Skip to main content

Advertisement

SpringerLink
Log in

Dysautonomia and response to guanfacine in individuals with an SCN9A variant

  • Letter to the Editor
  • Published:
Clinical Autonomic Research Aims and scope Submit manuscript

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  1. Benarroch EE (2021) “Dysautonomia”: a plea for precision. Clin Auton Res 31:27–29

    Article  PubMed  Google Scholar 

  2. Benjamin ER, Pruthi F, Olanrewaju S, Ilyin VI, Crumley G, Kutlina E, Valenzano KJ, Woodward RM (2006) State-dependent compound inhibition of Nav1. 2 sodium channels using the FLIPR Vm dye: on-target and off-target effects of diverse pharmacological agents. J Biomol Screen 11:29–39

    Article  CAS  PubMed  Google Scholar 

  3. Biaggioni I, Shibao CA, Diedrich A, Muldowney JAS 3rd, Laffer CL, Jordan J (2019) Blood pressure management in afferent baroreflex failure: JACC review topic of the week. J Am Coll Cardiol 74:2939–2947

    Article  PubMed  PubMed Central  Google Scholar 

  4. Darwin C (2005) The Expression of the Emotions in Man and Animals. Penguin Classics. London, England

  5. Davidson JR, Foa EB, Connor KM, Churchill LE (2002) Hyperhidrosis in social anxiety disorder. Prog Neuropsychopharmacol Biol Psychiatry 26:1327–1331

    Article  PubMed  Google Scholar 

  6. de Cassia CR, Lammens M, Blevins C, Rodgers K, Gurau A, Yamauchi S, Kim C, Forrester J, Liu E, Ha J, Mei Y, Boehm C, Wohler E, Sobreira N, Rowe PC, Valle D, Brock MV, Bosmans F (2024) Anxiety and dysautonomia symptoms in patients with a Na(V)1.7 mutation and the potential benefits of low-dose short-acting guanfacine. Clin Auton Res 34:191–201

    Article  Google Scholar 

  7. Dillon RC, Palma JA, Spalink CL, Altshuler D, Norcliffe-Kaufmann L, Fridman D, Papadopoulos J, Kaufmann H (2017) Dexmedetomidine for refractory adrenergic crisis in familial dysautonomia. Clin Auton Res 27:7–15

    Article  PubMed  Google Scholar 

  8. Faber CG, Hoeijmakers JG, Ahn HS, Cheng X, Han C, Choi JS, Estacion M, Lauria G, Vanhoutte EK, Gerrits MM, Dib-Hajj S, Drenth JP, Waxman SG, Merkies IS (2012) Gain of function Nanu1.7 mutations in idiopathic small fiber neuropathy. Ann Neurol 71:26–39

    Article  CAS  PubMed  Google Scholar 

  9. Han C, Hoeijmakers JG, Liu S, Gerrits MM, te Morsche RH, Lauria G, Dib-Hajj SD, Drenth JP, Faber CG, Merkies IS, Waxman SG (2012) Functional profiles of SCN9A variants in dorsal root ganglion neurons and superior cervical ganglion neurons correlate with autonomic symptoms in small fibre neuropathy. Brain 135:2613–2628

    Article  PubMed  Google Scholar 

  10. Jakala P, Riekkinen M, Sirvio J, Koivisto E, Kejonen K, Vanhanen M, Riekkinen P Jr (1999) Guanfacine, but not clonidine, improves planning and working memory performance in humans. Neuropsychopharmacology 20:460–470

    Article  CAS  PubMed  Google Scholar 

  11. Novak P, Systrom DM, Marciano SP, Knief A, Felsenstein D, Giannetti MP, Hamilton MJ, Nicoloro-SantaBarbara J, Saco TV, Castells M, Farhad K, Pilgrim DM, Mullally WJ (2024) Mismatch between subjective and objective dysautonomia. Sci Rep 14:2513

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  12. Palma JA, Yadav R, Gao D, Norcliffe-Kaufmann L, Slaugenhaupt S, Kaufmann H (2021) Expanding the Genotypic Spectrum of Congenital Sensory and Autonomic Neuropathies Using Whole-Exome Sequencing. Neurol Genet 7:e568

    Article  PubMed  PubMed Central  Google Scholar 

  13. Pasierski M, Kolba W, Szulczyk B (2023) Guanfacine inhibits interictal epileptiform events and sodium currents in prefrontal cortex pyramidal neurons. Pharmacol Rep 75:331–341

    Article  PubMed  PubMed Central  Google Scholar 

  14. Sorkin EM, Heel RC (1986) Guanfacine: a review of its pharmacodynamic and pharmacokinetic properties, and therapeutic efficacy in the treatment of hypertension. Drugs 31:301–336

    Article  CAS  PubMed  Google Scholar 

  15. Suarez GA, Opfer-Gehrking TL, Offord KP, Atkinson EJ, O’Brien PC, Low PA (1999) The autonomic symptom profile: a new instrument to assess autonomic symptoms. Neurology 52:523–528

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Dysautonomia Center and Autonomic Disorders Division, New York University Langone Health and New York University Grossman School of Medicine, 530 First Av, Suite 9Q, New York, NY, 10016, USA

    Jose-Alberto Palma

Authors
  1. Jose-Alberto Palma
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Jose-Alberto Palma.

Ethics declarations

Conflict of interest

Dr. Palma receives research funding support from the National Institutes of Health, the Familial Dysautonomia Foundation, and a salary from Eli Lilly.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Palma, JA. Dysautonomia and response to guanfacine in individuals with an SCN9A variant. Clin Auton Res 34, 303–305 (2024). https://doi.org/10.1007/s10286-024-01029-0

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10286-024-01029-0

Keywords

Search

Navigation