Objective: To report the long-term follow-up (10 years) of a patient with severe autonomic dysfunction as presenting symptom of Parkinson’s disease (PD), analyzing the possible differential diagnosis with pure autonomic failure (PAF) and multisystem atrophy (MSA).

Background: Autonomic failure can be a feature of PD and other α-synucleinopathies. However, the extent of central and peripheral norepinephrine deficiency, parasympathetic nuclei degeneration, and arterial baroreflex failure may be differentially impaired in these disorders.

Methods: We report the long-term follow-up of a 68-year-old man admitted to the emergency unit for syncope associated with orthostatism (blood pressure supine: 148/98 mmHg; upright: 80/50 mmHg). Over the prior 12 months he had reported three syncopal events, constipation, erectile dysfunction, and profuse sweating.

Results: The autonomic evaluation showed a “reverse dipping” pattern on ambulatory blood pressure monitoring, decreased heart rate variability and markedly low catecholamine plasma levels in both supine and orthostatic positions. The 123MIBG scan demonstrated widespread sympathetic denervation. He was managed with midodrine 15 mg/day, and fludrocortisone 0.1 mg/day. Three years later, he developed resting tremor in the right limbs, associated with bradykinesia. Brain DAT-SCAN was altered and a diagnosis of PD was made. A treatment with rasagiline (1 mg/day) was started, lowering the Unified Parkinson’s disease Rating Scale (UPDRS) part III from 11/108 to 6/108. Four years later, upon worsening of tremor and bradykinesia (UPDRS-III, 14/108); low-dose levodopa/carbidopa (50/12.5 t.i.d.) was administered, followed by significant motor improvement (UPDRS-III, 7/108). However, OH worsened, requiring withholding levodopa. After more than 10 years from the onset of autonomic features, dysautonomia with prominent orthostatism remained a levodopa-limiting major source of disability.

Conclusions: The early differential diagnosis of α-synucleinopathies may be challenging, despite the significantly different prognosis (10-year survival rate of 33% in MSA, 87% in PAF, 74% in PD+OH, and 93% in PD without OH) and the heterogeneous pathogenic mechanisms involved. In this case the clinical features initially suggested MSA or PAF, but the abnormal 123MIBG scan and the low orthostatic catecholamine plasma levels argued against a diagnosis of MSA, and the profuse sweating was atypical for PAF.

« Back to 2016 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/parkinsons-disease-prodromal-dysautonomia-versus-pure-autonomic-failure-and-msa/