Functional and morphometric study of the liver in motor neuron disease.

In routine liver function tests, 23 of 44 patients with motor neuron disease (MND) had abnormal findings, and there was disturbance of unconjugated bilirubin metabolism in 10 of the 33 patients tested. Liver-biopsy specimens from 10 MND patients were compared by electron microscopic examination with specimens from age-matched controls who had chronic persistent hepatitis. The MND patients had a higher incidence of intramitochondrial inclusions, less abundant mitochondria in a given area of cytoplasm and enlarged mitochondria. Electron-probe X-ray microanalysis of hepatocytic lysosomes found copper in 8 of 13 MND patients, but not in the controls. These findings suggest that the pathogenetic processes in MND may involve not only motor neurons but also hepatic cells.