THYROTROPIN-RELEASING-HORMONE AND ITS RECEPTOR IN THE SPINAL-CORD OF PATIENTS WITH X-LINKED RECESSIVE BULBOSPINAL MUSCULAR-ATROPHY - AN IMMUNOHISTOCHEMICAL STUDY

We have studied the distributions of thyrotropin-releasing hormone (TR H) and TRH receptor (TRH-R) in the spinal cord of patients with X-link ed recessive bulbospinal muscular atrophy (SBMA), using specific antib odies to TRH and TRH-R. In the lumbar spinal cord, the percentage of T RH-positive cells was significantly higher among the large- and medium -sized neural cells of the ventral horn but not in the dorsal horn, as compared to the controls. The percentage of TRH-R-positive cells amon g the large- and medium-sized ventral horn cells was lower than in the controls. In the lumbar spinal cord, percentages of large-, medium- a nd small-sized neural cells in the dorsal horn were significantly lowe r as compared with the controls. These findings indicate that the perc entage of TRH-positive cells increased, and that of TRH-R-positive cel ls decreased, in the ventral horn of the lumbar spinal cord of SBMA pa tients. These changes may play a pathophysiological role in SBMA, Med Sci Res 26:525-529 (C) 1998 Lippincott Williams & Wilkins.