Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy.
OBJECTIVE
To clarify the progression of autonomic symptoms and functional deterioration in pure autonomic failure (PAF), particularly in comparison with multiple system atrophy (MSA).


METHODS
The investigation involved eight patients with 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 neurological symptoms, activities of daily living, and autonomic function for more than seven years. Autonomic functional tests were carried out.


RESULTS
In PAF, fainting or sudomotor dysfunction occurred first, followed by constipation and syncope. Urinary dysfunction developed late, and respiratory dysfunction was not evident. This clinical course contrasted sharply with that in MSA, where early urinary dysfunction usually proceeded to sudomotor dysfunction or orthostatic hypotension (p = 0.004), followed by respiratory dysfunction (p = 0.0004). Results of pharmacological tests also distinguished PAF from MSA. Progression and prognosis in patients with PAF did not worsen, unlike the steady progressive autonomic dysfunction 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 significantly between PAF and MSA. Patients with PAF had slower functional deterioration and a better prognosis.
Mabuchi
N
N
Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya 466-8550, Japan.
Hirayama
M
M
Koike
Y
Y
Watanabe
H
H
Ito
H
H
Kobayashi
R
R
Hamada
K
K
Sobue
G
G
D000203:Activities of Daily Living / Q000145:classification; D000368:Aged; D001341:Autonomic Nervous System / Q000503:physiopathology; D001342:Autonomic Nervous System Diseases / Q000175:diagnosis* / Q000503:physiopathology; D001794:Blood Pressure / Q000502:physiology; D003627:Data Interpretation, Statistical; D003937:Diagnosis, Differential; D018450:Disease Progression; D004305:Dose-Response Relationship, Drug; D005260:Female; D005500:Follow-Up Studies; D006339:Heart Rate / Q000502:physiology; D006801:Humans; D007024:Hypotension, Orthostatic / Q000175:diagnosis / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D019578:Multiple System Atrophy / Q000175:diagnosis* / Q000503:physiopathology; D009460:Neurologic Examination / Q000706:statistics & numerical data; D009638:Norepinephrine / Q000097:blood; D011379:Prognosis; D018667:Tilt-Table Test
D009638:Norepinephrine