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The Link Between Idiopathic Intracranial Hypertension, Fibromyalgia, and Chronic Fatigue Syndrome: Exploration of a Shared Pathophysiology

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The Link Between Idiopathic Intracranial Hypertension, Fibromyalgia, and Chronic Fatigue Syndrome: Exploration of a Shared Pathophysiology Journal name: Journal of Pain Research Article Designation: Hypothesis Year: 2018 Volume: 11 Journal of Pain Research Dovepress Running head verso: Hulens et al Running head recto: The link between intracranial hypertension, fibromyalgia, and CFS open access to scientific and medical research DOI: http://dx.doi.org/10.2147/JPR.S186878 Open Access Full Text Article HYPOTHESIS The link between idiopathic intracranial hypertension, fibromyalgia, and chronic fatigue syndrome: exploration of a shared pathophysiology Mieke Hulens1 Purpose: Idiopathic intracranial hypertension (IICH) is a condition characterized by raised Ricky Rasschaert2 intracranial pressure (ICP), and its diagnosis is established when the opening pressure measured 3 during a lumbar puncture is elevated 20 cm H O in nonobese patients or 25 cm H O in obese Greet Vansant > 2 > 2 Ingeborg Stalmans4,5 patients. Papilledema is caused by forced filling of the optic nerve sheath with cerebrospinal Frans Bruyninckx6 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 Wim Dankaerts1 forced filling of the spinal nerves with CSF. Widespread pain and also several other character- 1Department of Rehabilitation For personal use only. istics of IICH share notable similarities with characteristics of fibromyalgia (FM) and chronic Sciences, Faculty of Kinesiology and Rehabilitation Sciences, fatigue syndrome (CFS), two overlapping chronic pain conditions. The aim of this review was Musculoskeletal Rehabilitation to compare literature data regarding the characteristics of IICH, FM, and CFS and to link the Research Unit, University of Leuven, shared data to an apparent underlying physiopathology, that is, increased ICP. Leuven, Belgium; 2Department of Neurosurgery, Sint-Jozefziekenhuis, Methods: Data in the literature regarding these three conditions were compared and linked to Bornem, Belgium; 3Department of the hypothesis of the shared underlying physiopathology of increased cerebrospinal pressure. Social and Primary Health Care, Public Health Nutrition, University Results: The shared characteristics of IICH, FM, and CFS that can be caused by increased ICP of Leuven, Leuven, Belgium; include headaches, fatigue, cognitive impairment, loss of gray matter, involvement of cranial 4 Department of Neurosciences, nerves, and overload of the lymphatic olfactory pathway. Increased pressure in the spinal canal Ophthalmology Research Group, University of Leuven KU Leuven, and in peripheral nerve root sheaths causes widespread pain, weakness in the arms and legs, Leuven, Belgium; 5Department of walking difficulties (ataxia), and bladder, bowel, and sphincter symptoms. Additionally, IICH, Journal of Pain Research downloaded from https://www.dovepress.com/ by 54.70.40.11 on 05-Nov-2019 Ophthalmology, University Hospitals FM, and CFS are frequently associated with sympathetic overactivity symptoms and obesity. UZ Leuven, Leuven, Belgium; 6Clinical Electromyography Laboratory, These conditions share a strong female predominance and are frequently associated with Ehlers- Department of Academic Consultants, Danlos syndrome. Faculty of Medicine, University IICH, FM, and CFS share a large variety of symptoms that might all be explained Hospitals UZ Leuven, Leuven, Belgium Conclusion: 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 Correspondence: Mieke Hulens Department of Rehabilitation Sciences, Plain language summary Faculty of Kinesiology and Rehabilitation The pathological mechanisms that cause both fibromyalgia (FM) and chronic fatigue syndrome Sciences, Musculoskeletal Rehabilitation (CFS) are incompletely understood. FM and CFS share very similar symptoms with idiopathic Research Unit, University of Leuven, Overwegstraat 14, 3051 Sint-Joris-Weert, intracranial hypertension (IICH), a condition characterized by an increase in intracranial Leuven, Belgium pressure (ICP) due to an unknown cause. The authors reviewed the literature to explore these Tel +32 478 338003 Fax +32 16 470559 common symptoms and to link them to the hypothesis that increased intracranial and spinal Email [email protected] fluid pressure is the possible mechanism that initiates the multitude of symptoms in these submit your manuscript | www.dovepress.com Journal of Pain Research 2018:11 3129–3140 3129 Dovepress © 2018 Hulens et al. This work is published and licensed by Dove