Bladowska Joanna, Kulej Dominika, Biel Anna, Zimny Anna, Kałwak Krzysztof, Owoc-Lempach Joanna, Porwolik Julita, Stradomska Teresa Joanna, Zaleska-Dorobisz Urszula, Sąsiadek Marek J
Department of General Radiology, Interventional Radiology and Neuroradiology, Chair of Radiology, Wrocław Medical University, Wrocław, Poland.
Department of Pediatric Bone Marrow Transplantation, Hematology and Oncology, Wrocław Medical University, Wrocław, Poland.
Pol J Radiol. 2015 Apr 10;80:181-90. doi: 10.12659/PJR.893285. eCollection 2015.
The aim of the study was to analyse MR images of the brain, including advanced MR techniques, such as single voxel spectroscopy (MRS) and diffusion tensor imaging (DTI), in children with X-linked adrenoleukodystrophy (X-ALD) before and after haematopoietic stem cell transplantation (HSCT) and to establish the imaging criteria which may be helpful in the assessment of disease staging, qualification to HSCT and follow-up.
MATERIAL/METHODS: Seven boys, aged 5-10 years, (mean 8.14 years) with biochemically proved X-ALD, underwent plain MR imaging with a 1.5 T unit before and after HSCT. Structural images were analyzed using an MRI severity scale (Loes scale). In one patient the follow-up examinations included MRS with the assessment of metabolite ratios (NAA/Cr, Cho/Cr, mI/Cr), as well as DTI with evaluation of fractional anisotropy (FA) and apparent diffusion coefficient (ADC) in several white matter tracts.
Two boys had an MRI severity score before HSCT equal to <8 points, and after HSCT they showed no clinical or radiological progression. In 5 patients with a higher severity score (from 8 to 16 points, mean 10.9) before HSCT, clinical and radiological progression was observed (MRI severity score from 17 to 25 points, mean 20.9). Follow-up advanced MRI techniques in one boy showed metabolic alterations, as well as decreased FA and ADC values in all evaluated areas.
Children at an early stage of X-ALD (below 8 points in MRI severity scale) are more likely to benefit from HSCT. DTI and MRS seem to be more useful imaging methods to assess the progression of X-ALD.
本研究旨在分析X连锁肾上腺脑白质营养不良（X-ALD）患儿造血干细胞移植（HSCT）前后的脑部磁共振成像（MR）图像，包括先进的MR技术，如单体素磁共振波谱分析（MRS）和弥散张量成像（DTI），并建立有助于疾病分期评估、HSCT资格认定及随访的成像标准。
材料/方法：7名年龄在5至10岁（平均8.14岁）、生化检查证实为X-ALD的男孩在HSCT前后接受了1.5T磁共振设备的平扫MR成像。使用MRI严重程度量表（Loes量表）分析结构图像。1例患者的随访检查包括MRS以评估代谢物比率（NAA/Cr、Cho/Cr、mI/Cr），以及DTI以评估多个白质束的分数各向异性（FA）和表观扩散系数（ADC）。
2名男孩在HSCT前MRI严重程度评分<8分，HSCT后未出现临床或影像学进展。5例在HSCT前严重程度评分较高（8至16分，平均10.9分）的患者出现了临床和影像学进展（MRI严重程度评分从17至25分，平均20.9分）。1名男孩的随访先进MR技术显示所有评估区域均有代谢改变以及FA和ADC值降低。
X-ALD早期（MRI严重程度量表低于8分）的儿童更有可能从HSCT中获益。DTI和MRS似乎是评估X-ALD进展更有用的成像方法。
