Leprosy Department, Beijing Tropical Medicine Research Institute, Beijing Friendship Hospital, Capital Medical University, Beijing, China.
Beijing Key Laboratory for Research On Prevention and Treatment of Tropical Diseases, Capital Medical University, Beijing, China.
Sci Rep. 2024 May 6;14(1):10374. doi: 10.1038/s41598-024-60457-0.
To elucidate the neurological features of Hansen disease. The medical records of patients with confirmed Hansen disease transferred from the neurology department were reviewed, and all medical and neurological manifestations of Hansen disease were assessed. Eleven patients with confirmed Hansen disease, 10 with newly detected Hansen disease and 1 with relapsed Hansen disease, who visited neurology departments were enrolled. The newly detected patients with Hansen disease were classified as having lepromatous leprosy (LL, n = 1), borderline lepromatous leprosy (BL, n = 2), borderline leprosy (BB, n = 2), borderline tuberculoid leprosy (BT, n = 1), tuberculoid leprosy (TT, n = 2), or pure neural leprosy (PNL, n = 2). All of the patients with confirmed Hansen were diagnosed with peripheral neuropathy (100.00%, 11/11). The symptoms and signs presented were mainly limb numbness (100.00%, 11/11), sensory and motor dysfunction (100.00%, 11/11), decreased muscle strength (90.90%, 10/11), and skin lesions (81.81%, 9/11). Nerve morphological features in nerve ultrasonography (US) included peripheral nerve asymmetry and segmental thickening (100.00%, 9/9). For neuro-electrophysiology feature, the frequency of no response of sensory nerves was significantly higher than those of motor nerves [(51.21% 42/82) vs (24.70%, 21/85)(P = 0.0183*)] by electrodiagnostic (EDX) studies. Nerve histological features in nerve biopsy analysis included demyelination (100.00%, 5/5) and axonal damage (60.00%, 3/5). In addition to confirmed diagnoses by acid-fast bacteria (AFB) staining (54.54%, 6/11) and skin pathology analysis (100.00%, 8/8), serology and molecular technology were positive in 36.36% (4/11) and 100.00% (11/11) of confirmed patients of Hansen disease, respectively. It is not uncommon for patients of Hansen disease to visit neurology departments due to peripheral neuropathy. The main pathological features of affected nerves are demyelination and axonal damage. The combination of nerve US, EDX studies, nerve biopsy, and serological and molecular tests can improve the diagnosis of Hansen disease.
为阐明麻风病的神经特征。我们回顾了从神经科转来的确诊麻风病患者的病历,并评估了所有麻风病的医学和神经表现。共纳入 11 例确诊麻风病患者,其中 10 例为新发现的麻风病患者,1 例为复发病例,这些患者均因神经科相关症状就诊。新发现的麻风病患者被分为瘤型麻风(LL,n=1)、界限型偏瘤型麻风(BL,n=2)、界限型麻风(BB,n=2)、界限型结核样型麻风(BT,n=1)、结核样型麻风(TT,n=2)和单纯神经炎麻风(PNL,n=2)。所有确诊的麻风病患者均被诊断为周围神经病(100.00%,11/11)。主要表现为四肢麻木(100.00%,11/11)、感觉和运动功能障碍(100.00%,11/11)、肌力下降(90.90%,10/11)和皮肤损伤(81.81%,9/11)。神经超声检查(US)显示神经形态学特征包括周围神经不对称和节段性增厚(100.00%,9/9)。神经电生理特征方面,感觉神经无反应的频率明显高于运动神经无反应的频率[(51.21%(42/82))比(24.70%(21/85)),P=0.0183*]。神经活检分析的神经组织学特征包括脱髓鞘(100.00%,5/5)和轴索损伤(60.00%,3/5)。除了酸染色(AFB)(54.54%,6/11)和皮肤病理分析(100.00%,8/8)确诊外,血清学和分子技术分别在 36.36%(4/11)和 100.00%(11/11)的确诊麻风病患者中呈阳性。麻风病患者因周围神经病就诊于神经科并不罕见。受累神经的主要病理特征是脱髓鞘和轴索损伤。神经超声、神经电生理研究、神经活检、血清学和分子检测的联合应用可以提高麻风病的诊断。