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7. Headache attributed to non-vascular intracranial disorder
7.2 Headache attributed to low cerebrospinal fluid pressure
7.2.1 Post-dural puncture headache
7.2.2 CSF fistula headache
7.2.3 Headache attributed to spontaneous intracranial hypotension
7. Headache attributed to non-vascular intracranial disorder General comment
Primary or secondary headache or both? When a headache occurs for the first time in close temporal relation to a non-vascular intracranial disorder, it is coded as a secondary headache attributed to that disorder. This remains true when the new headache has the characteristics of any of the primary headache disorders classified in Part one of ICHD-3 beta. When a pre-existing headache with the characteristics of a primary headache disorder becomes chronic, or is made significantly worse (usually meaning a two-fold or greater increase in frequency and/or severity), in close temporal relation to a non-vascular intracranial disorder, both the initial headache diagnosis and a diagnosis of 7. Headache attributed to non-vascular intracranial disorder (or one of its subtypes) should be given, provided that there is good evidence that the disorder can cause headache.
Introduction
In this chapter, the headaches are attributed to changes in intracranial pressure. Both increased and decreased cerebrospinal fluid (CSF) pressure can lead to headache. Other causes of headache here are non-infectious inflammatory diseases, intracranial neoplasia, seizures, rare conditions such as intrathecal injections and Chiari malformation type I, and other non-vascular intracranial disorders.
Compared with those on primary headaches, there are few epidemiological studies of these headache types. Controlled trials of therapy are almost non-existent.
For headache attributed to any of the non-vascular intracranial disorders listed here, the diagnostic criteria include whenever possible:
A. Headache fulfilling criterion C
B. A non-vascular intracranial disorder known to be able to cause headache has been diagnosed
C. Evidence of causation demonstrated by at least two of the following:
1. headache has developed in temporal relation to the onset of the non-vascular intracranial disorder
2. either or both of the following:
a) headache has significantly worsened in parallel with worsening of the non-vascular intracranial disorder
b) headache has significantly improved in parallel with improvement in the non-vascular intracranial disorder
3. headache has characteristics typical for the nonvascular intracranial disorder
4. other evidence exists of causation
D. Not better accounted for by another ICHD-3 diagnosis.
Headache persisting for more than 1 month after successful treatment or spontaneous resolution of the intracranial disorder usually has other mechanisms. Headache persisting for more than 3 months after treatment or remission of intracranial disorders is defined in the Appendix for research purposes. Such headache exists but has been poorly studied; Appendix entries are intended to stimulate further research into such headaches and their mechanisms.
7.2 Headache attributed to low cerebrospinal fluid pressure
Description:
Orthostatic headache in the presence of low cerebrospinal fluid (CSF) pressure (either spontaneous or secondary), or CSF leakage, usually accompanied by neck pain, tinnitus, changes in hearing, photophobia and/ or nausea. It remits after normalization of CSF pressure or successful sealing of the CSF leak.
Diagnostic criteria:
A. Any headache fulfilling criterion C
B. Low CSF pressure (<60 mm CSF) and/or evidence of CSF leakage on imaging
C. Headache has developed in temporal relation to the low CSF pressure or CSF leakage, or led to its discovery
D. Not better accounted for by another ICHD-3 diagnosis.
Comment:
7.2 Headache attributed to low cerebrospinal fluid pressure is usually but not invariably orthostatic. Headache that significantly worsens soon after sitting upright or standing and/or improves after lying horizontally is likely to be caused by low CSF pressure, but this cannot be relied on as a diagnostic criterion. Evidence of causation may depend on onset in temporal relation to the presumed cause together with exclusion of other diagnoses.
7.2.1 Post-dural puncture headache
Previously used term:
Post-lumbar puncture headache.
Description:
Headache occurring within 5 days of a lumbar puncture, caused by cerebrospinal fluid (CSF) leakage through the dural puncture. It is usually accompanied by neck stiffness and/or subjective hearing symptoms. It remits spontaneously within 2 weeks, or after sealing of the leak with autologous epidural lumbar patch.
