Howell D A, Lees A J, Toghill P J
J Neurol Neurosurg Psychiatry. 1979 Sep;42(9):773-85. doi: 10.1136/jnnp.42.9.773.
The clinical and pathological features of a fourth patient with progressive encephalomyelitis with rigidity are reported and compared with those previously described. It is suggested that the muscular rigidity, abnormal postures, painful muscular spasms, and myoclonus are a product of excessive and abnormal discharges of alpha motor neurones caused by their release from control by spinal internuncial neurones. A count of neuronal perikarya in the ventral horns confirmed that the disease selectively destroyed small and medium sized neurones, most of which were spinal internuncial neurones. Experimental, clinical, and pathological evidence concerning spinal internuncial neurones is reviewed and found to conform to this theory. The pathogenesis of opsoclonus may be similar.
报告了第四例进行性脑脊髓炎伴强直患者的临床和病理特征，并与先前描述的特征进行了比较。研究表明，肌肉强直、异常姿势、疼痛性肌肉痉挛和肌阵挛是α运动神经元从脊髓中间神经元的控制中释放出来后，过度且异常放电的产物。对腹角神经元胞体的计数证实，该疾病选择性地破坏了中小型神经元，其中大多数是脊髓中间神经元。对有关脊髓中间神经元的实验、临床和病理证据进行了综述，发现其与该理论相符。眼球阵挛的发病机制可能与之相似。
