Gastaut J L, Michel B, Figarella-Branger D, Somma-Mauvais H
Service de Neurologie, Hôpital Ste Marguerite, Marseilles, France.
Arch Neurol. 1988 May;45(5):509-13. doi: 10.1001/archneur.1988.00520290037011.
Although it was first described over a century ago (by Charcot in 1865; by Erb in 1875), the concept of primary lateral sclerosis (PLS) is still not universally accepted. Despite this skepticism, several well-documented cases of isolated degeneration with varying degrees of involvement of corticospinal pyramidal pathways have been reported in the literature. The clinical manifestations in these cases can take one of two forms, ie, isolated spasmodic paraplegia or tetraplegia on the one hand or spasmodic tetraplegia associated with a pseudobulbar syndrome featuring severe spastic dysarthria (chronic progressive bilateral spinobulbar spasticity) on the other hand. Obviously, without firm pathologic data, PLS is a hazardous diagnosis for isolated paraplegia or tetraplegia. Conversely, for bilateral spinobulbar spasticity, it would appear to be the only diagnosis possible once investigate findings have eliminated the other possibilities, such as a pyramidal form of amyotrophic lateral sclerosis or a spinal form of multiple sclerosis. To underscore this point, in this report, five cases of chronic progressive bilateral spinobulbar spasticity developed over 5, 10, 12, 10, and 28 years, respectively, for which the only possible diagnosis was PLS. It was concluded that there are three forms of degenerative diseases of the principal motor pathways: one involving both central and peripheral neurons, ie, amyotrophic lateral sclerosis; one involving only peripheral neurons, ie, spinal amyotrophy; and one involving only central motor neurons, ie, PLS.
尽管原发性侧索硬化症（PLS）的概念早在一个多世纪前就已被描述（1865年由夏科特描述；1875年由埃尔布描述），但仍未被普遍接受。尽管存在这种怀疑态度，但文献中已报道了几例记录详实的、伴有不同程度皮质脊髓锥体束受累的孤立性变性病例。这些病例的临床表现有两种形式，一方面是孤立性痉挛性截瘫或四肢瘫，另一方面是伴有严重痉挛性构音障碍（慢性进行性双侧脊髓延髓痉挛）的假性延髓综合征的痉挛性四肢瘫。显然，没有确凿的病理数据，对于孤立性截瘫或四肢瘫，PLS是一个危险的诊断。相反，对于双侧脊髓延髓痉挛，一旦检查结果排除了其他可能性，如肌萎缩侧索硬化症的锥体形式或多发性硬化症的脊髓形式，PLS似乎是唯一可能的诊断。为强调这一点，在本报告中，分别有5例慢性进行性双侧脊髓延髓痉挛病例，病程分别为5年、10年、12年、10年和28年，唯一可能的诊断是PLS。得出的结论是，主要运动通路的退行性疾病有三种形式：一种累及中枢和外周神经元，即肌萎缩侧索硬化症；一种仅累及外周神经元，即脊髓性肌萎缩症；还有一种仅累及中枢运动神经元，即PLS。
