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Abstract Details

Paraneoplastic Posterior Reversible Encephalopathy Syndrome (PRES) as a Harbinger of Lung Cancer
Neurotoxicology
P06 - (-)
210
BACKGROUND: Accepted theory hypothesizes that PRES is caused by failure of the blood-brain barrier which occurs from relative hypertension producing increased capillary hydrostatic pressure, and from vascular endothelial dysfunction.
DESIGN/METHODS: Case Report.
RESULTS: A 72 year-old right-handed female was evaluated for subacute onset encephalopahy and seizures. She had word-finding difficulty and right-sided hemianopia on examination. MRI brain showed bi-occipital symmetric, non-enhancing, T2-weighted and FLAIR hyperintensities without restricted diffusion. Cerebral angiography was normal. Cerebrospinal fluid revealed mildly elevated protein and CSF-IgG index but was otherwise unremarkable. Evaluation for infectious, metabolic and inherited causes of leukoencephalopathy was negative. Electroencephalography revealed bi-occipital focal status epilepticus which was successfully treated with multiple anti-epileptic drugs. Chest CT showed a small right-upper lobe nodule, but serum paraneoplastic panel was negative. Serial chest CT's showed progressive increase in the nodule size. Core-biopsy of the nodule 17-months after initial discovery revealed squamous cell carcinoma. PET was negative for metastasis. She underwent stereotactic body radiation therapy and the last follow-up 2-years after initial presentation showed excellent radiographic response with no residual cancer. She also returned to near baseline mental status. A repeat MRI brain showed complete resolution of the occipital T2-weighted hyperintensities.
CONCLUSIONS: Our patient's clinical presentation, imaging findings and complete improvement of both once the lung cancer was treated is consistent with the diagnosis of PRES. None of the usual triggering factors were identified and this supports our theory that PRES was likely paraneoplastic in etiology. The mechanisms involved in paraneoplastic PRES are likely immune-mediated and lie in the same spectrum of disorders that include paraneoplastic leukoencephalopathies. In the largest retrospective series on PRES, malignancy was present in 32% of the patients. Clinicians should consider a paraneoplastic etiology in cases of PRES of undetermined etiology.
Authors/Disclosures
Seby John, MD (Cleveland Clinic Abu Dhabi)
PRESENTER
Dr. John has nothing to disclose.
Jinny O. Tavee, MD (National Jewish Health) Dr. Tavee has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for CSL Behring. Dr. Tavee has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Johnson and Johnson. Dr. Tavee has received personal compensation in the range of $0-$499 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Dynamed. The institution of Dr. Tavee has received research support from Woolsey. The institution of Dr. Tavee has received research support from Milken Foundation. The institution of Dr. Tavee has received research support from CSL Behring. Dr. Tavee has received personal compensation in the range of $0-$499 for serving as a Article author with Medlink.
Lara Jehi, MD (Cleveland Clinic Epilepsy Center) Dr. Jehi has nothing to disclose.
Heinz Wiendl, MD, FAAN (University of Freiburg) Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bristol Myers Squibb. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Roche. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sanofi. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Immunovant. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Johnson&Johnson / Janssen. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. Dr. Wiendl has received personal compensation in the range of $0-$499 for serving as a Consultant for Idorsia. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Argenx. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Immunic. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Merck. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Lundbeck. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for LTS. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Samsung. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sangamo. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Dianthus. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for EMD Serono. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for INmune Bio_Syneos Health. Dr. Wiendl has received personal compensation in the range of $0-$499 for serving as a Consultant for Muna Therapeutics. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Myrobalan Therapeutics. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for PSL Group. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Red Nucleus. Dr. Wiendl has received personal compensation in the range of $0-$499 for serving as a Consultant for Swiss Multiple Sclerosis Society. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Teladoc Health. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Toleranzia . Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Viatris. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Merck Serono. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Janssen. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Wiendl has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Argenx. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Bristol Myers Squibb. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sandoz. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sandoz-Hexal. Dr. Wiendl has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cellerys. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Uniqure. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biocryst. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Galapagos. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Idorsia. Dr. Wiendl has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biontech. The institution of Dr. Wiendl has received research support from Biogen. The institution of Dr. Wiendl has received research support from Roche. The institution of Dr. Wiendl has received research support from Deutsche Forschungsgemeinschaft. The institution of Dr. Wiendl has received research support from European Union. The institution of Dr. Wiendl has received research support from Argenx. The institution of Dr. Wiendl has received research support from CSL Behring. The institution of Dr. Wiendl has received research support from Merck. The institution of Dr. Wiendl has received research support from UCB. Dr. Wiendl has received research support from Alexion Syneos Health iVH UK Ltd.. The institution of Dr. Wiendl has received research support from Deutsche Myasthenie-Gesellschaft. The institution of Dr. Wiendl has received research support from Winicker Norimed GmbH. The institution of Dr. Wiendl has received research support from Novartis. Dr. Wiendl has received publishing royalties from a publication relating to health care. Dr. Wiendl has received publishing royalties from a publication relating to health care. Dr. Wiendl has received publishing royalties from a publication relating to health care.