Seven consecutive patients with amyotrophic lateral sclerosis (ALS) we
re treated with intravenous immunoglobulins (IVIg; 0.4 g/kg per day fo
r 5 consecutive days followed by monthly 2-day infusions at the same d
aily dosage) continued with oral cyclophosphamide (1-2 mg/kg per day),
for 4-13 months (mean 8.1). Response to treatment was assessed by mea
ns of the Medical Research Council (MRC) rating scale for muscle stren
gth on 40 muscles (10 per Limb), a clinical scale for bulbar function
and a modified Rankin disability scale. All patients continued to dete
riorate during treatment on as regards both their MRC score and either
their bulbar or Rankin score or both. The progression of the disease
during treatment, expressed as the monthly variation in MRC score (mea
n = - 2.71; SD = 1.36), was no slower than that estimated before thera
py (mean = 1.81; SD = 0.93). Even if the results of this small, uncont
rolled study do not permit the exclusion of an effect of IVIg on the p
rogression of ALS, they also do not provide any evidence that this exp
ensive form of therapy consistently slows the course of the disease.