Pathology of myelinated fibers in cervical and lumbar ventral spinal roots in amyotrophic lateral sclerosis

Abstract

Pathological alterations were evaluated by morphometry and by a teased-fiber study on the 6th cervical (C6) and the 4th lumbar (L4) ventral spinal roots of cases of amyotrophic lateral sclerosis (ALS). The large-diameter myelinated fibers were severely affected in both spinal segments. However, small-diameter myelinated fibers were numerically well preserved. The number of large fibers in C6 and L4 ventral roots was strongly correlated to the strength of muscles innervated by C6 or L4 segments. There was no correlation of the number of small fibers with muscle strength. Teased fiber studies revealed a marked increase in the incidence of fibers showing axonal degeneration. Fibers considered to be regenerative were rarely observed. These observations suggest that large myelinated fibers, which correspond to α-motoneuron fibers, are selectively affected, and that small myelinated fibers, which are considered to correspond to γ-motoneuron fibers, are preserved to some extent in the C6 and L4 ventral spinal roots in ALS.