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a Department of
Neurology, Hôpital de la Salpêtrière, Paris, France, b Unité INSERM 495, Hôpital de la
Salpêtrière, Paris, France, c Service of Neurology CS "Principes Espana"
Hospitalet del Llobregat, Spain, d Service
of Neurology, Hospital Clinic i provincial, Barcelona, Spain, e Service of Neurology B and Inserm U 433, Hôpital Neurologique, Lyon, France, f Unité INSERM 472. Hôpital P, Brousse,
Villejuif Cedex, France
Correspondence to: Dr Jean-Yves Delattre, Service de Neurologie, Hôpital de la Salpêtrière, 47 Boulevard de l'Hôpital, 75651 Paris Cedex 13, France
Received 22
March 1999 and in revised form 25 August 1999;
Accepted 3
September 1999
OBJECTIVES
To evaluate
the effect of a combination of immunoglobulins (IVIg), cyclophosphamide
(CTX), and methylprednisolone (MP) on the clinical course of patients
with paraneoplastic neurological syndrome (PNS) and antineuronal
antibodies (Abs).
METHODSSeventeen
patients with paraneoplastic encephalomyelitis/sensory neuropathy
(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
combination of IVIg (0.5 g/kg/day from days 1 to 5), CTX (600 mg/m2 at
day 1) and MP (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 stabilisation in patients who were still ambulatory
(RS
3) at the onset of treatment, whereas only improvement, and not
stabilisation, was considered a therapeutic benefit in bedridden
patients (RS
4).
RESULTSTolerance was
good and no patient experienced grade 3/4 toxicity (World Health
Organisation). Sixteen patients were evaluable for response. Of the
seven patients with RS4, none improved. Of the nine patients with
RS3, none improved but three (two SN and one PCD) stabilised for 4, 35, and 16 months.
CONCLUSIONSThis study
suggests that vigorous immunosuppressive treatment is 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 further evaluation.
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