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Amyotrophic lateral sclerosis

Thyrotropin-releasing hormone and histidyl proline diketopiperazine in the spinal cord and cerebrospinal fluid

Division of Endocrinology (Dr. Jackson), Brown University, Rhode Island Hospital, Providence, RI; and the Division of Endocrinology (Ms. Forte and Dr. Lechan) and Neuromuscular Research Unit, Departments of Neurology (Dr. Munsat) and Pathology (Neuropathology) (Dr. Adelman), Tufts New England Medical Center, Boston, MA.

In spinal cords from seven amyotrophic lateral sclerosis (ALS) patients and four controls, we found no difference in thyrotropin-releasing hormone (TRH) concentration relative to protein content, but there was a reduction per tissue wet weight in ALS. Immunohistochemical localization of TRH in ALS cord was unaltered. Histidyl proline diketopiperazine (HisPro-DKP), a possible metabolite of TRH, was significantly elevated per protein content in ALS. CSF levels of TRH and HisPro-DKP were unchanged. These findings suggest that TRH neurons are not primarily affected in ALS, but TRH and tissue protein are lost together as the disease progresses.

Address correspondence and reprint requests to Dr. Jackson, Division of Endocrinology, Rhode Island Hospital, 593 Eddy Street, Providence. RI 02902.

Supported by NIH Grant AM 34540 to I.M.D. Jackson; NIH Grant MO1RR00054 (General Clinical Research Center), the Amyotrophic Lateral Sclerosis Society of America, to R.M. Lechan and T.L. Munsat; and grants from the Muscular Dystrophy Association.

Presented in part at the thirty-sixth annual meeting of the American Academy of Neurology. Boston, MA, April 1984.

Accepted for publication January 13, 1986.

This article has been cited by other articles:

				H. Mitsumoto, M. R. Hanson, and D. A. Chad Amyotrophic Lateral Sclerosis: Recent Advances in Pathogenesis and Therapeutic Trials Arch Neurol, February 1, 1988; 45(2): 189 - 202. [Abstract] [PDF]