A hereditary neurological disease in a family in Norway has been reported recently. The disease, which we refer to as Skogholt's disease, is a demyelinating disorder of both the central and the peripheral nervous system with adult onset. We investigated whether changes in trace element concentrations could play a role in Skogholt's disease. Using high resolution inductively coupled plasma mass spectrometry, we determined 31 elements in cerebrospinal fluid (CSF), blood plasma and whole blood from these patients, multiple sclerosis patients and a control group. More than threefold increased levels of Cu and Fe, and a twofold increase in Zn were found in the CSF of Skogholt patients compared to controls. Several other significant differences in trace element levels were also found. The increased levels of Cu and Fe in CSF may indicate an active role of these metals in the pathogenesis of Skogholt's disease. Apparently, these metal ions are transferred into the CSF through their protein chelation, as raised protein levels were also seen. We suggest that redistribution of metals from transport proteins into vulnerable sites in the central (and peripheral) nervous system may initiate critical lesions.