Quantitative Spectroscopic Imaging in Metachromatic Leukodystrophy: value in prognosis and treatment monitoring.
Diane van Rappard1, Antoine Klauser2, Marjan Steenweg1, Marjo van der Knaap1, Nicole Wolf1, and Petra Pouwels3
1Child Neurology, VU University Medical Center, Amsterdam, Netherlands, 2Centre d'Imagerie BioMédicale, Geneva University, Geneva, Switzerland, 3Physics & Medical Technology, VU University Medical Center, Amsterdam, Netherlands
Currently, hematopoietic stem cell
transplantation (HSCT) is the only treatment
option for patients with metachromatic leukodystrophy (MLD). This study in
MLD patients and controls investigated the possible additional prognostic value
of quantitative MRSI. In WM (consisting of lesions and NAWM), ratios of Cho/NAA
and Ins/NAA were significantly higher in patients who were considered non-eligible for HSCT than in eligible patients. Follow-up
of successfully treated patients showed partial normalization of concentrations
study suggests that quantitative MRS can support the decision whom to treat,
especially when neurological and cognitive examinations are ambiguous.
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