61% of Ehlers-Danlos (EH) syndromes cases have small fiber neuropathy
Fibromyalgia(FM) is now, in the worser forms, considered a small fiber neuropathy and in the milder forms with evidence of skin nerve immune attack. Hence EH could be said to have a version of FM with all the pain this entails. POTS like symptoms can occur in up to 3/4 cases.
Igharo D, Thiel JC, Rolke R, Akkaya M, Weis J, Katona I, Schulz JB, Maier A.
Skin biopsy reveals generalized small fibre neuropathy in hypermobile Ehlers-Danlos syndromes.
Eur J Neurol. 2023 Mar;30(3):719-728.
https://pubmed.ncbi.nlm.nih.gov/36437696/
The found a 10% frank postural tachycardia syndrome (PoTS).
However, with full investigation, some Dysautonomia is frequent:
Yael Gazit, A.Menahem Nahir, Rodney Grahame, Giris Jacob,
Dysautonomia in the joint hypermobility syndrome,
The American Journal of Medicine, Volume 115, Issue 1, 2003, Pages 33-40
https://www.sciencedirect.com/science/article/abs/pii/S0002934303002353
“Symptoms related to the autonomic nervous system, such as syncope and presyncope, palpitations, chest discomfort, fatigue, and heat intolerance, were significantly more common among patients.
Orthostatic hypotension, postural orthostatic tachycardia syndrome, and uncategorized orthostatic intolerance were found in 78% (21/27) of patients compared with in 10% (2/21) of controls.
Patients with the syndrome had a greater mean (± SD) drop in systolic blood pressure during hyperventilation than did controls (–11 ± 7 mm Hg vs. –5 ± 5 mm Hg, P = 0.02) and a greater increase in systolic blood pressure after a cold pressor test (19 ± 10 mm Hg vs. 11 ± 13 mm Hg, P = 0.06).
Patients with the syndrome also had evidence of α-adrenergic (as assessed by administration of phenylephrine) and β-adrenergic hyperresponsiveness (as assessed by administration of isoproterenol).”
Comment – Not only needing treatment for their dysautonomia, EH pains need to be treated like a FM small fiber neuraopathy. (Big topic upcoming)