Neuropathies associated to malignant lymphomas

Abstract

Background: Malignant lymphomas account for 3 – 4% of all cancers. The nerve damage in non-Hodgkin’s (NHL) and Hodgkin’s lymphoma (HL) may occur prior to the clinical expression of lymphoma or develop over time. Often, patients address neurologists without known lymphoma. Lymphomaassociated neuropathy shall be differentiated from the complications and side effects of lymphoma treatment. NHL is responsible for the most peripheral nerve complications. Diffuse nerve infiltration is the major cause of neuropathy with axonal damage. The clinically developed entities vary from multiple asymmetric mononeuropathy, polyneuropathy or plexopathy to more generalized patterns like polyradiculoneuropathy. The alteration of the peripheral nerves in HL is less common. HL implies immunological mediated inflammation and extensive demyelination, such as Guillain-Barré syndrome. Some patients, including those with neurolymphomatosis, register a positive response to immunomodulatory treatments, such as steroids and IVIG. In this regard, neurolymphomatosis is frequently misdiagnosed as chronic inflammatory demyelinating polyneuropathy. The electrodiagnostic criteria of definite chronic inflammatory demyelinating polyneuropathy of European Federation of Neurological Societies (EFNS) and Peripheral Nerve Society increase the accuracy of the diagnosis. Conclusions: Accurate clinical assessment combined with electrophysiology exam facilitate the early diagnosis and interventions in malignant lymphoma. A lymphomatous neuropathy should be considered even if the diagnostic criteria of chronic inflammatory demyelinating polyneuropathy are met, particularly in patients with associated pain syndrome. Electrophysiological evaluation is mandatory in any neuropathy of obscure etiology where lymphomas are placed for differential diagnosis.