An immunohistochemical study of neurofilament and microtubule-associated Tau protein in the enteric innervation in Hirschsprung's disease.

The distribution and localization of neurofilament (NF) and microtubule-associated tau protein (Tau) in the colon from Hirschsprung's disease were examined by immunohistochemistry. Specimens of the normoganglionic, oligoganglionic, and aganglionic segments of colons from nine patients with Hirschsprung's disease were used in this study. Normal colon specimens obtained at the time of colostomy closure from two patients with anorectal malformations were also examined as controls. In normoganglionic segments, anti-NF-H and anti-NF-M immunoreactivity appeared within the nerve fibers of both the myenteric and submucosal plexuses. These findings were also observed in the oligoganglionic segments. In the aganglionic segment, hypertrophic nerve fascicules and the nerve fibers in circular muscle were positively stained with anti-NF-H and anti-NF-M antibodies. Anti-Tau staining appeared in the ganglion cell bodies of both myenteric and submucosal plexuses and in nerve fibers distributed among the circular muscles of the normal control colons, and the normoganglionic and oligoganglionic Hirschsprung's specimens. Nerve fibers in the circular muscle layer of aganglionic segments were also stained with anti-Tau serum, although the hypertrophic nerve fascicules in the intermuscular and submucosal layers did not stain. The so-called nerve fascicules distributed in the subserosal layer also did not stain with anti-Tau. These results suggest that Tau may be used as a specific marker to identify ganglion cells and intrinsic nerve fibers in colons affected by Hirschsprung's disease.