Weis J, Nikolin S, Nolte K
Institut für Neuropathologie, Universitätsklinikum der RWTH, Aachen, Deutschland.
Pathologe. 2009 Sep;30(5):379-83. doi: 10.1007/s00292-009-1171-y.
Neurogenic muscular atrophy (NMA) is the most frequent diagnosis obtained from reading a muscle biopsy. It is characterized by specific histological changes which distinguish NMA from other important muscle pathologies including the primary myopathies such as the muscular dystrophies as well as the inflammatory muscle disorders. Within the group of denervation atrophies, NMAs due to motor neuron diseases are associated with particular histological patterns. The diagnosis of NMA in muscle biopsies requires special methods, mainly enzyme and immunohistochemistry, but also resin histology and in some cases electron microscopy. Analysis of a combined muscle and sural nerve biopsy provides the opportunity to compare the extent of degeneration in the motor and sensory systems, respectively. Muscle fiber typing by enzyme and immunohistochemistry also leads to the detection of selective type 1 and type 2 muscle fiber atrophies which are relevant in the differential diagnosis of neuromuscular diseases.
神经源性肌肉萎缩（NMA）是肌肉活检报告中最常见的诊断结果。其特征在于特定的组织学变化，这些变化可将NMA与其他重要的肌肉病变区分开来，包括原发性肌病（如肌肉营养不良症）以及炎性肌肉疾病。在去神经萎缩组中，由运动神经元疾病引起的NMA与特定的组织学模式相关。肌肉活检中NMA的诊断需要特殊方法，主要是酶学和免疫组织化学方法，还有树脂组织学方法，在某些情况下还需要电子显微镜检查。对肌肉和腓肠神经联合活检进行分析，分别为比较运动和感觉系统的变性程度提供了机会。通过酶学和免疫组织化学进行肌纤维分型，还能检测出1型和2型肌纤维的选择性萎缩，这在神经肌肉疾病的鉴别诊断中具有重要意义。
