Figure 3. Treatment and outcome in 105 patients comparing presence and absence of tumour and the use of second-line immunotherapy.

First-line immunotherapy consists of corticosteroids, IVIg and plasma exchange given alone or in combination (detailed below). *Second-line immunotherapy, consists of rituximab or cyclophosphamide, or both. Substantial improvement is defined as previously reported,⁸ and includes full recovery or minimum deficits estimated by physicians and family members as recovery of more than 90% of function. All other patients are judged to have little improvement or no change. In patients with tumour, first-line treatment indicates tumour removal and in most cases first-line immunotherapy. Although there was no difference between the proportion of patients who achieved substantial recovery with or without tumour (Fisher’s exact test, p=0·16), patients with tumour responded to first-line treatment more often than did patients without tumour (p=0·001). Those without tumour required second-line immunotherapy more often and the 5 patients who died were in this group. Whether these patients had occult tumours is unknown.