Decreased cerebrospinal fluid levels of substance P in patients with Rett syndrome

Academic Article


  • To clarify the mechanism of brain and spinal cord impairment in Rett syndrome (RS), we measured the cerebrospinal fluid (CSF) levels of substance P in 20 patients with RS including 16 childhood patients and 4 adult patients. Findings were compared with those obtained in age-matched controls and diseased controls. The CSF level of substance P was significantly lower in patients with RS compared with controls. The alteration in the CSF level of substance P may be related to the neurological impairment, especially autonomic dysfunction, and neuropathological involvement of dorsal root ganglia and peripheral nerve observed in RS.
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    Author List

  • Matsuishi T; Nagamitsu S; Yamashita Y; Murakami Y; Kimura A; Sakai T; Shoji H; Kato H; Percy AK
  • Start Page

  • 978
  • End Page

  • 981
  • Volume

  • 42
  • Issue

  • 6