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Natural History of Canavan Disease Revealed by Proton Magnetic Resonance Spectroscopy (1H‐MRS) and Diffusion-weighted MRI

Janson, C. G. ; McPhee, S. W. J. ; Francis, J. ; Shera, D. ; Assadi, M. ; Freese, A. ; Hurh, P. ; Haselgrove, J. ; Wang, D. J. ; Bilaniuk, L. ; Leone, P.

Neuropediatrics, 2006-08, Vol.37 (4), p.209-221 [Periódico revisado por pares]

Stuttgart: Thieme

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Título:
Natural History of Canavan Disease Revealed by Proton Magnetic Resonance Spectroscopy (1H‐MRS) and Diffusion-weighted MRI
Autor: Janson, C. G. ; McPhee, S. W. J. ; Francis, J. ; Shera, D. ; Assadi, M. ; Freese, A. ; Hurh, P. ; Haselgrove, J. ; Wang, D. J. ; Bilaniuk, L. ; Leone, P.
Assuntos: Age Factors ; Aspartic Acid - analogs & derivatives ; Aspartic Acid - metabolism ; Atrophy ; Biological and medical sciences ; Brain - pathology ; Canavan Disease - diagnosis ; Case-Control Studies ; Child, Preschool ; Confidence Intervals ; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases ; Diffusion Magnetic Resonance Imaging - methods ; Dipeptides - metabolism ; Female ; Humans ; Infant ; Investigative techniques, diagnostic techniques (general aspects) ; Magnetic Resonance Spectroscopy - methods ; Male ; Medical sciences ; Nervous system ; Neurology ; Protons ; Radiodiagnosis. Nmr imagery. Nmr spectrometry ; Rapid Communication ; Reference Values
É parte de: Neuropediatrics, 2006-08, Vol.37 (4), p.209-221
Descrição: Abstract Canavan disease is a childhood leukodystrophy caused by mutations in the gene for human aspartoacylase (ASPA), which leads to an abnormal accumulation of the substrate molecule N-acetyl-aspartate (NAA) in the brain. This study was designed to model the natural history of Canavan disease using MRI and proton magnetic resonance spectroscopy ( 1 H-MRS). NAA and various indices of brain structure (morphology, quantitative T1, fractional anisotropy, apparent diffusion coefficient) were measured in white and gray matter regions during the progression of Canavan disease. A mixed-effects statistical model was used to fit all outcome measures. Longitudinal data from 28 Canavan patients were directly compared in each brain region with reference data obtained from normal, age-matched pediatric subjects. The resultant model can be used to non-invasively monitor the natural history of Canavan disease or related leukodystrophies in future studies involving drug, gene therapy, or stem cell treatments.
Editor: Stuttgart: Thieme
Idioma: Inglês

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Natural History of Canavan Disease Revealed by Proton Magnetic Resonance Spectroscopy (1H‐MRS) and Diffusion-weighted MRI

Janson, C. G. ; McPhee, S. W. J. ; Francis, J. ; Shera, D. ; Assadi, M. ; Freese, A. ; Hurh, P. ; Haselgrove, J. ; Wang, D. J. ; Bilaniuk, L. ; Leone, P.

Stuttgart: Thieme

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