, and a complex partial seizure. With worsening mental status, antibiotic regimen was broadened including acyclovir and doxycycline for meningitis coverage. Initial lumbar puncture revealed CSF protein 97 mg/dL, glucose 44 mg/dL (serum 86 mg/dL), 3 WBC, and negative cultures. MRI brain showed nonspecific white matter changes; EEG demonstrated mild diffuse slowing. Pancytopenia was present with severe B12 deficiency. Serologies revealed ANA 1:320 (speckled) and SSA 8. CSF PCR panel including various bacterial/viral panel was negative; HIV, malaria, and tick-borne testing were negative. Treponemal antibody and RPR 1:4 were positive, though CSF VDRL was negative. CSF cytology was negative for malignant cells and did not reveal oligocloncal bands. The autoimmune panel (NMDA, AMPA, GABA-B, LGI1, CASPR2 antibody), as well as paraneoplastic markers (amphiphysin, ANNA-1/Hu, ANNA-2/Ri, CV2.1, PNMA MA2/Ta, PCA-1/Yo) were negative. On hospital day 9, the patient’s mental status deteriorated with hypotension requiring vasopressors. Repeat LP showed WBC 63 (55% neutrophils), protein 85, glucose 40; cultures and MTB PCR remained negative. Given persistent fevers and origin from a TB-endemic region, RIPE therapy was initiated. With continued negative infectious workup and positive SSA, patient was diagnosed with primary SS encephalitis and was treated with pulse-dose steroid followed by taper with significant clinical improvement. Discussion Sjogren syndrome is an autoimmune disease not uncommon in adults. However, its CNS manifestations such as encephalitis are rare. Neurological symptoms can precede sicca symptoms as reported in 25-60% of cases. Often, MRI brain of Sjogren encephalitis is nonspecific adding to diagnostic complexity. This case illustrates the diagnostic uncertainty of encephalopathy in the critically ill, where autoimmune, infectious, and metabolic etiologies overlapped. Improvement following steroids raised the possibility of SSA-associated autoimmune encephalitis versus CNS TB responsive to adjunctive steroids. The case highlights ICU decision-making when early treatment must precede diagnostic clarity. In septic patients with encephalopathy and negative initial studies, a broad empiric approach and early consideration of autoimmune CNS disease are warranted. This abstract is funded by: None
Park et al. (Fri,) studied this question.