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Abstract Details

Burkitt Lymphoma-associated GAD65 Encephalitis in a Child with New-onset, Refractory Seizures
Autoimmune Neurology
P1 - Poster Session 1 (8:00 AM-9:00 AM)
14-007

Immune-mediated processes are increasingly described as a significant cause of encephalitis in children. Associated antibodies, disease states, and clinical syndromes including paraneoplastic etiologies are continuously being elucidated. Anti-N-methyl-D-aspartate receptor encephalitis and its association with teratoma, and opsoclonus-myoclonus syndrome (OMS) with neuroblastoma are now well-characterized, but a growing number of antibodies are being implicated in pediatric paraneoplastic syndromes. Glutamic acid decarboxylase (GAD) 65-ab–associated neurological syndromes include stiff-person syndrome, epilepsy, cerebellar ataxia, encephalomyelitis, limbic encephalitis, and OMS. Though not classically considered high risk for association with an underlying malignancy, GAD65-abs have increasingly been implicated paraneoplastic neurological syndromes related to neuroendocrine, lung, and thymic neoplasms primarily. There have been rare reports of GAD65-abs disease related to hematologic malignancy, but a specific association between Burkitt lymphoma and GAD65 encephalitis has not yet been reported.

Refractory epilepsy is not uncommon in pediatric patients; consideration of whether seizures are acutely symptomatic of a primary disease process is critical. In this case, a healthy 7-year-old child presented with intractable focal seizures in the setting of a mild viral prodrome and headache. This case highlights a comprehensive work up of new-onset, refractory epilepsy in a patient with features of encephalitis, and it describes a diagnostic quandary that ultimately led to a rare unifying diagnosis. 

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In this case of explosive-onset, refractory epilepsy, broad work up revealed evidence of CNS inflammation and autoimmunity which ultimately led to the diagnosis of GAD65-ab–associated paraneoplastic encephalitis related to an underlying Burkitt lymphoma in a previously healthy 7-year-old patient. This case emphasizes the importance of considering paraneoplastic etiologies in pediatric patients with acute or subacute, progressive neurologic symptoms with evidence of inflammation based on CSF or neuroimaging.

Authors/Disclosures
MaryGlen Stanich, MD (Primary Children's Hospital)
PRESENTER
Dr. Stanich has nothing to disclose.
Melissa A. Wright, MD (University of Utah) Dr. Wright has nothing to disclose.