Abstract Details

Title Anti-Ri-Associated Paraneoplastic Brainstem Cerebellar Syndrome with Medically-Intractable Nausea in a Patient with Large Cell Neuroendocrine Lung Carcinoma: A Case Report

Topic Neuro-oncology

Presentation(s) P01 - (-)

Poster/Presentation
Number 113

Objective

Background Paraneoplastic neurologic syndromes (PNS) are rarely the first manifestations of occult malignancies. If left untreated, PNS often lead to significant morbidity and mortality. The presence of onconeural antibodies supports the paraneoplastic diagnosis, but is not absolutely essential. Anti-Ri autoantibodies are commonly associated with breast and small cell lung cancers. Cases of anti-Ri paraneoplastic cerebellar degeneration are reported, but none describe associated severe nausea and emesis as the major presenting and enduring symptoms.

Design/Methods Case report.

Results We report a case of a 75-year-old female with medically-intractable nausea, vomiting, diplopia, vertigo, and gait ataxia progressive over three months. Physical exam revealed rotary nystagmus, ocular skew deviation, horizontal diplopia, limb dysmetria, and gait ataxia. Paraneoplastic disease was suspected when neuroimaging and cerebrospinal fluid analysis returned unremarkable. After two courses of intravenous immunoglobulin, there was minimal improvement. Serum was anti-Ri antibody positive. Nuclear FDG positron emission tomography (PET/CT) detected a hypermetabolic region in the left lower lung lobe. The tumor measured 2.5 by 2.2 by 1.8 cm3 and histopathology revealed large cell neuroendocrine carcinoma with smaller admixed portions of adenocarcinoma. Final pathologic staging was T2 N0 M0. Six months after lower lung lobectomy and thorascopic lymphadenectomy, the patient clinically improved with nearly complete resolution of vertigo, diplopia and ataxia and complete resolution of nausea.

Conclusions This is the first report of paraneoplastic brainstem cerebellar syndrome involving anti-Ri antibody positivity from neuroendocrine tumor of the lung with nausea and vertigo as the major features and with marked improvement after tumor resection. Clinicians should suspect paraneoplastic syndromes from occult malignancy in patients with neurologic manifestations for which no cause can be found. The only definitive treatment for PNS is timely eradication of the underlying tumor.

Authors/Disclosures
Amber Mitchell, MD PRESENTER	Dr. Mitchell has nothing to disclose.
Earl A. Zimmerman, MD, FAAN (Albany Medical College)	No disclosure on file
	No disclosure on file

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