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Intranasal dexmedetomidine for adrenergic crisis in familial dysautonomia

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Abstract

Purpose

To report the use of intranasal dexmedetomidine, an α2-adrenergic agonist for the acute treatment of refractory adrenergic crisis in patients with familial dysautonomia.

Methods

Case series.

Results

Three patients with genetically confirmed familial dysautonomia (case 1: 20-year-old male; case 2: 43-year-old male; case 3: 26-year-old female) received intranasal dexmedetomidine 2 mcg/kg, half of the dose in each nostril, for the acute treatment of adrenergic crisis. Within 8–17 min of administering the intranasal dose, the adrenergic crisis symptoms abated, and blood pressure and heart rate returned to pre-crises values. Adrenergic crises eventually resumed, and all three patients required hospitalization for investigation of the cause of the crises.

Conclusions

Intranasal dexmedetomidine is a feasible and safe acute treatment for adrenergic crisis in patients with familial dysautonomia. Further controlled studies are required to confirm the safety and efficacy in this population.

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Authors and Affiliations

  1. Department of Neurology, Dysautonomia Center, New York University School of Medicine, New York, NY, USA

    Christy L. Spalink, Erin Barnes, Jose-Alberto Palma, Lucy Norcliffe-Kaufmann & Horacio Kaufmann

Authors
  1. Christy L. Spalink
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  2. Erin Barnes
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  3. Jose-Alberto Palma
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  4. Lucy Norcliffe-Kaufmann
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  5. Horacio Kaufmann
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Corresponding author

Correspondence to Horacio Kaufmann.

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Conflict of interests

Dr. Kaufmann is Editor-in-Chief of Clinical Autonomic Research; Dr. Palma is Managing Editor of Clinical Autonomic Research.

Funding

Dysautonomia Foundation and National Institutes of Health (U54-NS065736-01).

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Spalink, C.L., Barnes, E., Palma, JA. et al. Intranasal dexmedetomidine for adrenergic crisis in familial dysautonomia. Clin Auton Res 27, 279–282 (2017). https://doi.org/10.1007/s10286-017-0442-6

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  • DOI: https://doi.org/10.1007/s10286-017-0442-6

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