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Paraneoplastic neuropathy: wide-ranging clinicopathological manifestations

Koike, Haruki; Tanaka, Fumiaki; Sobue, Gen

doi: 10.1097/WCO.0b013e32834a87b7
Nerve, neuro-muscular junction and motor neuron diseases: Edited by Gerard Said

Purpose of review Recent progress in serological screening for paraneoplastic autoantibodies and diagnostic imaging techniques to detect malignancies has resulted in a broadening of the concept of paraneoplastic neurologic syndromes through the characterization of nonclassical clinical features. The goal of this article was to review the recent literature describing the wide-ranging clinicopathological manifestations of paraneoplastic neuropathy.

Recent findings The classical feature of paraneoplastic neuropathy is subacute sensory neuronopathy; in addition, sensorimotor neuropathies, such as Guillain–Barré syndrome, chronic inflammatory demyelinating polyneuropathy, brachial plexopathy, and vasculitic neuropathy, are sometimes observed. Some studies also describe the occurrence of autonomic neuropathies, including autoimmune autonomic ganglionopathy and chronic gastrointestinal pseudo-obstruction. Whole-body fluorodeoxyglucose positron emission tomography (FDG-PET) or FDG-PET/computed tomography may be helpful to detect malignancies that cannot be detected by conventional screening tests. The presence of paraneoplastic neuropathy should be considered in all patients with malignancy and can occur at any point in the disease, even during or after chemotherapy, radiation, or stem cell transplantation. The presence of paraneoplastic autoantibodies, especially anti-Hu and anti-CV2/CRMP-5 antibodies, may support the diagnosis of paraneoplastic neuropathy. Immunomodulatory treatment before, during, or after antineoplastic therapy may be of benefit for patients with paraneoplastic neuropathy and has been used even when the underlying malignancy cannot be identified.

Summary Recognition of the variable manifestations of paraneoplastic neuropathy is important, as diagnosis at an earlier stage facilitates prompt treatment and provides better chances of good outcomes.

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan

Correspondence to Fumiaki Tanaka, MD or Gen Sobue, MD, Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan Fax: +81 52 744 2384; e-mail:

© 2011 Lippincott Williams & Wilkins, Inc.