目的阐明汉森病的神经系统特征。回顾了从神经内科转诊的确诊汉森病患者的病历,并对Hansen病的所有医学和神经系统表现进行了评估。11例确诊汉森病患者,10例新发现的汉森病和1例复发的汉森病,谁访问了神经内科登记。新发现的汉森病患者被归类为麻风病(LL,n=1),边缘麻风病(BL,n=2),临界麻风病(BB,n=2),临界型结核性麻风病(BT,n=1),结核性麻风病(TT,n=2),或纯神经性麻风病(PNL,n=2)。所有确诊为Hansen的患者均诊断为周围神经病变(100.00%,11/11).症状和体征主要表现为肢体麻木(100.00%,11/11),感觉和运动功能障碍(100.00%,11/11),肌肉力量下降(90.90%,10/11),和皮肤损伤(81.81%,9/11).神经超声检查(US)的神经形态特征包括周围神经不对称和节段性增厚(100.00%,9/9)。对于神经电生理学特征,感觉神经无反应的频率明显高于运动神经[(51.21%42/82)vs(24.70%,21/85)(P=0.0183*)]通过电诊断(EDX)研究。神经活检分析的神经组织学特征包括脱髓鞘(100.00%,5/5)和轴突损伤(60.00%,3/5)。除了通过抗酸细菌(AFB)染色确认诊断外(54.54%,6/11)和皮肤病理分析(100.00%,8/8),36.36%(4/11)和100.00%(11/11)的Hansen病确诊患者血清学和分子技术检测阳性,分别。由于周围神经病变,汉森病患者去神经科就诊并不少见。受累神经的主要病理特征是脱髓鞘和轴突损害。神经的结合,EDX研究,神经活检,血清学和分子检测可以提高汉森病的诊断水平。
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.