Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, Anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone

Objectives-To evaluate the effect of a combination of immunoglobulins (IVIg ), cyclophosphamide (CTX), and methylprednisolone (MP) on the clinical cour se of patients with paraneoplastic neurological syndrome (PNS) and antineur onal antibodies (Abs). Methods-Seventeen patients with paraneoplastic encephalomyelitis/sensory ne uropathy (PEM/SN) with anti-Hu Abs (n=10) or cerebellar degeneration (PCD) with anti-Yo Abs (n=7) received one to nine cycles (mean 3.5) of a combinat ion of IVIg (0.5 g/kg/day from days 1 to 5), CTX (600 mg/m2 at day 1) and M P (1g/day from day 1 to 3). The Rankin scale (RS) was used to evaluate the response. A positive response was considered as either improvement or stabi lisation in patients who were still ambulatory (RS less than or equal to 3) at the onset of treatment, whereas only improvement, and not stabilisation , was considered a therapeutic benefit in bedridden patients (RS greater th an or equal to 4). Results-Tolerance was good and no patient experienced grade 3/4 toxicity (W orld Health Organisation). Sixteen patients were evaluable for response. Of the seven patients with RS greater than or equal to 4, none improved. Of t he nine patients with RS greater than or equal to 3, none improved but thre e (two SN and one PCD) stabilised for 4, 35, and 16 months. Conclusions-This study suggests that vigorous immunosuppressive treatment i s not useful in severely disabled PNS patients with antineuronal Abs. In a minority of patients (mainly with SN) who are not severely disabled at the onset of treatment, a transient stabilisation is possible and deserves furt her evaluation.