Multiple system atrophy
ICD-10 G23.2; G23.3 · ICD-11 8D87.0

Treatment of Neurogenic Orthostatic Hypotension in Multiple System Atrophy

Multiple system atrophy frequently involves autonomic dysfunction. When neurogenic orthostatic hypotension is identified, a first-line protocol guides the clinical response.

Clinical Scenario

This protocol applies to patients with multiple system atrophy who have neurogenic orthostatic hypotension — defined as a systolic blood pressure drop of at least 20 mm Hg, or a diastolic blood pressure drop of at least 10 mm Hg, occurring within 3 minutes of moving from supine to standing.

Management Approach

The initial step focuses on reviewing medications that may be aggravating orthostatic blood pressure drops — including antihypertensive and dopaminergic agents — with reductions made on an individual basis. Nonpharmacologic supportive measures and strategies to expand intravascular volume are also part of this first-line approach. The complete protocol specifies the full sequence and conditions for each intervention.

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References

DOI: 10.1212/cont.0000000000001598

Features of autonomic dysfunction may include neurogenic orthostatic hypotension (a systolic blood pressure decrease by at least 20 mm Hg or diastolic blood pressure decrease by at least 10 mm Hg within 3 minutes from being supine to standing or by a tilt-table test without a compensated increase in heart rate [less than 0.5 beats/min in every mm Hg decrease in systolic blood pressure]).

The first step to control orthostatic hypotension is to discontinue or reduce the dose of the aggravating drugs, such as antihypertensive medications.

Dopaminergic medications should be reduced based on an individual risk-benefit assessment because they can exacerbate orthostatic hypotension.

Other nonpharmacologic measures include elastic stockings or abdominal binders.

Approaches to expanding intravascular volume include the intake of two teaspoons of salt a day (corresponding to 11 g to 12 g of sodium chloride), 2 L to 2.5 L of water a day, and the mineralocorticoid agonist fludrocortisone of 0.1 mg orally 1 to 2 times a day.

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