Substance P immunoreactivity in Rett syndrome - 06/09/11

Doi : 10.1016/S0887-8994(00)00120-X

Kimiko Deguchi, MD, PhD , ^†,^‡,^§, Barbara A Antalffy, BSc , ^‡, LaVina J Twohill, MA , ^‡, Subhendu Chakraborty, MSc ^‡, Danial G Glaze, MD , ^†,^‡, Dawna D Armstrong, MD , ^†,⁎
Rett Center, Houston, Texas, USA
^† Texas Children’s Hospital, Houston, Texas, USA
^‡ Baylor College of Medicine, Houston, Texas, USA
^§ Department of Mental Retardation and Birth Defect Research; National Institute of Neuroscience; National Center of Neurology and Psychiatry; Kodaira and Department of Pediatrics; Kitasato University School of Medicine; Sagamihara, Kanagawa, Japan

^*Communications should be addressed to: Dr. Armstrong; Department of Pathology; Texas Children’s Hospital; 6621 Fannin; Houston TX 77030

Abstract

Severe autonomic dysfunction occurs in Rett syndrome (RS). Substance P, a tachykinin peptide that localizes to several brain regions, including the autonomic nervous system, is reduced in the cerebrospinal fluid of patients with RS. The anatomic localization and intensity of substance P immunoreactivity and glial fibrillary acidic protein-positive astrocytes in the brains of 14 patients with RS were compared with those in the brains of 10 age-matched normal patients. Substance P immunoreactivity expression was significantly decreased in RS tissue compared with control tissue in the following regions: dorsal horns, intermediolateral column of the spinal cord, spinal trigeminal tract, solitary tract and nucleus, parvocellular and pontine reticular nuclei, and locus ceruleus. A less significant decrease of substance P immunoreactivity occurred in the substantia nigra, central gray of the midbrain, frontal cortex, caudate, putamen, globus pallidus, and thalamus. Antiglial fibrillary acidic protein-positive astrocytes were increased in the areas in which substance P immunoreactivity was decreased and in other brain regions. Because many of the brain regions with the greatest decrease in substance P immunoreactivity are involved in the control of the autonomic nervous system, especially the solitary tracts and reticular formation, reduced substance P may contribute to the autonomic dysfunction in RS.

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