Helali Joshua S, Mahindra Anuj, Mohan Rajeev, Hwynn Nelson
Department of Internal Medicine, Scripps Clinic, San Diego, USA.
Department of Hematology and Oncology, Scripps Clinic, San Diego, USA.
Cureus. 2025 Jul 1;17(7):e87116. doi: 10.7759/cureus.87116. eCollection 2025 Jul.
Parkinson's disease and closely related Parkinsonian neurodegenerative diseases are frequently associated with neurogenic orthostatic hypotension. In many cases, the dysautonomia would be attributed to Parkinson's disease or its closely related neurodegenerative mimics. We present our experience diagnosing and managing a patient with dysautonomia attributed to an initial diagnosis of Parkinson's disease and systemic amyloidosis, which the coexistence of these two diseases have not been previously reported in the literature. Our 73-year-old patient developed levodopa-responsive symptoms initially suggestive of Parkinson's disease. Within three years, he developed progressively worsening neurogenic orthostatic hypotension and, eventually, symptomatic bradycardia requiring a pacemaker, and was found to have a condition causing the dysautonomia other than Parkinson's. Further workup led to a concurrent diagnosis of amyloid light-chain amyloidosis, which was successfully treated with chemotherapy. Despite being in remission of the amyloidosis and having satisfactory control of the Parkinson's motor symptoms, his neurogenic orthostatic hypotension continued to be severely disabling, and overall functioning continued to decline. Although neurogenic orthostatic hypotension is common in Parkinson's disease and closely related neurogenerative disorders, there may be clinical features discordant with a neurodegenerative disorder that could lead to an alternative explanation for the dysautonomia. This patient not only fell into the usual Parkinsonism with dysautonomia differential diagnosis but also suggests that Parkinsonian conditions and systemic amyloidosis may exert a synergistic effect on morbidity, explaining why management of dysautonomia in these patients and reducing disability may be a significant challenge.
帕金森病及与之密切相关的帕金森氏神经退行性疾病常伴有神经源性直立性低血压。在许多情况下，自主神经功能障碍可归因于帕金森病或与之密切相关的神经退行性疾病模仿症。我们介绍了我们对一名因最初诊断为帕金森病和系统性淀粉样变性而出现自主神经功能障碍的患者进行诊断和管理的经验，这两种疾病的共存此前在文献中尚未有报道。我们73岁的患者最初出现对左旋多巴有反应的症状，提示为帕金森病。在三年内，他逐渐出现神经源性直立性低血压加重，最终出现需要起搏器治疗的症状性心动过缓，并发现存在除帕金森病之外导致自主神经功能障碍的病症。进一步检查导致同时诊断出轻链淀粉样变性，经化疗成功治疗。尽管淀粉样变性已缓解且帕金森病运动症状得到满意控制，但他的神经源性直立性低血压仍然严重致残，整体功能持续下降。虽然神经源性直立性低血压在帕金森病及与之密切相关的神经退行性疾病中很常见，但可能存在与神经退行性疾病不一致的临床特征，从而导致对自主神经功能障碍的另一种解释。这名患者不仅陷入了伴有自主神经功能障碍的常见帕金森综合征鉴别诊断中，还提示帕金森病情况和系统性淀粉样变性可能对发病率产生协同作用，这解释了为何对这些患者的自主神经功能障碍进行管理和减少残疾可能是一项重大挑战。