Diagnostic criteria:
A. Any headache fulfilling criterion C
B. Dural puncture has been performed
C. Headache has developed within 5 days of the dural puncture
D. Not better accounted for by another ICHD-3 diagnosis.
Comment:
Independent risk factors for 7.2.1 Post-dural puncture headache have recently been demonstrated: female gender, age between 31 and 50 years, a previous history of 7.2.1 Post-dural puncture headache and orientation of the needle bevel perpendicular to the long axis of the spinal column at the time of the dural puncture.
7.2.2 CSF fistula headache
Description:
Orthostatic headache occurring after a procedure or trauma causing a persistent cerebrospinal fluid (CSF) leakage resulting in low intracranial pressure. It remits after successful sealing of the CSF leak.
Diagnostic criteria:
A. Any headache fulfilling criterion C
B. Both of the following:
1. a procedure has been performed, or trauma has occurred, known sometimes to cause persistent CSF leakage (CSF fistula)
2. low CSF pressure (<60 mm CSF) and/or evidence of low CSF pressure and/or of CSF leakage on MRI, myelography, CT myelography or radionuclide cisternography
C. Headache has developed in temporal relation to the procedure or trauma
D. Not better accounted for by another ICHD-3 diagnosis.
7.2.3 Headache attributed to spontaneous intracranial hypotension
Previously used terms:
Headache attributed to spontaneous low CSF pressure or primary intracranial hypotension; low CSF-volume headache; hypoliquorrhoeic headache.
Description:
Orthostatic headache caused by low cerebrospinal fluid (CSF) pressure of spontaneous origin. It is usually accompanied by neck stiffness and subjective hearing symptoms. It remits after normalization of CSF pressure.
Diagnostic criteria:
A. Any headache fulfilling criterion C
B. Low CSF pressure (<60 mm CSF) and/or evidence of CSF leakage on imaging
C. Headache has developed in temporal relation to the low CSF pressure or CSF leakage, or has led to its discovery
D. Not better accounted for by another ICHD-3 diagnosis.
Comments:
7.2.3 Headache attributed to spontaneous intracranial hypotension cannot be diagnosed in a patient who has had a dural puncture within the prior month.
The headache in patients with spontaneous CSF leaks or spontaneously low CSF pressure may resemble 7.2.1 Post-dural puncture headache, occurring immediately or within seconds of assuming an upright position and resolving quickly (within 1 minute) after lying horizontally. Alternatively it may show delayed response to postural change, worsening after minutes or hours of being upright and improving, but not necessarily resolving, after minutes or hours of being horizontal. Although there is a clear postural component in most cases of 7.2.3 Headache attributed to spontaneous intracranial hypotension, it may not be as dramatic or immediate as in 7.2.1 Post-dural puncture headache. The orthostatic nature of the headache at its onset should be sought when eliciting a history, as this feature may become much less obvious over time.
Although autologous epidural blood patches (EBPs) are frequently effective in sealing CSF leaks, the response to a single EBP may not be permanent, and complete relief of symptoms may not be achieved until two or more EBPs have been performed. However, some degree of sustained improvement, beyond a few days, is generally expected. In some cases, sustained improvement cannot be achieved with EBPs and surgical intervention may be required.
In patients with typical orthostatic headache and no apparent cause, after exclusion of postural orthostatic tachycardia syndrome (POTS) it is reasonable in clinical practice to provide autologous lumbar EBP.
It is not clear that all patients have an active CSF leak, despite a compelling history or brain imaging signs compatible with CSF leakage. Cisternography is an outdated test, now infrequently used; it is significantly less sensitive than other imaging modalities (MRI, CT or digital subtraction myelography). Dural puncture to measure CSF pressure directly is not necessary in patients with positive MRI signs such as dural enhancement with contrast.
The underlying disorder in 7.2.3 Headache attributed to spontaneous intracranial hypotension may be low CSF volume. A history of a trivial increase in intracranial pressure (e.g. on vigorous coughing) is sometimes elicited. Postural headache has been reported after coitus: such headache should be coded as 7.2.3 Headache attributed to spontaneous intracranial hypotension because it is most probably a result of CSF leakage.
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脳脊髄液減少症
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