Hui Monalisa, Uppin Megha S, Challa Sundaram, Meena A K, Kaul Subhash
Department of Pathology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India.
Department of Neurology, Nizam's Institute of Medical Sciences, Punjagutta, Hyderabad, Telangana, India.
Ann Indian Acad Neurol. 2015 Jul-Sep;18(3):292-7. doi: 10.4103/0972-2327.162284.
Demonstration of lepra bacilli is essential for definite or unequivocal diagnosis of pure neuritic leprosy (PNL) on nerve biopsy. However, nerves always do not show bacilli owing to the changes of previous therapy or due to low bacillary load in tuberculoid forms. In absence of granuloma or lepra bacilli, other morphologic changes in endoneurium and perineurium can be of help in making a probable diagnosis of PNL and treating the patient with multidrug therapy.
Forty-six biopsies of PNL were retrospectively reviewed and histologic findings were compared with 25 biopsies of non leprosy neuropathies (NLN) including vasculitic neuropathy and chronic inflammatory demyelinating polyneuropathy (CIDP). The distribution of endoneurial infiltrate and fibrosis, perineurial thickening, and myelin abnormalities were compared between PNL and NLN biopsies and analyzed by Chi-square test.
Out of 46 PNL casses, 24 (52.17 %) biopsies were negative for acid fast bacilli (AFB). In these cases, the features which favor a diagnosis of AFB-negative PNL were endoneurial infiltrate (51.1%), endoneurial fibrosis (54.2%), perineurial thickening (70.8%), and reduced number of myelinated nerve fibers (75%).
Nerve biopsy is an efficient tool to diagnose PNL and differentiate it from other causes of NLN. In absence of AFB, the diagnosis of PNL is challenging. In this article, we have satisfactorily evaluated the various hisopthological features and found that endoneurial inflammation, dense fibrosis, and reduction in the number of myelinated nerve fibers are strong supportive indicators of PNL regardless of AFB positivity.
在神经活检中，麻风杆菌的显示对于明确诊断纯神经炎型麻风（PNL）至关重要。然而，由于既往治疗的影响或结核样型中细菌载量较低，神经活检时并不总能发现杆菌。在没有肉芽肿或麻风杆菌的情况下，神经内膜和神经束膜的其他形态学改变有助于对PNL作出可能的诊断，并采用多药疗法治疗患者。
回顾性分析46例PNL患者的活检标本，并将组织学结果与25例非麻风性神经病（NLN）（包括血管炎性神经病和慢性炎症性脱髓鞘性多发性神经病（CIDP））的活检标本进行比较。比较PNL和NLN活检标本中神经内膜浸润和纤维化的分布、神经束膜增厚及髓鞘异常情况，并采用卡方检验进行分析。
在46例PNL病例中，24例（52.17%）活检标本抗酸杆菌（AFB）阴性。在这些病例中，支持AFB阴性PNL诊断的特征为神经内膜浸润（51.1%）、神经内膜纤维化（54.2%）、神经束膜增厚（70.8%）和有髓神经纤维数量减少（75%）。
神经活检是诊断PNL并将其与NLN的其他病因相鉴别的有效工具。在没有AFB的情况下，PNL的诊断具有挑战性。在本文中，我们对各种组织病理学特征进行了满意的评估，发现无论AFB是否阳性，神经内膜炎症、致密纤维化和有髓神经纤维数量减少都是PNL的有力支持指标。
