Marchiafava-Bignami病的临床及影像学研究

Clinical and radiologic study in Marchiafava-Bignami disease

目的探讨Marchiafava-Bignami病（MBD）的临床及影像学改变。方法回顾性分析了7例MBD患者的临床和CT、MRI资料，包括病灶形态、分布、信号或密度改变等影像学特征；4例同时行CT和MRI检查，2例仅行CT检查，1例仅行MRI检查。结果本组患者急性型5例，均表现为胼胝体肿胀及长T1、长T2信号改变，均有双侧脑室周围白质、额叶皮层下白质对称性累及；慢性型2例，胼胝体明显萎缩变薄，并呈长T1、长T2信号及FLAIR像点片状或线样低信号灶。5例患者DM显示病灶区信号明显增高并有2例出现弥散受限改变。结论MBD具有特征性MRI表现，其影像学改变可能反映其临床及预后。

Objective To investigate the clinicoradiologic characteristics of Marchiafava- Bignami disease （MBD）, Methods The clinical and neuroimaging findings, including lesion morphology, distribution, signal intensity/density in 7 MBD patients were retrospectively analyzed, Diffusion-weighted imaging （DWI） was performed in 5 cases, Of the 7 cases, 4 underwent both CT and MRI, 2 only CT and 1 MRI. Results With extensive iso- or slightly hypo-intensity on T1-weighted images and hyper-intensity on T2-weighted images, 5 patients were manifested as acute onset, in which white matter （WM） was symmetrically involved in bilateral periventricular and frontal subcortical regions. Punctate or linear hypo-intensity on FLAIR images was found in the atrophied corpus callosum in 1 case presented as chronic type, with scattered patchy hyper-intensity in periventricular WM and frontal subcortical WM. DWI showed markedly hyper-intensity with diffusion restriction in 2 cases in acute phase with apparent diffusion coefficient （ADC） values （0.52-0.55）× 10^3 mm^2/s. Brain atrophy was found in all the 7 patients. Conclusions The MRI features of MBD are characteristic and may be associated with the clinical spectrum and prognosis.