Kitaguchi H, Tomimoto H, Miki Y, Yamamoto A, Terada K, Satoi H, Kanda M, Fukuyama H
Department of Neurology, Graduate School of Medicine, Kyoto University, Sakyo-ku, Kyoto 606-8507, Japan.
Neuroradiology. 2005 Sep;47(9):652-6. doi: 10.1007/s00234-005-1399-z. Epub 2005 Jun 10.
Reversible posterior leukoencephalopathy syndrome (RPLS) is caused by various heterogeneous factors, the commonest being hypertension, followed by nonhypertensive causes such as eclampsia, renal diseases and immunosuppressive therapy. Patients with RPLS exhibit bilateral white and gray matter abnormalities in the posterior aspects of the cerebral hemispheres. However, this syndrome may affect the brainstem predominantly, and these cases are designated as hypertensive brainstem encephalopathy. We present here two patients with reversible brainstem encephalopathy: one with hypertension and the other without hypertension. These patients presented with swelling and diffuse hyperintensities of the brainstem in fluid-attenuated inversion-recovery (FLAIR) and T2-weighted MRI, but with relatively mild clinical symptoms. They recovered without major neurological deficits, but had residual lacunar lesions in the pons. Reversible brainstem encephalopathy with characteristic MRI features was found in both hypertensive and nonhypertensive patients. These patients were diagnosed with a brainstem variant of RPLS, which is potentially fully reversible after an adequate treatment, and therefore should be carefully differentiated from other brainstem disease conditions.
可逆性后部白质脑病综合征(RPLS)由多种不同因素引起,最常见的是高血压,其次是非高血压病因,如子痫、肾脏疾病和免疫抑制治疗。RPLS患者在大脑半球后部出现双侧白质和灰质异常。然而,该综合征可能主要影响脑干,这些病例被称为高血压脑干脑病。我们在此报告两名可逆性脑干脑病患者:一名患有高血压,另一名无高血压。这些患者在液体衰减反转恢复(FLAIR)和T2加权磁共振成像(MRI)上表现为脑干肿胀和弥漫性高信号,但临床症状相对较轻。他们康复后无严重神经功能缺损,但脑桥有残留腔隙性病变。高血压和非高血压患者均发现具有特征性MRI表现的可逆性脑干脑病。这些患者被诊断为RPLS的脑干变异型,经适当治疗后可能完全可逆,因此应与其他脑干疾病仔细鉴别。