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Objective: To clarify the progression of autonomic symptoms and functional de terioration in pure autonomic failure (PAF), particularly in comparison with mul tiple system atrophy (MSA). Methods: The investigation involved eight patients w ith PAF (M/F=7/1; mean age at onset, 57 years) and 22 with probable MSA matched for age at onset (M/F=14/8; onset 56 years). Subjects were followed up for neuro logical symptoms, activities of daily living, and autonomic function for more th an seven years. Autonomic functional tests were carried out. Results: In PAF, fa inting or sudomotor dysfunction occurred first, followed by constipation and syn cope. Urinary dysfunction developed late, and respiratory dysfunction was not ev ident. This clinical course contrasted sharply with that in MSA, where early uri nary dysfunction usually proceeded to sudomotor dysfunction or orthostatic hypot ension (p=0.004), followed by respiratory dysfunction (p=0.0004). Results of pha rmacological tests also distinguished PAF from MSA. Progression and prognosis in patients with PAF did not worsen, unlike the steady progressive autonomic dysfu nction in MSA (p < 0.0001, p < 0.0001, p=0.0009, and p=0.003, for progression to modified Rankin scale grade III, IV, V, and death, respectively). Conclusions: The time course and pattern of progression of autonomic failure differed signifi cantly between PAF and MSA. Patients with PAF had slower functional deterioratio n and a better prognosis.
Objective: To clarify the progression of autonomic symptoms and functional deiioration in pure autonomic failure (PAF), particularly in comparison with mul tiple system atrophy (MSA). Methods: The investigation involved eight patients w ith PAF (M / F = 1; mean age at onset, 57 years) and 22 with probable MSA matched for age at onset (M / F = 14/8; onset 56 years). Subjects were followed up for neuro logical symptoms, activities of daily living, and autonomic Results: In PAF, fa inting or sudomotor dysfunction occurred first, followed by constipation and syn cope. Urinary dysfunction of late, and respiratory dysfunction was not ev ident. This clinical course contrasted sharply with that in MSA, where early uri nary dysfunction usually proceeded to sudomotor dysfunction or orthostatic hypotension (p = 0.004), followed by respiratory dysfunction (p = 0.0004). Results of pha rmacological tests also distingu ished PAF from MSA. Progression and prognosis in patients with PAF did not worsen, unlike the steady progressive autonomic dysfuction in MSA (p <0.0001, p <0.0001, p = 0.0009, and p = 0.003 for progression to modified Rankin scale grade III, IV, V, and death, respectively). Conclusions: The time course and pattern of progression of autonomic failure differed signifi cantly between PAF and MSA. Patients with PAF had slower functional deterioratio n and a better prognosis.