Paraneoplastic syndromes are remote effects of cancer, usually by hormone like substances secreted by a cancer. They can range for very common, such as cancer induced fatigue or cachexia, to uncommon neurological syndromes., as in this case.
Early detection and treatment of the underlying tumor is the approach that offers the greatest chance for neurological improvement or symptom stabilization. It is unusual that a paraneoplastic polyneuropathy continues after complete response of the lymphoma.
There are no national guidelines. Several uncontrolled trials using intravenous immunoglobulin, steroids, plasma exchange, rituximab, or cyclophosphamide have been performed in small series of patients with PNS including SSN . The effect of these treatments is diffi cult to assess, but patients with neuropathy or a recent onset of the PNS may sometimes show benefi ts. However, a larger series failed to demonstrate a clear benefit of intravenous immunoglobulin, steroids, plasma exchange, or cyclo-phosphamide, alone or in combination
Orange D, Frank M, Tian S, Dousmanis A, Marmur R, Buckley N, Parveen S, Graber JJ, Blachre N, Darnell RB. Cellular immune suppression in paraneoplastic neurologic syndromes targeting intracellular antigens.Arch Neurol. 2012 Sep;69(9):1132-40
Greenlee JE. Treatment of paraneoplastic neurologic disorders. Curr Treat Options Neurol. 2010 May;12(3):212-30
C. A. Vedeler et al, CHAPTER 31 Paraneoplastic neurological syndromes, European Handbook of Neurological Management, Second Edition, Volume 1, Second Edition 2011