Kwon S, Koo J, Lee S
Department of Pediatrics, Kyungpook National University Hospital, Samdeok 2-50, Joong-Gu, Taegu 7000-721, South Korea.
Pediatr Neurol. 2001 May;24(5):361-4. doi: 10.1016/s0887-8994(01)00265-x.
Reversible posterior leukoencephalopathy syndrome is a recently recognized disorder with characteristic radiologic findings that mainly involve the white/gray matter of the parieto-occipital lobes. This complex syndrome is associated with cyclosporine A therapy or a variety of other conditions in which blood pressure rises acutely. Twelve patients from a variety of conditions who met the diagnostic criteria for this syndrome were studied. Interestingly, three of these patients had intra-abdominal neurogenic tumors, which have rarely been reported. Initial cranial magnetic resonance imaging scans revealed fairly symmetric areas of increased T(2) signal involving both white and gray matter of parieto-occipital lobes in the majority of the patients. However, the lesions were often located outside the parieto-occipital regions. Four patients had occipital region magnetic resonance spectroscopy during the acute phase, which revealed high lactate peak and normal N-acetyl aspartate/creatine and choline peaks. With appropriate treatment, most patients recovered from this syndrome and experienced almost complete resolution of brain lesions on follow-up magnetic resonance imaging. One patient, however, continued to have small residual hemosiderin deposits on a follow-up magnetic resonance imaging with neurologic sequellae. In conclusion, a better understanding of this complex syndrome may obviate unnecessary investigations and allow management of the associated problems in prompt and appropriate ways.
可逆性后部白质脑病综合征是一种最近才被认识的疾病,具有特征性的影像学表现,主要累及顶枕叶的白质/灰质。这种复杂的综合征与环孢素A治疗或多种其他血压急性升高的情况有关。对12名符合该综合征诊断标准的各种疾病患者进行了研究。有趣的是,其中3名患者患有腹腔内神经源性肿瘤,这种情况很少有报道。最初的头颅磁共振成像扫描显示,大多数患者顶枕叶白质和灰质的T(2)信号增强区域相当对称。然而,病变常常位于顶枕叶区域之外。4名患者在急性期进行了枕叶磁共振波谱分析,结果显示乳酸峰升高,N-乙酰天门冬氨酸/肌酸和胆碱峰正常。经过适当治疗,大多数患者从该综合征中康复,随访磁共振成像显示脑病变几乎完全消退。然而,有1名患者在随访磁共振成像时仍有小的残留含铁血黄素沉积,并伴有神经后遗症。总之,更好地了解这种复杂的综合征可能避免不必要的检查,并能及时、恰当地处理相关问题。