Abstract
Rett syndrome (RS) is a progressive neurological pediatric disorder which affects only girls. The aim of our study is to determinate if brain perfusion abnormalities might be able to explain the clinical manifestations and progression of the disease. Quantitative global and regional brain blood flow was evaluated in 25 RS girls and compared with a reference group of 9 children. SPET revealed a considerable global reduction in cerebral perfusion in the group of RS girls and a great statistical difference was recorded when compared with the control group. The reduction of cerebral perfusion reflects the functional disturbances in the brains of RS girls, which might be associated with precocious brain atrophy, even when morphologic imaging (MRI) appears normal.
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References
Rett A: Über ein eigenartiges hirnatrophisches syndrom bei hyperammonämie im kindesalter. Wien Med Wochenschr 1966; 116: 723–726.
Hagberg B: Rett syndrome: Swedish approach to analysis of prevalence and cause. Brain Dev 1985; 7: 277–279.
The Rett syndrome diagnostic criteria work group: Diagnostic criteria for Rett syndrome. Ann Neurol 1988; 23: 425–428.
Hagberg B, Witt-Engerström I: Rett syndrome: a suggested staging system for describing the impairment profile with increasing age towards adolescence. Am J Med Gen 1986; 24 (suppl.): 47–59.
Armstrong D: The neuropathology of Rett syndrome. Brain Dev 1992; 14: 89–94.
Hagberg B, Skjeldal OH: Rett variants: a suggested model for inclusion criteria. Pediatr Neurol 1990; 47: 982–986.
Elian M, Rudolf N de M: EEG and respiration in Rett syndrome. Acta Neurol Scand 1991; 83: 123–128.
Nielsen JB, Friberg L, Lou H, Lassen NA, Sam ILK: Immature pattern of brain activity in Rett syndrome. Arch Neurol 1990; 47: 982–986.
Yoshikawa H, Fucki N, Suzuki H, Sakuragawa N, Ilio M: Cerebral blood flow and oxygen metabolism in the Rett syndrome. Brain Dev 1992; 14 (suppl.): S69–S74.
Reiss AL, Faruque F, Naidu S, Abrams M, Beaty T, Bryan RN, Moser H: Neuroanatomy of Rett syndrome: a volumetric imaging study. Ann Neurol 1993; 34: 227–234.
Colamussi P, Giganti M, Cittanti C, Dovigo L, Trotta F, Tola MR, Tamarozzi R, Lucignani G, Piffanelli A: Brain single-photon emission tomography with 99mTc-HMPAO in neuropsychiatric systemic lupus erythematosus: relations with EEG and MRI findings and clinical manifestations. Eur J Nucl Med 1995; 22: 17–24.
Keymeulen B, de Metz K, Cluydts R, Bossuyt A, Somers G: Technetium-99m hexamethylpropylene amine oxime single-photon emission tomography of regional cerebral blood flow in insulin-dependent diabetes. Eur J Nucl Med 1996; 23: 163–168.
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© 1999 Springer Basel AG
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Burroni, L. et al. (1999). Assessment of Brain Perfusion by ECD-Tc99m SPET IN RETT Syndrome. In: Bergmann, H., Köhn, H., Sinzinger, H. (eds) Radioactive Isotopes in Clinical Medicine and Research XXIII. Advances in Pharmacological Sciences. Birkhäuser, Basel. https://doi.org/10.1007/978-3-0348-8782-3_41
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DOI: https://doi.org/10.1007/978-3-0348-8782-3_41
Publisher Name: Birkhäuser, Basel
Print ISBN: 978-3-0348-9772-3
Online ISBN: 978-3-0348-8782-3
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