Autonomic function testing and symptom severity in patients with suspected mast cell activation disorders, 2025, Stino et al.

[SNT Gatchaman]

Autonomic function testing and symptom severity in patients with suspected mast cell activation disorders

Spoiler: Authors

Amro M Stino; Jodi Nelson; Olivia Gutgsell; Ahmed Eldokla; Jasmine Liu; Cem Akin

We assessed both objective and subjective measures of autonomic severity in patients with formally classified mast cell activation disorder (MCAD), hereditary alpha tryptasemia (HaT), and systemic mastocytosis. In all subjects, autonomic disease severity was objectively mild but subjectively moderate to severe.

The presence of MCAD did not yield consistent differences on subjective or objective severity measures, although HaT did associate with lower objective (but not subjective) severity measures. In conclusion, assessment of MCAD and related disorders can be conducted using formal classification criteria, and in those with suspected dysautonomia, the workup should incorporate objective and subjective measures of dysautonomia.

Web | DOI | PDF | Autonomic Neuroscience: Basic and Clinical | Open Access

 

[SNT Gatchaman]

in our small cohort, we observed a potential mismatch between the objective and subjective autonomic disease burden in patients with mast cell disorders.

At present, there appears to be a mismatch between objective and subjective autonomic disease burden in patients with mast cell disorders, although substantiation of a definite pathophysiology underlying the subjective component is difficult, and there is a very real possibility that we selected for and tested individuals who had the greatest complaints. We also do not rule out the possibility that this mismatch may be due to limitations inherent in the measurement of the autonomic reflex screen.

Patients underwent a full battery of autonomic reflex screen testing (ARS), including QSART testing, heart rate responses to deep breathing (HRDB), heart rate and blood pressure responses to Valsalva, and head up tilt testing

Referencing Electrodiagnostic assessment of the autonomic nervous system: A consensus statement endorsed by the American Autonomic Society, American Academy of Neurology, and the International Federation of Clinical Neurophysiology (2021, Clinical Neurophysiology)

A "full battery" does not include any cerebrovascular assessment.

Noting in Is it time to move beyond blood pressure and heart rate during head-up tilt testing? (2024, Clinical Autonomic Research) —

As autonomic specialists we should all be aware that orthostatic changes in CBF, EtCO2, electrocortical activity, HRV, or oxygen extraction, among other physiological changes, can occur in the presence of a completely normal BP or HR response on HUTT, and that neglecting to measure such changes can be a missed opportunity, most importantly for some of our patients.

 

[Turtle]

Would be nice if researchers tried to include POTS, dOH and normal BP and heartrate in the OI studies.
I always feel forgotten when it's POTS, POTS, POTS sometimes dOH, and not my OI.
Thanks @SNT Gatchaman !

 

[SNT Gatchaman]

For orthostatic intolerance separate to neurodegenerative diseases, there seems to be a blind spot in relation to autonomic nervous system innervation of brain vasculature (and circulating chemical messenger control). As well as interactions with the trigeminovascular system.

See various reviews (mostly paywalled) in eg —

Autonomic Nervous System and Cerebrovascular Diseases (2023)

Autonomic control of cerebral blood flow: fundamental comparisons between peripheral and cerebrovascular circulations in humans (2022)

Chapter 16: Autonomic nervous system control of the cerebral circulation (2013)

The role of the autonomic nervous system in cerebral blood flow regulation in stroke: A review (2023)

Alterations in autonomic cerebrovascular control after spinal cord injury (2017)

The role of the autonomic nervous system in cerebral blood flow regulation in dementia: A review (2022)