Headaches from spontaneous spinal CSF leaks and intracranial hypotension

Because I have had a headache almost every day for over two months now, I’ve been investigating the kind of headaches that are more common with connective tissue disorders, like EDS.  Below are several articles from PubMed that describe these headaches:

Connective tissue disorders with spontaneous sp… [Neurosurgery. 2004] – PubMed – NCBI

OBJECTIVE: Intracranial hypotension attributable to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of posturalheadaches. The cause of these leaks is poorly understood, but it is likely multifactorial and may involve a primary connective tissue disorder. We undertook a study to estimate the contribution of systemic connective tissue disorders to the development of spontaneous spinal CSF leaks.

CONCLUSION: Findings suggesting connective tissue disorders are common among patients with spontaneous spinal CSF leaks, and manifestations may be subtle. A variety of disorders can be identified, probably reflecting genetic heterogeneity. Problems with wound healing may occur as a result of the systemic nature of the underlying connective tissue disorder.

Diagnostic Criteria for Spontaneous Spinal CSF Leaks and Intracranial Hypotension

BACKGROUND AND PURPOSE: Comprehensive diagnostic criteria encompassing the varied clinical and radiographic manifestations of spontaneous intracranial  hypotension are not available. Therefore, we propose a new set of diagnostic criteria.

CONCLUSIONS:  A new diagnostic scheme is presented reflecting the wide spectrum of clinical and radiographic manifestations of spontaneous  spinal CSF leaks and intracranial hypotension.

Criterion A:  The demonstration of a spinal CSF leak on spinal imaging

Criterion B, if criterion A not met:  Cranial MR imaging changes of spontaneous intracranial hypotension and at least one of the following: 1) low opening pressure,  2) spinal meningeal diverticulum, or 3) improvement of symptoms after epidural blood patch

A positive MR imaging result is  defined as the presence of at least one of the 3 major MR imaging findings of spontaneous intracranial hypotension (ie, subdural  fluid collections, enhancement of the pachymeninges, and sagging of the brain).

Criterion C, if criteria A and B not met:  The presence of all of the following or at least 2 of the following if typical orthostatic headaches are present: 1) low opening  pressure, 2) spinal meningeal diverticulum, and 3) improvement of symptoms after epidural blood patch.

In our practice, epidural blood patching or conservative treatment consisting of bed rest, oral hydration, and oral caffeine  is recommended after cranial MR imaging confirms the clinical suspicion of spontaneous intracranial hypotension

Discussion

However, it has become well established that the clinical spectrum of spontaneous intracranial hypotension is unusually varied.1,5 An orthostatic headache is the prototypical clinical manifestation of spontaneous intracranial hypotension, but unlike the  ICDH-2 criteria, the headache does not invariably occur within 15 minutes of sitting or standing.1 Moreover, many different headache patterns have been reported in spontaneous intracranial hypotension, such as thunderclap  headache, nonpositional headache, exertional headache, cough headache, “second-half-of-the-day” headache, and even paradoxic  headaches that worsen when lying down, whereas rarely there is no history of headache

The main clinical component of the diagnostic criteria is improvement of symptoms after epidural blood patching.  The placement of an epidural blood patch is the most commonly used initial treatment technique for spontaneous intracranial  hypotension, and most patients react favorably to an epidural blood patch, even if only temporarily.

CT myelography is the study of choice to detect the CSF leak and is more reliable than radionuclide cisternography

Cranial MR imaging has revolutionized our understanding of spontaneous intracranial hypotension and is an important component  of the current diagnostic criteria. Characteristic findings consist of subdural fluid collections, enhancement of the pachymeninges,  engorgement of venous structures, pituitary hyperemia, and sagging of the brain (mnemonic, SEEPS).1 Of these 5 findings, only subdural fluid collections, enhancement of the pachymeninges, and sagging of the brain are reliably  demonstrated on the initial MR imaging examination

Abnormal MR imaging results, particularly  the enhancement of the pachymeninges, have been considered to be the sine qua non of spontaneous intracranial hypotension,22 but it has become well established that a sizeable minority of patients (28% in our study) have normal results on brain MR  imaging

Spontaneous Low Pressure Headaches: Historical Background, Terminology, and Objective

Although the triad of orthostatic headaches, low CSF pressures, and diffuse pachymeningeal enhancement is the classic hallmark of this disorder, the variability is indeed substantial. This includes patients who do not display meningeal enhancement,[12] those who may not have headaches, or patients who may show CSF OPs that are well within normal limits.

Spontaneous Low Pressure Headaches: Orthostatic Headaches Without CSF Leak

Orthostatic Headaches Without CSF Leak  Orthostatic headaches are the hallmark of CSF leaks. However, as discussed earlier, not all headaches of CSF leaks are orthostatic and also not all orthostatic headaches are due to CSF leaks.

Recurrent spontaneous spinal cerebrospinal fluid… [J Neurosurg. 2003] – PubMed – NCBI

Objective: Intracranial hypotension due to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of posturalheadaches, but reliable follow-up data are lacking. The authors undertook a study to determine the risk of a recurrent spontaneous spinal CSF leak.

A recurrent spinal CSF leak was defined on the basis of computerized tomography myelography evidence of a CSF leak in a previously visualized but unaffected spinal location. Five patients (28%) developed a recurrent spinal CSF leak; the mean age of these four women and one man was 36 years.

A recurrent CSF leak developed in five (38%) of 13 patients who had undergone surgical CSF leak repair, compared with none (0%) of five patients who had been treated non-surgically (p = 0.249). The recurrent leak occurred between 10 and 77 months after the initial CSF leak, but within 2 or 3 months of successful surgical repair of the leak in all patients.

CONCLUSIONS:  Recurrent spontaneous spinal CSF leaks are not rare, and the recent successful repair of such a leak at another site may be an important risk factor.

Spontaneous spinal cerebrospinal fluid leaks and intrac… [JAMA. 2006] – PubMed – NCBI

OBJECTIVE: To summarize existing evidence regarding the epidemiology, pathophysiology, diagnosis, and management of spontaneous spinal CSF leaks and intracranial hypotension.

CONCLUSIONS: Spontaneous intracranial hypotension is not rare but it remains underdiagnosed

Orthostatic headache without CSF leak. [Neurology. 2008] – PubMed – NCBI

OBJECTIVE:  To describe the syndrome of orthostatic headache without CSF leak and propose potential mechanism

After mean follow-up of 45.5 months (range 31 to 67), one patient had experienced a complete spontaneous recovery while five had persistent orthostatic headache.

CONCLUSIONS:  Orthostatic headaches can occur without evidence of intracranial hypotension or detectable CSF leak despite extensive diagnostic testing. Clinical features alone are unlikely to differentiate between orthostatic headache with and without identifiable CSF leak. Potential mechanisms include 1) very slow or intermittent CSF leak that cannot be detected at the time of evaluation or by current diagnostic means or 2) increased compliance of the lower spinal CSF space without actual leak.