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The link between idiopathic intracranial hypertension, fibromyalgia, and chronic fatigue syndrome: exploration of a shared pathophysiology

Authors Hulens M, Rasschaert R, Vansant G, Stalmans I, Bruyninckx F, Dankaerts W

Received 8 September 2018

Accepted for publication 30 October 2018

Published 10 December 2018 Volume 2018:11 Pages 3129—3140

DOI https://doi.org/10.2147/JPR.S186878

Checked for plagiarism Yes

Review by Single-blind

Peer reviewers approved by Dr Colin Mak

Peer reviewer comments 2

Editor who approved publication: Dr Michael E Schatman


Mieke Hulens,1 Ricky Rasschaert,2 Greet Vansant,3 Ingeborg Stalmans,4,5 Frans Bruyninckx,6 Wim Dankaerts1

1Department of Rehabilitation Sciences, Faculty of Kinesiology and Rehabilitation Sciences, Musculoskeletal Rehabilitation Research Unit, University of Leuven, Leuven, Belgium; 2Department of Neurosurgery, Sint-Jozefziekenhuis, Bornem, Belgium; 3Department of Social and Primary Health Care, Public Health Nutrition, University of Leuven, Leuven, Belgium; 4Department of Neurosciences, Ophthalmology Research Group, University of Leuven KU Leuven, Leuven, Belgium; 5Department of Ophthalmology, University Hospitals UZ Leuven, Leuven, Belgium; 6Clinical Electromyography Laboratory, Department of Academic Consultants, Faculty of Medicine, University Hospitals UZ Leuven, Leuven, Belgium

Purpose: Idiopathic intracranial hypertension (IICH) is a condition characterized by raised intracranial pressure (ICP), and its diagnosis is established when the opening pressure measured during a lumbar puncture is elevated >20 cm H2O in nonobese patients or >25 cm H2O in obese patients. Papilledema is caused by forced filling of the optic nerve sheath with cerebrospinal fluid (CSF). Other common but underappreciated symptoms of IICH are neck pain, back pain, and radicular pain in the arms and legs resulting from associated increased spinal pressure and forced filling of the spinal nerves with CSF. Widespread pain and also several other characteristics of IICH share notable similarities with characteristics of fibromyalgia (FM) and chronic fatigue syndrome (CFS), two overlapping chronic pain conditions. The aim of this review was to compare literature data regarding the characteristics of IICH, FM, and CFS and to link the shared data to an apparent underlying physiopathology, that is, increased ICP.
Methods: Data in the literature regarding these three conditions were compared and linked to the hypothesis of the shared underlying physiopathology of increased cerebrospinal pressure.
Results: The shared characteristics of IICH, FM, and CFS that can be caused by increased ICP include headaches, fatigue, cognitive impairment, loss of gray matter, involvement of cranial nerves, and overload of the lymphatic olfactory pathway. Increased pressure in the spinal canal and in peripheral nerve root sheaths causes widespread pain, weakness in the arms and legs, walking difficulties (ataxia), and bladder, bowel, and sphincter symptoms. Additionally, IICH, FM, and CFS are frequently associated with sympathetic overactivity symptoms and obesity. These conditions share a strong female predominance and are frequently associated with Ehlers-Danlos syndrome.
Conclusion: IICH, FM, and CFS share a large variety of symptoms that might all be explained by the same pathophysiology of increased cerebrospinal pressure.

Keywords: chronic pain, fatigue, headache, Ehlers-Danlos, sympathetic activity, lymphatic olfactory pathway, small fiber neuropathy, Ménière’s disease, Tarlov cysts

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