With constant headaches, who is the real "culprit"?

How to diagnose and differential diagnosis for new-onset daily headache patients? The Journal of Neurology reported a case of a patient who had no previous history of headaches and presented to the doctor because of new-onset daily persistent headaches for 1 year
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Let's learn the clinical reasoning process of this case together
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Yimaitong compiles and organizes, please do not reprint without authorization
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Case brief introduction The patient is a 66-year-old male who presented to the doctor with "new-onset daily persistent headache for 1 year"
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The patient has no history of headaches.
The headaches started 1 year ago without obvious predisposing factors
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The pain is mild and persistent whole-head compression, accompanied by photophobia and phonophobia
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The patient denied nausea/vomiting or migraine with aura-like symptoms
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Migraine preventive drugs including botulinum toxin A injection, nortriptyline, gabapentin and metoprolol are ineffective
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Nervous system examinations, including fundoscopy, were normal
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Questions to ponder: 1.
What are the differential diagnoses of new daily persistent headaches? 2.
What other clinical or examinations can help narrow the differential diagnosis? Diagnosis and differential diagnosis New-onset daily persistent headache (NDPH) is a unique primary headache.
The headache starts on a certain day, but does not relieve afterwards.
The patient can always determine the exact date of the onset of the headache [1]
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Although some patients have reported some triggers before the onset of headache, there is no obvious causal trigger for NDPH, and its pathophysiology is still unclear [2]
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There are many primary and secondary headaches that are clinically misdiagnosed as NDPH
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Careful recording of the medical history is essential to narrow the scope of the differential diagnosis, and special attention should be paid to classic red flags and medical history (Table 1)
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On further questioning, the patient stated that he experienced immediate headaches during the Valsalva maneuver and bending forward
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The headache caused by Valsalva may last up to an hour and will improve within 15-30 minutes after lying down
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The patient denied a history of head or neck trauma
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 Questions to ponder: 1.
Which diagnosis should be considered in view of the new medical history? 2.
What are the typical MRI findings that may appear in this situation? When diagnosed without a lumbar puncture, a postural headache relieved by lying down in an upright position indicates spontaneous CSF spinal cord leakage, which is called spontaneous intracranial hypotension (SIH)
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However, this term is somewhat misleading, because patients generally have normal CSF pressure [3]
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Since the disease was first described in 1938, the disease has been intensively studied [4], but it is estimated that 46%-55% of SIH patients have no clear leakage site and are difficult to treat effectively [5]
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SIH is still one of the important differential diagnoses of NDPH [4]
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The typical manifestation of SIH is an upright headache, which usually occurs or worsens within 15 minutes of an upright position, but some patients get worse after a few hours in an upright position
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The headache improves within 15-30 minutes after lying down
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SIH headaches vary in nature.
They can be pulsatile or non-pulsatile, diffuse or focal, and are most commonly found in the occipital or suboccipital area, and their severity varies greatly
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Although some patients may describe lightning headache, it is generally subacute onset [4]
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Although postural headache is a typical symptom of SIH, this postural component may become less obvious over time, and some patients may not have the postural component of headache from the onset
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Other patients reported significant headaches caused by Valsalva, half-day headaches, or paradoxical headaches that aggravated when lying down [4]
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Other symptoms of SIH include neck pain or meningitis, hearing changes, tinnitus, imbalance, and cognitive decline or confusion [6]
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For orthostatic headaches, consideration should also be given to: autonomic adjustment disorders (such as postural orthostatic tachycardia syndrome), Chiari malformation decompression surgery, cervicogenic headache, and third ventricle colloid cyst [6]
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The MRI manifestations of SIH can be summarized as SEEPS: (1) subdural effusion, (2) enhanced dura mater (diffuse), (3) hyperemia of the venous structure, (4) hyperemia of the pituitary gland, and (5) sagging of the brain tissue (figure) 1) [4]
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Ptosis of brain tissue can be manifested as cerebellar tonsils (sometimes imitating Chiari type 1 malformation), disappearance of the prepontic or supraspontic cistern with curvature of the optic chiasm on the pituitary fossa, flat anterior pons and crowding of the posterior fossa [4,6]
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These imaging findings are compensatory changes in CSF volume reduction [6,7]
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Figure 1 The characteristics of SIH on cranial MRI
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(A) Sagittal T1-weighted image shows spontaneous intracranial hypotension with prolapse of the brain; narrowing of the prepontic cistern (arrow); downward shift of the optic chiasm and optic tract (arrow)
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(B) Axial T1 enhanced image shows diffuse dural enhancement (arrow)
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(C) Coronal T1 enhanced image shows diffuse dural enhancement (arrow) and bilateral subdural effusion (arrow)
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The patient's head MRI scan in the hospital was normal, but we read the film again to consider that there may be slight prolapse
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Spinal MRI did not show epidural effusion indicating CSF leakage
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 Questions to ponder: 1.
