PDF(Pubmed)
Abstract：
Background and Objectives: Sensory ganglionopathy is a rare neurological disorder caused by degeneration of the neurons composing the dorsal root ganglia. It manifests as various sensory disturbances in the trunk, proximal limbs, face, or mouth in a patchy and asymmetrical pattern. Harlequin syndrome is characterized by unilateral flushing and sweating of the face, neck, and upper chest, concurrent with contralateral anhidrosis. Here, we present and discuss a clinical case of sarcoidosis-associated ganglionopathy and Harlequin syndrome. Case presentation: A 31-year-old woman complained of burning pain in the right side of the upper chest and the feet. She also experienced episodes of intense flushing and sweating on the right side of her face, neck, and upper chest. Three years before these symptoms began, the patient was diagnosed with pulmonary sarcoidosis. On neurological examination, sensory disturbances were present. In the trunk, the patient reported pronounced hyperalgesia and allodynia in the upper part of the right chest and some patches on the right side of the upper back. In the extremities, hypoalgesia in the tips of the fingers and hyperalgesia in the feet were noted. An extensive diagnostic workup was performed to eliminate other possible causes of these disorders. A broad range of possible metabolic, immunological, and structural causes were ruled out. Thus, the final clinical diagnosis of sarcoidosis-induced sensory ganglionopathy, small-fiber neuropathy, and Harlequin syndrome was made. Initially, the patient was treated with pregabalin and amitriptyline, but the effect was inadequate for the ganglionopathy-induced pain. Therefore, therapeutic plasma exchange as an immune-modulating treatment was selected, leading to partial pain relief. Conclusions: This case report demonstrates the possible autoimmune origin of both sensory ganglionopathy and Harlequin syndrome. It suggests that an autoimmune etiology for these disorders should be considered and the diagnostic workup should include screening for the most common autoimmune conditions.
摘要：
背景与目的：感觉神经节病是由构成背根神经节的神经元变性引起的一种罕见的神经系统疾病。它表现为躯干的各种感觉障碍，近端肢体,脸,或嘴巴呈斑驳和不对称的图案。丑角综合征的特征是面部单侧潮红和出汗，脖子,和上胸部，同时伴有对侧无汗症。这里,我们介绍并讨论了结节病相关神经节病和丑角综合征的临床病例。病例介绍：一名31岁的妇女抱怨上胸部右侧和脚的灼痛。她还经历了脸部右侧强烈的潮红和出汗的发作，脖子,和上胸部。这些症状开始前三年，患者被诊断为肺结节病。在神经系统检查中，存在感觉障碍。在后备箱里,患者报告右胸部上部明显的痛觉过敏和异常性疼痛，右侧上背部有一些补片。在四肢，注意到指尖的痛觉减退和脚的痛觉过敏。进行了广泛的诊断检查以消除这些疾病的其他可能原因。广泛的可能代谢，免疫学，排除了结构性原因。因此，结节病引起的感觉神经节病变的最终临床诊断，小纤维神经病,制造了丑角综合症。最初，患者接受普瑞巴林和阿米替林治疗，但对于神经节病引起的疼痛效果不足。因此,选择治疗性血浆置换作为免疫调节治疗，导致部分疼痛缓解。结论：该病例报告证明了感觉神经节病变和丑角综合征的可能自身免疫起源。这表明应考虑这些疾病的自身免疫性病因，诊断检查应包括筛查最常见的自身免疫性疾病。
