Clinical,imaging and pathological characteristics of twin brothers with adrenoleukodystrophy
- VernacularTitle:孪生兄弟患肾上腺脑白质营养不良的临床、影像学和病理特征
- Author:
Xiaonan SONG
;
Shihe LIN
;
Jiexu ZHAO
- Publication Type:Journal Article
- Keywords:
twin brothers;
adrenoleukodystrophy;
adrenomyeloneuropathy
- From:
Journal of Clinical Neurology
2001;0(05):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To explore clinic,imageology and pathological characteristics of twin brothers with adrenoleukodystrophy(ADL).Methods Clinical data of twin brothers with ALD and pathological data of one case were analyzed retrospectively.Results Clinical manitestation of elder brother was cerebral ADL,T1-weighted of MRI with low intensity lesion and T2-weighted with high intensity lesion were shown widely in the parietooccipital and postero-corpus callosum white matter.The pathological changes were myelinopothy diffused in the parietooccipital white matter,but U fibber was maintained in the subcortex.Clinical manitestation of the young brother was shown spinal damage.His cerebral MRI was normal.Spinal MRI had shown spinal cord thinning,line-like equal signal was found in the periphery of the lesion.He might be juvenile adrenomyeloneuropathy.Conclusions Although the twin brothers are both suffer from adrenoleukodystrophy,their clinical manifestation,MRI and pathological changes are different.