Key result
Despite successful remission of AL amyloidosis and adequate control of Parkinsonian motor symptoms, the patient's severe neurogenic orthostatic hypotension continued to progress and cause disability.
Why the study?
The coexistence of Parkinson's disease and systemic amyloidosis causing dysautonomia had not been previously reported in the literature.
Case Report (n=1)
No
The concurrent presence of Parkinson's disease and systemic AL amyloidosis may exert a synergistic pathophysiologic effect on the autonomic nervous system, making the management of neurogenic orthostatic hypotension highly challenging even after amyloidosis remission.
nOH may remain disabling after amyloid remission with coexistent PD; leaves open synergistic autonomic injury and should not yet change practice.
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.
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Helali et al. (2025) conducted a case report in Parkinson's disease and systemic AL amyloidosis (n=1). Chemotherapy and dysautonomia management was evaluated on Clinical course of dysautonomia. Despite successful remission of AL amyloidosis and adequate control of Parkinsonian motor symptoms, the patient's severe neurogenic orthostatic hypotension continued to progress and cause disability.
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