Rozhold O, Vojácek K
Institute of Clinical and Experimental Neurology, Palacký University, Olomouc.
Acta Univ Palacki Olomuc Fac Med. 1991;130:163-7.
Our file comprises bioptic and necroptic materials of skeletal muscles: progressive spinal muscle atrophy of adults 16, amyotrophic lateral sclerosis 12, infantile progressive spinal amyotrophy Werdnig-Hoffmann 9, chronic progressive amyotrophy Oppenheim 6, syringomyelia and syringobulbia 3, conditions after spinal cord accidents. Diagnosis was difficult because one time the neurological marks were at the head and other time the muscular ones. In the clinical picture there was muscle weakness and atrophy, the spontaneous and palpation painfulness was missing. Muscle weakness und its intensity did not correspond to the degree of muscle atrophies. The discrepancies of electromyographic and histopathologic pictures are explained by occurrence of so-called concomitant myopathies, which arise most probably from the disorders of histioceptive-proprioceptive system.
16例成人进行性脊髓性肌萎缩症、12例肌萎缩侧索硬化症、9例婴儿进行性脊髓性肌萎缩症(韦尼克 - 霍夫曼病)、6例慢性进行性肌萎缩症(奥本海姆病)、3例脊髓空洞症和延髓空洞症、脊髓损伤后的情况。诊断困难,因为有时神经学体征位于头部,有时则是肌肉体征。临床表现为肌肉无力和萎缩,无自发痛和触压痛。肌肉无力及其强度与肌肉萎缩程度不相符。肌电图和组织病理学图像的差异可通过所谓的伴随性肌病的发生来解释,这种肌病很可能源于组织感受性 - 本体感受系统的紊乱。