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Abstract Details

Paraneoplastic Myeloneuropathies: characterization of a distinguishable phenotype and clinical outcomes
Autoimmune Neurology
P5 - Poster Session 5 (8:00 AM-9:00 AM)
15-003
To describe a distinguishable clinical phenotype of paraneoplastic myeloneuropathies.

There is limited data to suggest that myeloneuropathies exist within the spectrum of classical paraneoplastic syndromes.

We analyzed patients with clinical/radiographic/electrodiagnostic evidence of co-occurring myelopathy and peripheral neuropathy seropositive for onconeural autoantibodies, and/or a diagnosis of cancer within 5 years of symptom onset. Comparison to historical cohort of copper-deficiency myeloneuropathies was performed.

Among thirty-two patients with paraneoplastic myeloneuropathy, twenty-six had detectable onconeural antibodies (Amphiphysin, 8; ANNA-1/anti-Hu, 6; CRMP-5, 5; PCA-1/anti-Yo, 2; PCA-2, 1; Kelch-like-Protein-11, 1; combinations thereof: ANNA-1 and CRMP-5, 1; ANNA-1 and Amphiphysin, 1; ANNA-3 and CRMP-5,1). Among these, twenty-two had underlying malignancy (small-cell lung cancer, 10), and four had lung nodules/masses (not evaluated further). Six patients had malignancies without classified onconeural antibodies (unclassified neural-autoantibodies, 3). Weight loss (>10 lbs, 63%), bowel/bladder dysfunction (69%) and gastrointestinal dysmotility (46%) were common presenting symptoms. The majority of neuropathies were non-length dependent (88%), asymmetric (69%), and presented with dysesthesia at onset (63%). A considerable proportion had polyradicular localization (56%). Co-existing upper and lower motor neuron involvement was documented on initial examination in 81%. However, 56% initially received an alternative diagnosis. Inflammatory CSF was noted in 82% (median nucleated-cell count, 13 cell/mm3 [range 0-74]). Tract-specific changes on cervical/thoracic MRI were seen in 9/29(31%) patients.  In comparison to copper-deficiency myeloneuropathy (n=11), asymmetric sensory presentations, subacute progression, anorexia/weight loss, dysautonomia, taste/smell alterations, inflammatory CSF and gadolinium enhancement of spinal cord or lumbosacral roots were significantly more frequent in paraneoplastic myeloneuropathies (p<0.05). Ten of thirty-one (32%) paraneoplastic patients with long-term follow-up data were wheelchair bound at last follow-up. Median modified-Rankin-Score at last follow-up was 3 (range 1-6).

A paraneoplastic etiology should be considered in the differential of subacute, progressive myeloneuropathy. Onconeural antibody evaluation and malignancy screening among these cases may aid in diagnosis of occult malignancy. 
Authors/Disclosures
Shailee S. Shah, MD
PRESENTER
Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon Therapeutics. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Shah has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Shah has received publishing royalties from a publication relating to health care.
Rocio Vazquez Do Campo, MD (University of Alabama at Birmingham) Dr. Vazquez Do Campo has received personal compensation in the range of $5,000-$9,999 for serving as a Advisor with US Department of Health, vaccine compensation program.
Sean J. Pittock, MD, FÂé¶¹´«Ã½Ó³»­ (Mayo Clinic Dept of Neurology) Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Pittock has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for UCB. The institution of Dr. Pittock has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche/Genentech. The institution of Dr. Pittock has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Alexion/AstraZeneka. The institution of Dr. Pittock has received research support from NIH. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received intellectual property interests from a discovery or technology relating to health care. Dr. Pittock has received publishing royalties from a publication relating to health care.
Andrew McKeon, MD (Mayo Clinic) The institution of Dr. McKeon has received research support from National Institutes of Health. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received intellectual property interests from a discovery or technology relating to health care. Dr. McKeon has received publishing royalties from a publication relating to health care.
Neeraj Kumar, MD (Mayo Clinic, Dept of Neurology) Dr. Kumar has nothing to disclose.
Eoin P. Flanagan, MBBCh, FÂé¶¹´«Ã½Ó³»­ (Mayo Clinic) The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Merck. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to Âé¶¹´«Ã½Ó³»­ interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to Âé¶¹´«Ã½Ó³»­ interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to Âé¶¹´«Ã½Ó³»­ interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to Âé¶¹´«Ã½Ó³»­ interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology that is relevant to Âé¶¹´«Ã½Ó³»­ interests or activities.
Christopher J. Klein, MD, FÂé¶¹´«Ã½Ó³»­ (Mayo Clinic) Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NMD Pharma.
Divyanshu Dubey, MD, FÂé¶¹´«Ã½Ó³»­ (Mayo Clinic) The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys. The institution of Dr. Dubey has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UCB . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from Department of Defense . Dr. Dubey has received research support from UCB. Dr. Dubey has received research support from David J. Tomassoni ALS Research Grant Program . Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care. Dr. Dubey has received intellectual property interests from a discovery or technology relating to health care.