Abstract Details

Title A Case of Thymoma Associated Paraneoplastic Encephalitis with Identification of a Novel Neuronal Antibody

Topic Autoimmune Neurology

Presentation(s) P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 066

Objective To report a case of a healthy adult male presenting with classic autoimmune encephalitis leading to discovery of thymoma, with identification of novel neuronal antibody in cerebrospinal fluid (CSF).

Background A 44-year-old male presented with subacute onset of vertigo, headaches, subtle personality changes, short-term memory deficits, and a weight loss of 11 pounds. He had 2 unprovoked seizures that were preceded by an aura of left tongue and arm paresthesias. Initial physical exam was normal, other than fixed delusions of a "string" from his left hand and tongue. MRI and continuous EEG were normal. His laboratory evaluation was notable for hyponatremia and CSF with elevated protein and pleocytosis.

Design/Methods NA

Results A CT chest/abdomen/pelvis revealed a 5.3 cm pericardial mass abutting the mainstem pulmonary artery, which biopsy identified as a thymoma. He became increasingly psychotic and disoriented, with persistent symptoms involving the left face and hand. Despite negative EEG, these were treated as focal seizures and improved. He also treated with IVIg and high-dose steroids. His other symptoms (insomnia, psychosis, and hyponatremia) were resistant to treatment. Due to the complexity of thymoma location, neoadjuvant chemotherapy was recommended. He received cisplatin, doxorubicin, and cyclophosphamide coinciding with a second course of IVIg and high-dose steroids, after which his neurologic status slowly normalized over days. Eventually, his serum ACh-binding Ab was positive, as was striated muscle Ab IgG, with titer 1:1280. Autoimmune panels of serum and CSF sent to Mayo Clinic identified cross-reactivity with Neurochondrin, indicating a novel neuronal antibody pending further investigation. He has since had resection of thymoma.

Conclusions Patients with subacute onset of neurological decline need evaluation for autoimmune encephalitis and paraneoplastic syndromes. With ongoing rapid expansion of antibody detection capabilities, it is important to describe phenotype-specific presentations of paraneoplastic encephalitis. This case demonstrates the importance of early detection of malignancy such as thymoma.

Authors/Disclosures
Alexa L. Carreras, MD PRESENTER	Dr. Carreras has nothing to disclose.
Michael V. Robers, MD (Barrow Neurological Institute)	Dr. Robers has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Robers has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG therapeutics. Dr. Robers has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi. Dr. Robers has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for TG Therapeutics. The institution of Dr. Robers has received research support from Bristol Myers Squibb Foundation.
Martha E. Orozco Cortes, MD	Dr. Orozco Cortes has nothing to disclose.
Megan N. Thomas, MD (Barrow Neurological Institute)	Dr. Thomas has nothing to disclose.
Justin Hoskin, MD (Barrow Neurological Institute)	Dr. Hoskin has nothing to disclose.
Shane Root, MD (Barrow Neurological Institute)	Dr. Root has nothing to disclose.

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