(P100) First Episode Psychosis in a Young Female: Concomitant Nmda-receptor Encephalitis and Multiple Sclerosis (Radiologically Isolated Syndrome)

Chief Complaint : Hallucinations, Psychosis

History of Present Illness : 27 year-old G4P4004 female (9 months post-partum) without significant PMH presented to the emergency department after 2 weeks of new onset psychosis. Evaluated by outside hospital in the setting of command auditory hallucinations instructing her to hurt herself, husband, and children. She presented without focal neurologic deficits, with normal vital signs, and in appropriate behavioral control. Screening labs and urine toxicology were unremarkable. No personal or family history of psychosis, seizures, or behavioral health symptoms. Husband noted self-isolation and inattention to children. Patient was medically cleared, evaluated by social work, and voluntarily admitted to inpatient psychiatry for first-episode schizophrenia. She remained voluntarily admitted for 4 days prior to leaving AMA due to minimal effect of Aripiprazole. She re-presented 1 week later to our ED with worsening auditory and new onset visual hallucinations with worsening inattentiveness. Husband endorsed a single short period of non-responsiveness and rightward gaze deviation that resolved spontaneously.

Pertinent Physical Exam: Her neurologic exam on presentation was without focal neurologic deficits beyond bilateral horizontal nystagmus and altered mental status. There was no evidence of central hypoventilation. Normal strength in bilateral upper and lower extremities. Sensation intact in bilateral upper and lower extremities. Finger to nose and heel to shin intact. No cranial nerve deficits. Intermittently decreased attentiveness. No ataxia. Vital signs within normal limits and stable. No tremor, no erratic movements. Normal tone, no rigidity.

Pertinent Laboratory Data: Complete blood count, comprehensive metabolic panel, urinalysis, serum pregnancy testing, and blood alcohol level were unremarkable. Urine toxicology screen was notable for cannabinoids alone. CSF negative for HSV1/2, JC virus, EB virus, Quantiferon TB, VDRL, and mycobacteria. CSF/blood cultures unremarkable. CSF cytometry/pathology negative. CSF cell studies demonstrated slight lymphocytic pleocytosis with 10 nucleated cells, 0% PMNs, 95% lymphocytes, glucose of 52, and protein of 44. Her serum studies were pan-negative for HCV, HBV, RPR, HIV, toxoplasmosis, oligoclonal bands, anti-aquaporin 4 antibodies, and anti-MOG antibodies. CSF positive for NMDAR antibody. Serum negative for NMDAR antibody.

Case Discussion: Neuroimaging was pursued with CT head w/o contrast and MRI with and without contrast of brain . CT head notable for a left frontoparietal lobe lesion (1.1 x 1.3 cm). MRI brain confirmed a rim-enhancing lesion of undetermined etiology in the left temporoparietal lobe as well as T2/FLAIR hyperintense signal abnormalities throughout the supratentorial and infratentorial white matter, highly suggestive of central demyelinating disease, specifically multiple sclerosis. She was admitted to the neurology service where she continued to deteriorate from a psychiatric standpoint, requiring physical restraints and antipsychotic medications. She was initiated on Lacosamide for seizure prophylaxis. An EEG was performed and unremarkable for epileptiform activity. She underwent MRI full spine which did not reveal any evidence of lesions in her spinal cord. Differential remained broad with autoimmune encephalitis, limbic encephalitis, viral/bacterial encephalitis, toxoplasmosis, Creutzfeldt-Jakob disease, progressive multifocal leukoencephalopathy, neurosyphilis, primary psychiatric disorder, among others. Due to concern for possible HSV encephalitis, she was not initiated on immunosuppressants on admission. Lumbar puncture was performed and CSF testing was unremarkable and with cell count without evidence of meningitis. Given CSF HSV negative, she was started on IV methylprednisolone 1g daily x 5 days. Given high concern for NMDAR encephalitis, CT imaging of her chest, abdomen, and pelvis was performed as well as a transvaginal ultrasound without evidence of ovarian teratoma or primary malignancy. On Day 8, she was weaned off antipsychotic medications. Further CSF testing was noted for elevated oligoclonal bands and a positive NMDAR antibody screen, though her serum studies were negative for NMDAR antibodies. Given high likelihood of NMDAR encephalitis with concomitant multiple sclerosis, she was started on a 5 day course of IVIG at 400mg/kg with marked improvement in her mental status. She was ultimately discharged on Lacosamide for seizure prophylaxis with plan for outpatient Rituximab infusions.

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