Familial Dysautonomia Medication

Updated: Sep 11, 2018
  • Author: Robert A D'Amico, MD, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
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Medication

Medication Summary

Labile autonomic status results in precarious homeostasis. Any stress, emotional or physical, may trigger a dysautonomic crisis, which can result in ocular complications as increased exposure and dehydration of the corneal surface can be associated. Diazepam, in conjunction with clonidine, is the most effective drug for the dysautonomic crisis. Initial dose of diazepam should be effective in stopping vomiting, normalizing the blood pressure, and decreasing agitation. However, if hypertension persists, then clonidine is added. Crisis usually resolves abruptly and is marked by normalization of personality and return of appetite.

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Benzodiazepines

Class Summary

By binding to specific receptor-sites these agents appear to potentiate the effects of gamma-aminobutyric acid (GABA) and facilitate inhibitory GABA neurotransmission and other inhibitory transmitters.

Diazepam (Valium)

Suppresses crisis symptoms by enhancing GABA; secondarily decreases dopamine release.

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H2 receptor antagonists

Class Summary

These agents are reversible competitive blockers of histamine at the H2 receptors, particularly those in the gastric parietal cells where they inhibit acid secretion. The H2 antagonists are highly selective, do not affect the H1 receptors, and are not anticholinergic agents.

Ranitidine (Zantac)

H2 antagonist that may be a useful adjunct in reducing emesis volume.

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Alpha-adrenergic agonists

Class Summary

These agents stimulate alpha-adrenoreceptors in brain stem, activating an inhibitory neuron, which in turn results in reduced sympathetic outflow. These effects result in a decrease in vasomotor tone and heart rate.

Clonidine (Catapres)

A central alpha-adrenergic agonist that suppresses peripheral release of norepinephrine, resulting in lower blood pressure; used to control symptomatic hypertension suggested if diastolic hypertension (>85 mm Hg) persists 30 min after diazepam administration.

Midodrine (ProAmatine)

A pure peripheral alpha-adrenergic agonist, which causes peripheral vasoconstriction and raises blood pressure without stimulating cardiac receptors; used for treatment of postural hypotension.

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Corticosteroids

Class Summary

These agents have profound and varied metabolic effects.

Fludrocortisone (Florinef)

A mineralocorticoid; promotes retention of sodium and water, which increases intravascular volume; used for treatment of postural hypotension.

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