Lydia Figueroa, MD
PGY3 Department of Pediatrics, San Juan City Hospital
Hospital Municipal De San Juan
Disclosure information not submitted.
Mayra Perez Mendoza, MD
PGY3
Department of Pediatrics, San Juan City Hospital, United States
Disclosure information not submitted.
Yamel Liriano Cepeda, MD
PGY3
Department of Pediatrics, San Juan City Hospital, United States
Disclosure information not submitted.
Carlos Ocasio Lopez, MD
Pediatric Critical Care Associate Professor
University Pediatric Hospital, Puerto Rico, United States
Disclosure information not submitted.
Patricia Fernandez, MD
Neurology Fellow
UPR School of Medicine, United States
Disclosure information not submitted.
Noelia Flores De Jesus, MD
PGY3
Hospital la Concepcion, United States
Disclosure information not submitted.
Sanet Torres, MD
Program Director, Pediatric Residency Program San Juan City Hospital
n/a, Puerto Rico, United States
Disclosure information not submitted.
Title: Rapidly progressive Anti-NMDAR encephalitis: Challenge in patient management admitted to the PICU
Introduction: Paraneoplastic NMDAR encephalitis, associated with ovarian teratomas have a widespread presentation, predominating acute onset psychiatric symptoms, rapidly decline level of consciousness, seizures, dysautonomia in combination with neurological findings often requiring critical care support.
Description: A healthy 19- year-old female with onset of behavioral changes including hypersexuality, disorganized ideas, and speech apraxia. Evaluated and referred to a psychiatric institution for management of psychosis after normal head CT. Despite antipsychotic treatment, patient remained with catatonia and seizures-like episodes; transferred to our institution for evaluation of organic etiologies. Patient admitted to PICU with severe focal facial/brachial tonic movements, refractory to multiple anti-seizures medications (levetiracetam, lacosamide and lorazepam). She developed hypoxemic respiratory failure, requiring mechanical ventilation, midazolam drip and IV antibiotic/acyclovir prophylaxis without improvement of movements. CSF analysis non-suggestive of infectious process. EEG with rhythmic burst movements, encephalopathic state and non-specific cortical dysfunction. Normal Brain MRI. During first 48 hours, she developed AKI and bullous lesions throughout her body. Patient fulfill criteria for autoimmune encephalitis with RITE 9 / APE 11 scores, requiring immunosuppressive treatment with IVIG’s. Once infection ruled out, Solu-medrol was started. Rheumatologic work up was negative. Abdominopelvic CT revealed right adnexal mass. AntiNMDA antibodies previously ordered (VGKC, LGI-1) confirmed diagnosis. Patient taken to OR, mass pathology consistent with ovarian teratoma.
Discussion: New onset psychiatric symptoms or changes in personality and mood in a young woman without psychiatric illness, along with refractory focal seizures should always rise suspicion of anti-NMDAR encephalitis and grant further evaluation. This case is uncommon, our patient developed acute respiratory failure, AKI, and bullous lesions over the first 48hrs of admission. Prompt immunosuppressive therapy and high index of suspicion for paraneoplastic mass as etiology of her autoimmune encephalitis, helped control refractory focal movements, skin lesions and frail state in a timely manner.