Machner B, Gottschalk S, Kimmig H, Helmchen C
Klinik für Neurologie, Universitätsklinikum Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, Lübeck, Germany.
Nervenarzt. 2007 Dec;78(12):1440-3. doi: 10.1007/s00115-007-2340-y.
The concurrence of amyotrophic lateral sclerosis (ALS) and multiple sclerosis (MS) is extremely rare. We report on a 55-year-old female patient presenting with a progressive gait disorder for 6 months and a speech disorder for 3 months. Neurological examination revealed a spastic paraparesis and mild dysarthria and dysphagia. Technical and laboratory investigations met the diagnostic criteria for MS: magnetic resonance imaging showed multiple periventricular white matter and cervical lesions; cerebrospinal fluid showed a typical autoimmune response. Within the following 3 months generalized fasciculations, atrophy of the small hand muscles and bulbar signs were noticed. Nerve conduction studies revealed acute and chronic signs of denervation in all limbs without nerve conduction block. Hence clinical and paraclinical examination met the El Escorial criteria for ALS. Although myelitic lesions in the anterior horn cells may lead to peripheral segmental denervation, the generalized denervation suggested the unusual coincident combination of ALS and MS in this patient. In clinical praxis motoneuron diseases should also be considered in patients with pronounced peripheral denervations once "definite" MS has been diagnosed.
肌萎缩侧索硬化症(ALS)与多发性硬化症(MS)同时出现极为罕见。我们报告了一名55岁女性患者,她出现进行性步态障碍6个月,言语障碍3个月。神经系统检查发现痉挛性截瘫、轻度构音障碍和吞咽困难。技术和实验室检查符合MS的诊断标准:磁共振成像显示多个脑室周围白质和颈部病变;脑脊液显示典型的自身免疫反应。在接下来的3个月内,发现全身肌肉束颤、手部小肌肉萎缩和延髓体征。神经传导研究显示所有肢体均有急性和慢性失神经征象,无神经传导阻滞。因此,临床和辅助检查符合ALS的埃尔埃斯科里亚尔标准。虽然前角细胞的脊髓炎病变可能导致周围节段性失神经,但全身失神经提示该患者存在ALS与MS不寻常的同时并发情况。在临床实践中,一旦诊断为“明确的”MS,对于有明显周围神经失神经的患者也应考虑运动神经元疾病。