Matsunaga K, Iwamoto M, Tsuji S, Hashimoto T, Murai Y
Department of Neurology, University of Occupational and Environmental Health.
Rinsho Shinkeigaku. 1997 Mar;37(3):233-8.
A 57-year-old man developed severe muscle weakness and atrophy of the upper extremities within a five-month period. Neurological examination revealed severe weakness and atrophy in the scapular muscles and proximal and distal muscles of the upper extremities. Fasciculations were also observed in the various muscles of the upper extremities. There was neither muscle weakness, atrophy nor fasciculation in either his face, neck muscles or lower extremities. He had no pseudobulbar or bulbar signs. Tendon reflexes were mildly hyperactive in the jaw and lower extremities, and normal in the upper extremities. There were no pathological reflexes, spasticity or sensory disturbances. The needle EMG study revealed denervation potentials in all muscles of the upper extremities examined. The nerve conduction study revealed no findings of the conduction block. Cervical spine X-rays revealed the narrowing of the spinal foramens at the left C3/C4 and bilateral C4/C5, C5/C6, and C6/C7 intervertebral levels. In addition, magnetic resonance imaging (MRI) revealed compressions of the cervical cord at C4/C5 and C5/C6 intervertebral levels. These clinical and neuroradiological findings resembled those of the cervical spondylotic amyotrophy (CSA). However, the motor evoked potential (MEP) study revealed the pyramidal tract dysfunction above the levels of the pyramidal decussation. Furthermore, brain MRI revealed abnormal foci in both internal capsules which were characterized by hyperintense relative to cortical gray matter on T2-weighted images and still hyperintense to white matter on proton-density-weighted images. In addition, T2-weighted images demonstrated a low signal within the motor cortex and hyperintense lesions in the white matter of the precentral gyri. These MRI findings indicated the degeneration of the pyramidal tract and corresponded to those found in the patients with amyotrophic lateral sclerosis (ALS) which have been recently reported. It has been difficult to distinguish ALS from CSA. However, MEP and brain MRI studies were useful for distinguishing these two diseases in this patient. In addition, this patient showed typical MRI findings suggesting the degeneration of the pyramidal tract, although this patient had a relatively short course of illness and did not show obvious physical findings suggesting pyramidal tract dysfunction.
一名57岁男性在5个月内出现严重的上肢肌肉无力和萎缩。神经系统检查发现肩胛肌以及上肢近端和远端肌肉严重无力和萎缩。上肢各肌肉还观察到肌束震颤。其面部、颈部肌肉及下肢均无肌肉无力、萎缩或肌束震颤。他没有假性球麻痹或球麻痹体征。下颌和下肢的腱反射轻度亢进，上肢正常。无病理反射、痉挛或感觉障碍。针极肌电图检查显示所检查的上肢所有肌肉均有失神经电位。神经传导研究未发现传导阻滞。颈椎X线片显示左侧C3/C4以及双侧C4/C5、C5/C6和C6/C7椎间水平的椎间孔狭窄。此外，磁共振成像（MRI）显示C4/C5和C5/C6椎间水平的颈髓受压。这些临床和神经放射学表现类似于颈椎病性肌萎缩（CSA）。然而，运动诱发电位（MEP）研究显示锥体交叉水平以上的锥体束功能障碍。此外，脑部MRI显示双侧内囊有异常病灶，在T2加权图像上相对于皮质灰质呈高信号，在质子密度加权图像上相对于白质仍呈高信号。此外，T2加权图像显示运动皮质内有低信号，中央前回白质有高信号病变。这些MRI表现提示锥体束变性，与最近报道的肌萎缩侧索硬化（ALS）患者的表现相符。一直以来，ALS与CSA很难区分。然而，MEP和脑部MRI研究有助于在该患者中区分这两种疾病。此外，该患者显示出提示锥体束变性的典型MRI表现，尽管该患者病程相对较短，且未表现出提示锥体束功能障碍的明显体征。
