Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy

Abstract

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.

Cardiac Organ Damage and Arterial Stiffness in Autonomic Failure

“…Los subtipos de INV primaria tienen un pronóstico diferente: la INVP tiene buen pronóstico, y las causas de muerte en general no están relacionadas con el trastorno neurovegetativo, 42 mientras que la AMS tiene mal pronóstico, con una mediana de supervivencia de ≈ 10 años a partir del diagnóstico 43 ; es probable que la EP con INV tenga un pronóstico intermedio; la EP en sí misma conlleva un mayor riesgo de mortalidad en comparación con la población general 44 ; en la población con INV, se ha comunicado muerte súbita de origen cardíaco y mortalidad de origen cardiovascular; 45 y la hipotensión ortostática se relaciona con una mayor morbimortalidad de origen cardiovascular en la población general. 46 En el contexto de INV primaria, la detección de hipertensión nocturna y en decúbito supino es útil para elegir el tipo y el momento de administración de agentes antihipotensivos.…”

“…Subtypes of primary AF have a different prognosis: PAF has a good prognosis, and causes of death are usually not related to the autonomic disorder, 42 whereas MSA has a poor prognosis, with a median survival of ≈10 years from diagnosis 43 ; PD with AF probably have an intermediate prognosis: PD itself bears an increased risk of mortality compared with the general population 44 ; sudden cardiac death and cardiovascular mortality have been described in AF population; 45 and orthostatic hypotension is related to an increased cardiovascular morbidity and mortality in the general population. 46 In the setting of primary AF, the detection of nighttime and supine hypertension is helpful in the choice of type and timing of antihypotensive agents.…”

Is there delayed gastric emptying in patients with multiple system atrophy? An analysis using the 13C-acetate breath test

“…Patients with MSA complain of constipation at a relatively early stage of the disease [22]. Furthermore, the rectal sphincter in MSA is frequency-denervated because of degeneration of Onuf's nuclei in the sacral cord [23].…”

Arterial Stiffness and Cardiovascular Autonomic Dysfunction in Patients with Parkinson's Disease

“…Our findings fit the model that cardiovascular autonomic failure modulates architectural changes in arterial walls, and the release of sympathetic outflows from baroreflex restraint contributes to autonomic BP disturbances, and therefore the disturbed autonomic BP serve the development of vascular hypertrophy. In PD, the mechanisms of target organ damage might be similar to those described in pure autonomic failure, because these patients are characterized by peripheral noradrenergic denervation [24,25]. Greater arterial stiffness would be expected to be associated with a decreased baroreflex function, and therefore we can postulate that complex interactions among arterial stiffness and OH may contribute to the microvascular injury in PD.…”