一项关于神经肌肉疾病组织病理学模式及其临床病理相关性的研究。

Karim M S, Kamal M, Rahman A J E N

Dept. of Pathology, Dhaka Medical College, Dhaka.

Bangladesh Med Res Counc Bull. 2003 Apr;29(1):11-22.

Neuromuscular disorders are frequently seen in our clinical practice, though no certain data are available in this regard. This prospective, observational study which was done as a partial fulfillment of M.Phil (Pathology) degree in BSMMU. The study was undertaken at the Department of Pathology of Bangabandhu Shiekh Mujib Medical University from August 1999 to December 2000 to study the histomorphological pattern of neuromuscular disorders in a selected group of patients attending the neuromedicine, paediatrics and medicine departments of BSMMU. Another purpose of this study was to evaluate the diagnostic utility of muscle biopsy and clinico-pathologic correlation in the diagnosis of neuromuscular disorders. In this study, 55 cases of clinically diagnosed neuromuscular disorders of different kinds were included. Detail clinical history was obtained in all the cases. Clinical diagnosis were made on the basis of history, physical examination, reports of routine and special laboratory tests whenever available. Muscle biopsy was performed in all the cases and histological changes could be identified in 42 cases. In the remaining thirteen undiagnosed cases; eight cases revealed 'essentially normal muscle tissue' and five cases were inadequate for histological evaluation. So, the later two categories were not included for further analysis. Among the histologically diagnosed 42 cases of neuromuscular disorders, basically two different classes of diseases were identified; 1) Dystrophic type of muscular diseases 64.28% and 2) Non dystrophic (acquired) type of neuromuscular diseases such as Inflammatory myopathy 21.82% and Neurogenic muscular atrophy 7.14%. Within the dystrophic group the maximum number of diseases were diagnosed as Duchenne muscular dystrophy 21.49%, Primary myopathy-unclassified 19.04%, Baker muscular dystrophy 4.76% and Limb girdle muscular dystrophy 4.76% etc. Properly executed muscle biopsy is usually the most useful and effective technique for diagnosis of neuromuscular diseases in cases where immunohistochemical, genetic and electron microscopic examination is not possible.

在我们的临床实践中，神经肌肉疾病很常见，不过目前尚无这方面的确切数据。本前瞻性观察性研究是作为孟加拉国谢赫穆吉布医学大学哲学硕士（病理学）学位的部分研究内容开展的。该研究于1999年8月至2000年12月在孟加拉国谢赫穆吉布医学大学病理学系进行，旨在研究孟加拉国谢赫穆吉布医学大学神经医学、儿科学和医学科部分选定患者群体中神经肌肉疾病的组织形态学模式。本研究的另一个目的是评估肌肉活检在神经肌肉疾病诊断中的诊断效用以及临床病理相关性。本研究纳入了55例临床诊断为不同类型神经肌肉疾病的病例。所有病例均获取了详细的临床病史。临床诊断基于病史、体格检查以及常规和特殊实验室检查报告（如有）。所有病例均进行了肌肉活检，42例可识别出组织学变化。在其余13例未确诊病例中，8例显示“肌肉组织基本正常”，5例因组织学评估不足而未纳入。因此，后两类未纳入进一步分析。在组织学诊断的42例神经肌肉疾病病例中，基本确定了两种不同类型的疾病：1）营养不良型肌肉疾病，占64.28%；2）非营养不良（后天性）型神经肌肉疾病，如炎性肌病，占21.82%，神经源性肌肉萎缩，占7.14%。在营养不良组中，诊断出疾病数量最多的是杜氏肌营养不良，占21.49%，原发性肌病（未分类），占19.04%，贝克肌营养不良，占4.76%，肢带型肌营养不良，占4.76%等。在无法进行免疫组织化学、基因和电子显微镜检查的情况下，正确实施的肌肉活检通常是诊断神经肌肉疾病最有用和有效的技术。