Plasmapheresis and antineoplastic treatment in CNS paraneoplastic syndromes with antineuronal autoantibodies

Abstract

We retrospectively evaluated the effect of plasmapheresis (PE) in seven patients with paraneoplastic encephalomyelitis (PEM), small-cell lung carcinoma, and anti-Hu antibodies, and four patients with paraneoplastic cerebellar degeneration (PCD), ovarian or breast cancer, and anti-Yo antibodies. In addition to PE, patients received prednisone (nine), cyclophosphamide (eight), or treatment of the tumor (five). All but one patient were severely disabled by the time PE began. The clinical outcome was compared with that of five patients (PEM, four; PCD, one) who only had treatment of the tumor. Only one of these five patients had a severe neurologic deficit at the onset of the antineoplastic treatment. No patient improved. Two patients treated with PE and antineoplastic therapy and three who only received treatment of the tumor remained stable for at least 6 months. Four of the five patients with a stable course started the treatment when the neurologic deficit was not severe. We conclude that the efficacy of PE with other immunosuppressive therapies in the stabilization of the neurologic deficit is uncertain.

Improvement of Anti-Hu-associated Paraneoplastic Sensory Neuropathy after Chemoradiotherapy in a Small Cell Lung Cancer Patient.

“…This is in contrast to LEMS,which tends to improve in response to therapy probably because neuronal cell bodies are spared in this syndrome ( 13). Although prednisolone, azathioprine, immunoglobulin or plasmapheresis have been tried, these have not proved effective for most PSN patients (14). The patient in this report presented with marked abnormalities in sensory nerves in contrast to the minimal change in motor nerve function.…”

Paraneoplastic Cerebellar Degeneration (PCD) Associated with Squamous Cell Carcinoma of the Lung

“…Treatment of PCD with plasmapheresis (20) and immunosuppressive therapy (21) has been reported, but the results are not encouraging. In several reports, antineoplastic treatment (surgery or chemotherapy) has been effective for neurological symptoms to some extent (22,23).…”

Management of Immune-Mediated Paraneoplastic Neurological Disorders

“…Should the patient not improve with these regimes, treatment escalation to an apheresis therapy (plasma exchange or immunoadsorption) can be undertaken. Basically, IVIG treatment and apheresis therapies are more effective in patients with Abs directed against surface antigens compared to in-tracellular antigens with mainly T cell-mediated immune responses [3,16,19,20]. If the patient does no improve with acute treatment, an early (typically up to 2 weeks after the above-mentioned primary treatment) escalation to cyclophosphamide (as a shortterm high-dose treatment with 750-1000 mg/m 2 IV) or rituximab (e. g., 500 mg IV on days 1 and 15) can be undertaken [16,21,22].…”