MRI demyelination pattern and clinical course in a child with cerebral X-linked adrenoleukodystrophy (X-ALD)

被引:7
作者
Nowak, Johannes [1 ,2 ]
Loebel, Ulrike [3 ]
Woelfl, Matthias [4 ]
Schlegel, Paul-Gerhardt [4 ]
Warmuth-Metz, Monika [1 ,2 ]
机构
[1] Univ Hosp Wurzburg, Dept Radiol, D-97080 Wurzburg, Germany
[2] Univ Hosp Wurzburg, Dept Neuroradiol, D-97080 Wurzburg, Germany
[3] Univ Med Ctr Hamburg Eppendorf, Dept Neuroradiol, Hamburg, Germany
[4] Univ Hosp Wurzburg, Dept Pediat, D-97080 Wurzburg, Germany
关键词
X-linked adrenoleukodystrophy (X-ALD); magnetic resonance imaging (MRI); Loes score; demyelination; hematopoietic stem cell transplantation;
D O I
10.1177/2047981615573655
中图分类号
R8 [特种医学]; R445 [影像诊断学];
学科分类号
1002 ; 100207 ; 1009 ;
摘要
The clinical spectrum in boys with X-linked adrenoleukodystrophy (X-ALD) ranges from isolated adrenocortical insufficiency and slowly progressive myelopathy to devastating cerebral demyelination. In the individual case, the disease course still remains unpredictable. Research findings suggest an important role of brain magnetic resonance imaging (MRI) lesion patterns as prognostic markers for X-ALD. Hence, familiarity with imaging features of childhood X-ALD in combination with clinical manifestation is required in order to stratify affected patients for therapy. We report on MRI findings and clinical course of cerebral X-ALD in a young boy with a rare subtype of white matter demyelination.
引用
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页数:3
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