Medical Press Limited. The full terms of this license are available at https://www.dovepress.com/terms. php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work http://dx.doi.org/10.2147/JPR.S186878 you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed. For permission for commercial use of this work, please see paragraphs 4.2 and 5 of our Terms (https://www.dovepress.com/terms.php). Powered by TCPDF (www.tcpdf.org) 1 / 1 Hulens et al Dovepress conditions. The symptoms include neck pain, back pain, pain in increased spinal pressure. Figure 1 depicts an overview of arms and legs, numbness/tingling, headaches, fatigue, cognitive the interactions of ICP with all the nerves presented in this impairment, gradual loss of gray matter, in addition to symptoms paper, the olfactory lymphatic pathway, and the inner ear. involving cranial nerves, overload of the lymphatic system in the nasal mucosa and disturbance of the autonomic nervous system. Other shared characteristics include higher frequency in females Symptoms associated with and family members, and an association with obesity and Ehlers increased ICP Danlos syndrome (a connective tissue disorder). Headache These findings are relevant as they provide an alternative Most patients with IICH present with headaches. Approxi- hypothesis concerning the pathological mechanisms in FM and CFS. mately one in three patients with IICH may already suffer from chronic headaches before diagnosis.101 Additionally, Introduction there is significant overlap between the headache types IICH is a condition characterized by elevated ICP of unknown observed in patients with IICH and the headache types of etiology. Patients may present with headache, papilledema, primary headache disorders.59 When performing a lumbar and visual disturbances. The diagnosis is established when the puncture in patients with unresponsive migraine without opening pressure (OP) measured during a lumbar puncture papilledema, 86% display an OP >20 cm H O. Evacuation of is elevated >20 cm H O in nonobese patients and >25 cm 2 2 CSF induces headache remission in 77%.25 However, when H O in obese patients.100 2 the ICP is reduced in patients with IICH using a shunt, the Recently, these cutoff values used to define IICH have different types of headaches may persist. Therefore, headache been debated. ICP might rather be a continuum with no clear is a poor marker of disease activity.104 cutoff value. Additionally, headache and/or papilledema may In patients with FM or CFS, different types of primary be absent even if the ICP is above these cutoff values. As headaches such as migraine and tension headache are highly IICH and CFS share similar symptoms such as fatigue and prevalent.51,79 For personal use only. headache, Higgins et al hypothesized that the milder forms In patients with CFS suffering from headaches, a lumbar of IICH may present as CFS.41 puncture revealed an OP ≥20 cm H O in 40% of patients, Common but underappreciated symptoms of IICH 2 and evacuation of CSF improved headaches, alertness, and/ are neck pain, back pain, and radicular pain radiating to or fatigue in 85%.41 the arms and legs. These associated symptoms are due to the forced filling of the nerve roots with cerebrospinal fluid (CSF), irritating or compressing the nerve root fibers Fatigue inside.10,38,43,53,67,72,88,101 Therefore, it is likely that milder forms Fatigue is the most pronounced symptom of IICH and one of elevated ICP may also present as FM or unexplained pain. of the core symptoms of CFS and FM. Patients with CFS Indeed, FM has sensory symptoms and signs comparable to respond to spinal fluid evacuation the same way that patients 16,61,71,80,93,102 with IICH do, that is, improvement of the headaches and Journal of Pain Research downloaded from https://www.dovepress.com/ by 54.70.40.11 on 05-Nov-2019 those of peripheral neuropathy. Moreover, elec- 41 trophysiologic abnormalities have been detected in the limbs fatigue. of patients with FM.15,97 This hypothesis may also provide an explanation for Cognitive impairment several overlapping chronic pain conditions. Patients with IICH may suffer multidomain cognitive impair- While IICH is characterized by a significant increase in ment and impaired processing speed, which does not improve cerebrospinal pressure (CSP), FM and CFS may be the result after normalization of the ICP.103,105 of intermittent and/or mild chronic increased CSP. It is well established that FM and CFS patients may The data in the literature regarding the signs and symp- experience mental fogginess. In patients with FM, the term toms of these three conditions were compared and linked to “fibrofog” is used.
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