Serum level of CNTF is elevated in patients with amyotrophic lateral sclerosis and correlates with site of disease onset.

We measured serum levels of neurotrophic cytokines ciliary neurotrophic factor (CNTF) and leukaemia inhibiting factor (LIF) in 96 patients either with familial amyotrophic lateral sclerosis (FALS, n = 18) or sporadic ALS (SALS, n = 78) and in 27 inflammatory neurological controls (13 multiple sclerosis and 14 Guillain-Barré syndrome) and in 27 healthy controls. Serum level of CNTF was significantly higher in ALS patients than in inflammatory neurological controls or healthy controls, and significantly higher in patients with ALS onset from upper or lower extremities than in patients with a purely bulbar onset of the disease. Serum CNTF levels did not significantly differ between patients with FALS and SALS, and it did not correlate with the age of onset or duration of the disease. No detectable serum levels of LIF were observed in the patient groups or in the healthy controls.