Sarnat H B
Can J Neurol Sci. 1985 May;12(2):111-20.
The importance of suprasegmental cerebral influence on developing human fetal muscle is less well understood than the control of histochemical differentiation of muscle by the motor neuron. Muscle biopsies of 21 hypotonic infants and children with nonprogressive congenital lesions of the cerebellum and/or brainstem were studied by histochemical methods. Two neonates who died with severely dysplastic brains and no descending motor tracts had normal muscle. The others, particularly those with cerebellar hypoplasia, had delayed muscle maturation, selective predominance of type I or II muscle fibres or disproportion in fibre sizes. It is concluded that the motor unit is capable of developing normally without suprasegmental influence, but that an abnormal balance of descending impulses may alter histochemical differentiation of fetal muscle. The small 'subcorticospinal' pathways arising in the brainstem probably are more influential than the larger corticospinal tract because of later myelination in the latter. The muscle biopsy thus serves to provide evidence of suprasegmental disease in infantile hypotonia.
大脑皮质上部分对人类胎儿肌肉发育的影响,其受理解程度低于运动神经元对肌肉组织化学分化的控制。采用组织化学方法,对21例患有小脑和/或脑干非进行性先天性病变的低张力婴幼儿进行了肌肉活检。两名患有严重发育不良大脑且无下行运动束的新生儿肌肉正常。其他患儿,尤其是小脑发育不全的患儿,出现肌肉成熟延迟、I型或II型肌纤维选择性优势或纤维大小不均衡的情况。结论是,运动单位在没有皮质上部分影响的情况下能够正常发育,但下行冲动的异常平衡可能会改变胎儿肌肉的组织化学分化。由于皮质脊髓束髓鞘形成较晚,脑干中产生的小“皮质下脊髓”通路可能比大的皮质脊髓束更具影响力。因此,肌肉活检有助于为婴儿型肌张力低下的皮质上部分疾病提供证据。