Session Time: 11:15am-12:45pm
Location: Research Hub - Kiosk 3
Disclosures: David Liang, MD: Nothing to disclose
Case Description: A 9-year-old female was diagnosed with anti-N-methyl-D-aspartate (NMDA) receptor encephalitis at 6 years of age after presenting with seizures which progressed to status epilepticus. Prior to this event, she had been healthy with normal development. She was placed in a medically induced coma for 7 days due to persistent seizures. Immunomodulatory therapies were administered, however decline continued with development of dystonia and choreoathetosis. Imaging of the brain showed diffuse volume loss. The patient underwent prophylactic bilateral oophorectomy in October 2016 due to concerns for occult malignancy. However, pathology returned negative for teratoma. Since August 2015, the patient had remained in a vegetative state and was admitted to a long-term care facility in early 2017.
Setting: Pediatric long-term care facility.
Patient: 9-year-old female with anti-NMDA receptor encephalitis.
Assessment/Results: The patient received rehabilitation services as a resident of the long-term care facility. In March 2018, staff began to note that she could purposefully clasp her hands together and demonstrate occasional echolalic speech of single words. Further functional improvements rapidly followed. She began to follow limited commands and respond verbally to greetings. Motor control improved in the trunk and upper extremities. Tone, which was initially increased in the lower extremities, began to normalize. By fall of 2018, the patient remarkably could formulate full sentences, demonstrate near-baseline memory, self-propel her wheelchair, and feed herself orally. Her cognitive skills are currently at the 6-year-old developmental level.
Discussion: To our knowledge, this is the only case of a pediatric patient with anti-NMDA encephalitis who demonstrated such rapid functional improvement after spending roughly 30 months in a vegetative state.
Conclusion: Physicians and parents of pediatric patients with anti-NMDA receptor encephalitis should understand that significant functional improvements can still occur even years after initial diagnosis. It is therefore crucial that these patients are provided with high quality rehabilitation oversight and care.
Level of Evidence: Level V
To cite this abstract in AMA style:Liang D, Moran A, Shelton JE. Remarkable Functional Recovery of a 9-year-old Female Nearly 3 Years After Initial Diagnosis of Anti-N-Methyl-D-Aspartate Receptor Encephalitis: A Case Report [abstract]. PM R. 2019; 11(S2)(suppl 2). https://pmrjabstracts.org/abstract/remarkable-functional-recovery-of-a-9-year-old-female-nearly-3-years-after-initial-diagnosis-of-anti-n-methyl-d-aspartate-receptor-encephalitis-a-case-report/. Accessed February 27, 2024.
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PM&R Meeting Abstracts - https://pmrjabstracts.org/abstract/remarkable-functional-recovery-of-a-9-year-old-female-nearly-3-years-after-initial-diagnosis-of-anti-n-methyl-d-aspartate-receptor-encephalitis-a-case-report/