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Paraneoplastic downbeat nystagmus and cerebellar ataxia due to small cell lung carcinoma

By Department of Neurology The Johns Hopkins School of Medicine MD Kelly Sloane

Abstract

This is a 61-year-old woman (non-smoker) who developed a gait disorder, dizziness and oscillopsia that was progressive over 2 months. Exam demonstrated spontaneous downbeat nystagmus with side pocket nystagmus in lateral gaze (a combination of horizontal gaze-evoked and downbeat nystagmus, giving an oblique [down and out] appearance), in addition to hypermetric saccades, saccadic smooth pursuit and VOR suppression (not shown in this video). Mild limb dysmetria and gait ataxia were also present. MRI was normal, and there was a mild CSF pleocytosis. CT of the chest/abdomen/pelvis revealed a left hilar mass with extension into the bronchus, and biopsy revealed small cell carcinoma of the lung. It was thought that a paraneoplastic cerebellopathy was the most likely culprit despite a negative serum paraneoplastic panel. There was subjective improvement in vertical oscillopsia and objective improvement in her downbeat nystagmus with IVIG followed by chemotherapy. ; Paraneoplastic cerebellar disease typically develops over the course of days to weeks, and can be accompanied by other bulbar signs or symptoms. Most commonly, as in this case, presentation precedes cancer detection and recognition may allow for expedited diagnosis and treatment of the responsible malignancy. Work up may reveal inflammatory changes (e.g., pleocytosis) within the CSF, and autoantibodies may be detected. The most commonly found antibody-malignancy pairs are anti-Yo (ovarian and breast), anti-Hu (small cell lung carcinoma) and anti-Tr or anti-mGluR1 (both Hodgkins lymphoma). When an older patient presents with the subacute onset of a cerebellar syndrome, the clinician should have a high suspicion for a paraneoplastic disorder.Number of Videos and legend for each:1, Patient presenting with subacute onset of downbeat nystagmus and cerebellar ataxia due to presumed paraneoplastic etiolog

Topics: Jerk Nystagmus, Downbeat Nystagmus, Cerebellar Ataxia, Carcinoma
Publisher: Spencer S. Eccles Health Sciences Library, University of Utah
Year: 2017
OAI identifier: oai:collections.lib.utah.edu:ehsl_novel_gold/1277124
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