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  • Title: Decreased cerebrospinal fluid levels of substance P in patients with Rett syndrome.
    Author: Matsuishi T, Nagamitsu S, Yamashita Y, Murakami Y, Kimura A, Sakai T, Shoji H, Kato H, Percy AK.
    Journal: Ann Neurol; 1997 Dec; 42(6):978-81. PubMed ID: 9403492.
    Abstract:
    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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