原发性侧索硬化的临床和影像学特征

目的 探讨胀发性侧索硬化（PLS）的临床和影像学特征。方法 对3例PLS患者的临床和影像学资料进行分析。结果 本组PLS患者女2例、男1例，均为缓慢起病。其中例1首发症状为右上肢僵硬、无力，例2为两下肢僵硬、无力，例3表观为假性延髓麻痹，随病情的进展，均出现四肢痉挛性瘫痪，肌力Ⅲ～Ⅳ级，肌张力呈折刀样增高，四肢腱反射亢进，两侧踝阵孪及Babinski征（＋），头颅MRI T2 WI可见两侧内囊、大脑脚、运动区皮质及腑桥丛底部有异常高信号，^1H磁共振波谱（^1HMRS）示在病灶区有乙酰天门冬氨酸盐（NAA）降低及NAA／肌酸（Cr）比值降低。结论 PLS的临床症状、体征和MRI有特征性改变，^1HMRS的改变也有助于诊断。

Clinical and imaging features of primary lateral sclerosis

Objective To explore the clinical and imaging features of primary lateral sclerosis(PLS).Methods The clinical and imaging data of 3 patients with PLS were analysed.Results 3 patients included 2 females and 1 male.The onset of the disease was insidious.The initial symptoms included rigidity and weakness of right upper limb(case 1),rigidity and weakness of legs(case 2),and pseudobulbar palsy(case 3). As the disease progressed slowly,the patients presented with spastic paralysis of four extremities,reduced muscle strength(3 to 4 degree),clasp-knife like of muscular tension,hyperactivity of tendon reflexes,ankle clonus and presence of Babinski sign.In T2-weighted images of the brain MRI,the abnormal high signals were symmetrically situated in the motor cortex,the posterior limbs of internal capsules,cerebral peduncles and basilar part of pons.1H magnetic resonance spectroscopy(1HMRS) showed decreases of N-acetylasartate(NAA) and the ratio of N-acetylaspartate/Creatine(NAA/Cr) in the focal regions.Conclusion PLS presents with characteristic features of clinical symptoms,signs and abnormal changes of MRI.In addition,1HMRS may be helpful in diagnosis of this disease.