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Paraneoplastic striatal encephalitis and myelitis associated with anti-CV2/CRMP-5 antibodies in a patient with small cell lung cancer

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Paraneoplastic neurological syndromes (PNS) affecting the central nervous system are infrequent, presenting in< 1% of all those with cancer. The relevant auto-antibodies, that are detected in serum or cerebrospinal fluid,… Click to show full abstract

Paraneoplastic neurological syndromes (PNS) affecting the central nervous system are infrequent, presenting in< 1% of all those with cancer. The relevant auto-antibodies, that are detected in serum or cerebrospinal fluid, can target either neuronal cytoplasmic/nuclear proteins or neuronal cell surface proteins such as ion channels. The type of antibodies detected may determine the underlying malignancy and also response to immunotherapy. Anti-CV2/CRMP-5 is a 62-kDa neuronal cytoplasmic protein of the collapsin response-mediator family that is usually correlated with thymoma or small cell lung cancer (SCLC). The PNS associated with antiCV2/CRMP-5 is generally characterized by encephalomyelitis, paraneoplastic sensory neuronopathy, uveitis or chorea [1]. We have recently encountered a patient with prominent motor weakness and behavioral changes whose cranial MR investigation was compatible with striatal encephalitis and spinal MR imaging revealed longitudinal myelitis. Paraneoplastic antibody screening was positive for anti-CV2/ CRMP-5 antibodies. Meticulous workup disclosed small cell lung cancer (SCLC) as the underlying pathology.

Keywords: anti cv2; cancer; cell; small cell; cv2 crmp

Journal Title: Clinical Neurology and Neurosurgery
Year Published: 2018

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