A paradigm for chronic fatigue syndrome: caught between idiopathic intracranial hypertension and spontaneous intracranial hypotension; caused by cranial venous outflow obstruction

Abstract

Background: Clinical similarities between chronic fatigue syndrome and idiopathic intracranial hypertension, supported by measurements of intracranial pressure, invite suggestions that they may be connected, the first representing a mild version of the second. Yet, if this is to be the basis for a structural explanation for chronic fatigue syndrome, it already seems incomplete, failing to explain cases where disability seems disproportionate. Is there some other confounding variable? Purpose: To refine, in this theoretical paper, an earlier model connecting chronic fatigue syndrome with idiopathic intracranial hypertension to allow for a cerebrospinal fluid (CSF) leak. Model: In this model, the primary structural problem is acquired obstruction to cranial venous outflow. This obstruction can take different forms, may be intermittent and subtle, and even be mistaken for normal venous anatomy, yet would be the driving force behind a tendency towards increased intracranial pressure. This chronic elevation of intracranial pressure stresses the dural membrane maintaining the integrity of the subarachnoid space, which can rupture at a weak point, allowing CSF to leak away and intracranial pressure to fall. The clinical manifestation of this disorder is the product of the severity of cranial venous outflow compromise and of the competing forces on intracranial pressure. In some instances, a CSF leak will mitigate the effects of venous compromise, in others it will compound it, producing a disease spectrum ranging through idiopathic intracranial hypertension, chronic fatigue syndrome, fibromyalgia, and spontaneous intracranial hypotension. Conclusion:In chronic fatigue syndrome a normal intracranial pressure does not exclude significant physiological disturbance.

Chronic Fatigue Syndrome and Multiple Sclerosis have Reduced Craniospinal Compliance and Dilated Pressurized Bridging Cortical Veins: A Hypothesis Illustrated with Two Case Studies

“…Higgins et al have hypothesised that there is a reversal of the transmural pressure gradient in CFS on the basis of a similarity of the disorder to idiopathic intracranial hypertension [32]. Higgins et al suggest the sinus pressures are elevated but the CSF pressure is moderated by a chronic leakage of CSF [32]. A pressure gradient reversal would also dilate the sinuses, similar to our suggestion that they are stiffer than normal.…”

“…In CFS the same three possibilities will also apply if the veins are larger. Higgins et al have hypothesised that there is a reversal of the transmural pressure gradient in CFS on the basis of a similarity of the disorder to idiopathic intracranial hypertension [32]. Higgins et al suggest the sinus pressures are elevated but the CSF pressure is moderated by a chronic leakage of CSF [32].…”

Case report: Post-traumatic Tourette syndrome resolving with treatment of jugular venous narrowing; reconciling organic brain dysfunction following whiplash trauma with the absence of direct brain injury

“…Both conditions are characterized by the multiple symptoms cited above, and the clinical improvement seen with lumbar puncture applied not just to headache (usually present) but to many of the other symptoms as well ( 20 , 21 ). Various combinations of headache and these other complaints, therefore, rather than confounding the classical features of disordered intracranial pressure, may, in fact, be relatively strong indicators of a pressure disturbance ( 22 ).…”

“…Thus, cranial venous outflow obstruction might cause a CSF leak or may perpetuate a leak if a leak has developed for another reason. Moreover, symptoms may be complex, reflecting the balance between the clinical effects of the primary pathology (venous obstruction), the mitigating influence of a CSF leak on intracranial pressure, and the compounding problem of CSF depletion on brain function ( 22 ).…”

Life changing response to successive surgical interventions on cranial venous outflow: A case report on chronic fatigue syndrome

“…Many of the complex symptoms described in spontaneous intracranial hypotension are also seen in severe cases of chronic fatigue syndrome ( 11 , 12 ), and recently, a modification of the IIH/chronic fatigue hypothesis has been published to allow for the possibility of a CSF leak ( 13 ). In this modification, chronic fatigue syndrome represents a form of IIH that may be mild because the underlying pathology is mild, or a form in which the underlying pathology is more severe, but whose outward manifestation is modified, for better or worse, by the presence of a CSF leak.…”