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Washington University Experience | NEURODEGENERATION | Anti-NMDA Receptor (Paraneoplastic) Encephalitis | 2A0 Case 2 History
Case 2 History ---- The patient is a 32 year old woman who was previously well with a history of mild intermittent anxiety, and past incidental discovery of an ovarian teratoma coincident at the time of c-section (4 years ago) but apparently not removed at that time. She presented with a history of anxiety in early April 2016, with additional visuospatial/perceptual complaints that the "room was spinning". Peripheral leukocytosis was noted, and she was discharged with a prescription for antibiotics. Vertigo continued, with worsening vision (visual hallucinations were suspected). Subsequent evaluation, including MRI, did not reveal any explanation for her symptoms. Over the ensuing days, anxiety dramatically worsened, and was accompanied by additional mental status changes, culminating in psychiatric inpatient admission. She did not respond to typical symptomatic management. Further evaluations, including LP, confirmed central leukocytosis (25 WBC) with normal protein (27.2), glucose (56) and bands (2). TPN was initiated due to poor oral intake. A consultation was sought from Barnes Neurology, and pelvic imaging recommended which revealed interval development of an “dermoid” (aka ovarian teratoma) (May 9 2016). CSF testing for NMDAR established the diagnosis of NMDAR encephalitis. Laparoscopic right salpingo-oophorectomy and left salpingectomy were completed in mid-May 2016, with pathology confirming a mature cystic teratoma with neural elements and well-organized, closely approximated lymphoid follicles. She was treated with IVIG with interval improvement in her neurological status. Unfortunately, her inpatient course was complicated by development of gram negative sepsis (? related to TPN) and severe respiratory compromise, requiring extensive ICU admission and aggressive management (with intubation). Associated acute renal failure was thoroughly evaluated, including with a renal biopsy, which revealed granulomatous tubulointerstitial nephritis with acute tubular injury (May 20, 2016). PLEX was provided to treat psychoses which became increasingly apparent as the patient improved. Methylprednisolone was provided prior to discharge, with oral steroids continued for 2 weeks during her rehabilitation admission. A single treatment of rituximab was provided, prior to discharge, but stopped due to an allergic reaction. She was discharged to inpatient rehabilitation before being discharged home in June 2016. She has continued to have ongoing difficulties with recall of recently acquired information. She does not have any direct memories of the two month period during which she was admitted to the hospital. She is capable of all activities of daily living but her husband estimates his wife's function to be 60% of what it was before. She is cognitively able to do most things, but she feels "like an 80 year-old doing things." She complains of easy fatigue. She feels rested on awakening. She is progressing well with rehab and continues to benefit from this. She estimates her function today as 75 to 80%, with residual deficits relating to fatigue. Subsequently she made a remarkable recovery, with near-complete return to her prior high level of cognitive functioning with no residual cognitive deficits (CDR 0). Mild physical limitations persist, as may be expected following her severe medical illness.
