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71-year-old Man with Nystagmus and Oscillpsia
Teaching NeuroImages Neurology Resident and Fellow section © 2016 American Academy of Neurology
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Vignette 71-year-old Caucasian man with a strong smoking history, without any known malignancy, presented with an eleven-month history of initial left monocular followed by binocular elliptical nystagmus and oscillopsia . Physical examination showed binocular elliptical nystagmus that did not improve with fixation (Video 1) and MRI brain revealed extensive periventricular T2- weighted signal changes also involving the corpus callosum (Image 1) Cerebrospinal fluid (CSF) revealed protein of 102 mg/dL; glucose, white blood cell count, cytology and high-volume flow cytometry were normal Anti-Neuronal-type Voltage-Gated Potassium Channel (VGKC) and anti-P/Q-type Voltage-Gated Calcium Channel (VGCC) antibodies titers were elevated in serum Mistry et al. © 2016 American Academy of Neurology
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Imaging Image 1. Extensive T2 FLAIR signal changes in the periventricular area, splenium, and genu of corpus callosum that is non enhancing. Mistry et al. © 2016 American Academy of Neurology
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Imaging Video 1: Binocular elliptical nystagmus that did not improve with fixation Mistry et al. © 2016 American Academy of Neurology
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P/Q-Type Voltage-gated Calcium Channel associated Paraneoplastic Elliptical Nystagmus
The patient was treated with carbamazepine(for symptomatic relief) followed by high dose intravenous methylprednisolone, resulting in a moderate improvement. No malignancy was found on PET scan and CT scan of his chest, abdomen, and pelvis. Anti-VGCC antibodies have been implicated in paraneoplastic nystagmus and small cell lung cancer is the most common associated malignancy.1,2 An underlying malignancy may or may not be present at the time of diagnosis; therefor, close follow up after a comprehensive workup is often required. Immunomodulatory therapies such as steroids, intravenous immunoglobulin, plasma exchange, and rituximab have been tried, but they often do not provide a complete resolution of the symptoms.2 References: 1. Ko MW, Dalmau J, Galetta SL. Neuro-ophthalmologic manifestations of paraneoplastic syndromes. J Neuroophthalmol. 2008;28(1):58-68. 2. Bekircan-Kurt CE, Derle Ciftci E, Kurne AT, Anlar B. Voltage gated calcium channel antibody-related neurological diseases. World J Clin Cases. 2015;3(3): Mistry et al. © 2016 American Academy of Neurology
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