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Abstract Details

Association Between Paraneoplastic Rhombencephalitis and Hypertrophic Olivary Degeneration
Autoimmune Neurology
Autoimmune Neurology Posters (7:00 AM-5:00 PM)
107
Our goal is to report a series of six patients with onconeuronal antibody positive paraneoplastic rhombencephalitis who all developed MRI findings of hypertrophic olivary degeneration during their disease course.

Hypertrophic olivary degeneration is a rare condition caused by interruption of the dentato-rubro-olivary pathway. It is often caused by masses or lesions within this pathway. However, it has been described in the absence of any detectable lesion. We have anecdotally observed cases of hypertrophic olivary degeneration in patients with paraneoplastic rhombencephalitis.
We retrospectively searched our electronic medical record's database for patients with serum or CSF positivity for onconeuronal antibodies known to be associated with paraneoplastic rhombencephalitis. We reviewed the medical records of all patients with these antibodies and ultimately located 30 patients with a clinical diagnosis of paraneoplastic rhombencephalitis. We reviewed all available brain MR imaging in these patients, specifically evaluating for hallmark imaging findings of hypertrophic olivary degeneration, including T2 hyperintensity and enlargement of the inferior olives.

Out of the 30 patients with paraneoplastic rhombencephalitis reviewed, 6 had typical imaging findings of hypertrophic olivary degeneration. One patient had lesions in the dentate nuclei, whereas the other 5 had no other brainstem or cerebellar lesions. One of these 6 patients had palatal myoclonus. Serum and/or CSF antibodies detected included KLHL11 (n=2), Ma-2 (n=2), ANNA-2 (n=1), and PCA-1 (n=1). Underlying malignancies in the patients included testicular carcinoma (n=4) and breast cancer (n=2). All six patients had progressive disease course despite aggressive immunotherapy.

 

 
Our study suggests a potential novel association between paraneoplastic rhombencephalitis and hypertrophic olivary degeneration. Knowledge of this association is important. Hypertrophic olivary degeneration could potentially help corroborate a diagnosis of paraneoplastic rhombencephalitis. Moreover, hypertrophic olivary degeneration could be a marker of poor prognosis, given that all six of our patients declined despite aggressive immunotherapy.
Authors/Disclosures
Ajay Madhavan
PRESENTER 		Ajay Madhavan has nothing to disclose.
Divyanshu Dubey, MD, FAAN (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.
Carrie Carr (Mayo Clinic - Rochester) Carrie Carr has nothing to disclose.
Karl Krecke Karl Krecke has nothing to disclose.
No disclosure on file
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.