Division of Neuromuscular Disorders, Department of Neurology, Ribeirão Preto School of Medicine, University of São Paulo, Ribeirão Preto 14048-900, Brazil.
Department of Internal Medicine, National Reference Centre for Sanitary Dermatology and Leprosy, Clinical Hospital, Federal University of Uberlândia (CREDESH/HC/UFU), Uberlândia 38400-902, Brazil.
Brain. 2022 May 24;145(4):1499-1506. doi: 10.1093/brain/awab396.
Disability in leprosy is a direct consequence of damage to the peripheral nervous system which is usually worse in patients with no skin manifestations, an underdiagnosed subtype of leprosy known as primary neural leprosy. We evaluated clinical, neurophysiological and laboratory findings of 164 patients with definite and probable primary neural leprosy diagnoses. To better understand the disease progression and to improve primary neural leprosy clinical recognition we compared the characteristics of patients with short (≤12 months) and long (>12 months) disease duration. Positive and negative symptoms mediated by small-fibres were frequent at presentation (∼95%), and symptoms tend to manifest first in the upper limbs (∼68%). There is a consistent phenotypic variability between the aforementioned groups. Deep sensory modalities were spared in patients evaluated within the first 12 months of the disease, and were only affected in patients with longer disease duration (∼12%). Deep tendon reflexes abnormalities were most frequent in patients with longer disease duration (P < 0.001), as well as motor deficits (P = 0.002). Damage to large fibres (sensory and motor) is a latter event in primary neural leprosy. Grade-2 disability and nerve thickening was also more frequent in cases with long disease duration (P < 0.001). Primary neural leprosy progresses over time and there is a marked difference in clinical phenotype between patients with short and long disease duration. Patients assessed within the first 12 months of symptom onset had a non-length-dependent predominant small-fibre sensory neuropathy, whilst patients with chronic disease presented an asymmetrical all diameter sensory-motor neuropathy and patchily decreased/absent deep tendon reflexes.
麻风病导致的残疾是外周神经系统损伤的直接后果，在没有皮肤表现的患者中更为严重，这些患者属于一种未被充分诊断的麻风病亚型，即原发性神经麻风病。我们评估了 164 例明确和可能的原发性神经麻风病患者的临床、神经生理学和实验室检查结果。为了更好地了解疾病的进展并提高对原发性神经麻风病的临床认识，我们比较了病程短（≤12 个月）和长（>12 个月）的患者的特征。小纤维介导的阳性和阴性症状在发病时很常见（约 95%），且症状往往首先出现在上肢（约 68%）。在上述各组之间存在一致的表型变异性。在疾病发病的头 12 个月内接受评估的患者中，深部感觉模式保留完好，而病程较长（>12 个月）的患者则仅受影响（约 12%）。在病程较长的患者中，深腱反射异常更为常见（P<0.001），运动缺陷也更为常见（P=0.002）。大纤维（感觉和运动）的损伤是原发性神经麻风病的后期事件。在病程较长的患者中，2 级残疾和神经增厚也更为常见（P<0.001）。原发性神经麻风病会随着时间的推移而进展，且病程短和长的患者的临床表型存在显著差异。在症状出现的头 12 个月内接受评估的患者表现为非长度依赖性的主要小纤维感觉神经病，而慢性疾病患者则表现为不对称的全直径感觉运动神经病，且深部腱反射减弱/消失呈斑片状。
