Paraneoplastic neuropathies

This review describes relevant advances in paraneoplastic neuropathies with emphasis on particular syndromes and the impact of new therapies.

Sensory neuronopathy may present with symptoms that do not raise the suspicion of a paraneoplastic origin. A recent study on sensory neuronopathies of different causes identified paraneoplastic cases in a group of older (>60 years) male patients with subacute onset early pain, and frequent involvement of the arms. Paraneoplastic sensorimotor polyneuropathies may be confused with chronic inflammatory demyelinating polyneuropathy (CIDP) and in lymphomas with direct infiltration of nerves (neurolymphomatosis). Recent neurophysiological studies indicate that the polyneuropathy of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M component, and skin changes) can be differentiated from CIDP by the presence of diffuse demyelination and more severe axonal loss. Neuropathy in Waldenström macroglobulinemia is heterogeneous. Up to 38% have demyelinating features and the rest show axonal degeneration due to different causes (dysimmune, amyloidosis, or tumoral infiltration). Isolated case reports suggest that the combination of cyclophosphamide and rituximab may be effective in paraneoplastic neuronopathies. Lenalidomide and dexamethasone are effective to control the neuropathy of POEMS patients who are not suitable for or progress after autologous stem cell transplantation.

Clinical and neurophysiological studies are helpful to correctly identify particular paraneoplastic neuropathies.