Paraneoplastic anti-CV2 antibodies react with peripheral nerve and are associated with a mixed axonal and demyelinating peripheral neuropathy

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Abstract
Subacute sensory neuronopathy with anti‐Hu antibodies is the best‐characterized paraneoplastic peripheral neuropathy associated with carcinoma. Anti‐CV2 antibodies, another group of paraneoplastic antibodies, react with a 66‐kd brain protein belonging to the family of Ulip/CRMP proteins. The manifestations associated with anti‐CV2 antibodies include cerebellar degeneration, uveitis, and peripheral neuropathy. Some of these patients also have anti‐Hu antibodies. We have compared the clinical, electrophysiological, and pathological characteristics of the peripheral neuropathy in 9 patients with anti‐CV2 antibodies (3 of whom also had anti‐Hu antibodies) and 12 patients with only anti‐Hu antibodies. Data for patients with anti‐Hu antibodies alone indicated subacute sensory neuronopathy. Patients with anti‐CV2 antibodies had a mixed axonal and demyelinating sensory motor neuropathy that was sometimes superimposed on subacute sensory neuronopathy when both anti‐CV2 and anti‐Hu antibodies were present. Unlike anti‐Hu antibodies, anti‐CV2 antibodies reacted with peripheral nerve antigens, as shown by their ability to bind to a 66‐kd protein in human and rat nerve on Western blot analysis and to immunolabel peripheral nerve axons and sensory neurons on immunohistochemical study. Ann Neurol 2001;49:214–221