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Abstract Details

Enhancement in Paraneoplastic Cerebellar Degeneration Portends Leptomeningeal Metastases
Autoimmune Neurology
P1 - Poster Session 1 (8:00 AM-9:00 AM)
14-020
Our objective was to analyze imaging findings in patients with paraneoplastic cerebellar degeneration (PCD). 
PCD is an autoimmune process that destroys cerebellar Purkinje cells, causing nystagmus, ataxia, and dysarthria. The most common antibodies associated with PCD are PCA1, PCA2, anti-Ma2, anti-Ri, and anti-Tr, but some cases are antibody negative. Most cases of PCD are associated with an underlying malignancy, with breast, ovarian, and small cell lung cancers being most common. The consensus on imaging findings in PCD patients remains unclear, however.  Initial MRI’s in PCD patients are often unremarkable or have non-specific FLAIR changes without enhancement. Over time, cerebellar atrophy is evident on imaging and hypometabolism can be seen on FDG-PET. Some review papers and case reports have cited that MRI in PCD can have leptomeningeal enhancement. Cytology from CSF is somewhat insensitive in definitively diagnosing leptomeningeal metastases. 
We analyzed a retrospective database of 26 patients with PCD. We analyzed their MRI's throughout the course of their disease, their antibody status, and their overall prognosis. 
Of these 26 patients, only 3 had cerebellar enhancement during their disease course and all 3 of these patients had leptomeningeal disease and subsequently died. Two of these patients had positive PCA1, with the other patient having antibody negative disease. 
Thus, we report that leptomeningeal enhancement in PCD patients should prompt investigations for leptomeningeal metastases.  
Authors/Disclosures
Trusha Shah (University of Washington, Neurology)
PRESENTER 		Dr. Shah has nothing to disclose.
Alaina Prince Ms. Prince has nothing to disclose.
Jerome J. Graber, MD, MPH, FAAN (University of Washington) Dr. Graber has received personal compensation for serving as an employee of Binaytara Foundation. Dr. Graber has received personal compensation in the range of $500-$4,999 for serving as a Consultant for 好色先生. Dr. Graber has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Dickie McCamey Attorneys at Law. Dr. Graber has a non-compensated relationship as a Editorial Board member with Neuro-Oncology: Practice, published by Oxford that is relevant to AAN interests or activities. Dr. Graber has a non-compensated relationship as a Editorial Board Member with Journal of Pain and Symptom Management that is relevant to AAN interests or activities. Dr. Graber has a non-compensated relationship as a Board of Directors with American Society of Neuroimaging that is relevant to AAN interests or activities. Dr. Graber has a non-compensated relationship as a Board of Directors and Certification Exam Committee Member with United Council of Neurological Subspecialties that is relevant to AAN interests or activities. Dr. Graber has a non-compensated relationship as a Question of the Day 'app' committee and NeuroSAE and Continuum with 好色先生 that is relevant to AAN interests or activities. Dr. Graber has a non-compensated relationship as a Editorial Board Member with Practical Neurology (BMC) that is relevant to AAN interests or activities.