What treatment methods or neuroimaging examinations will be considered at present? Treatment There are many treatment methods for suspicious spinal leakage based on clinical manifestations or MRI manifestations
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Conservative methods include bed rest, hydration, increased caffeine intake, and use of abdominal adhesives
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In addition, SIH patients can resolve spontaneously without any special treatment
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Although a purely conservative approach may be effective for some patients, many patients are ineffective and other treatments can be considered
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Steroids, intravenous caffeine and theophylline have limited effectiveness [6]
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The most commonly used treatment is epidural blood enrichment (EBP)
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EBP may be blind or targeted at suspicious target areas
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If the leak site is known, a fibrin sealant can be considered
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In a retrospective study of 25 patients receiving EBP treatment, 9 patients (36%) responded well to the first patch, and 5 patients (33%) were asymptomatic after applying the second patch; 8 received Among patients who had more than 3 (4 on average) EBP, 4 (50%) responded well [8]
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For severe patients, intrathecal injection of normal saline or artificial CSF may temporarily relieve symptoms, but permanent repair of leakage can be a permanent relief
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For patients whose non-surgical measures have failed and the location of CSF leakage is clear, surgical treatment should be performed [4]
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In the case of uncertain diagnosis, when a CSF leak is suspected clinically but there is no obvious evidence on MRI, the radionuclide water level map can be considered to determine whether there is a leak [4]
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The patient received L1/L2 EBP and had no headaches for 24 hours
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When the headache recurs, its severity is reduced, and the duration of the headache caused by Valsalva is shorter
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The second L1/L2 EBP and the third T10/T11 and L4/L5 dual-level EBP did not produce significant benefits
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Amitriptyline slightly improved the severity of headaches caused by Valsalva
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The level chart shows an incomplete distribution or a flat appearance on a convex surface
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Plain and enhanced MRI of the head showed similar mild sagging and mild dural enhancement
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The patient's headache spontaneously worsened after 4 months, and re-examination of MRI showed enhanced dura mater and prolapse
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If SIH is suspected from the clinical history and neuroimaging findings, and conservative measures (such as EBP) fail, the next step is to determine the location of the leak
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The patient's CT myelography failed to identify the leakage site, but showed obvious paravertebral veins at T3-4/T4-5
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Targeted repair with fibrin glue cannot be improved
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DSA examination showed that the contrast agent leaked significantly into the right paraspine area of ​​T7-8, indicating the presence of a slow-flowing CSF venous fistula (Figure 2)
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The patient immediately underwent surgical ligation of the right T7/T8 nerve root, and the headache was completely relieved, and remained headache-free during the 6-month follow-up
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Figure 2 Digital subtraction myelography (DSM) image of the patient
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DSM is a form of dynamic imaging that injects contrast agent in the sheath under fluoroscopy and has the ability to subtract the pre-contrast image to enhance the contrast effect
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The patient’s DSM (A–C) showed significant leakage of contrast agent into the right paravertebral T7-T8 area (arrow), indicating the presence of a slow-flowing fine CSF venous fistula
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 Discussion SIH is an important cause of new daily headaches, but its clinical and imaging manifestations are different, and even experienced neurologists may misdiagnose
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This case highlights the importance of understanding the secondary SIH of CSF venous fistula
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CSF venous fistula is relatively unresponsive to EBP, which further emphasizes the importance of correct diagnosis, because according to our experience, correct diagnosis is very important, which can be treated by surgery [10-12] to completely relieve pain in patients who are otherwise difficult to treat
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References: [1] Headache Classification Committee of the International Headache Society.
The International Classification of Headache Disorders, 3rd edition (beta version).
Cephalalgia 2013;33:629–808.
[2] Uniyal R, Paliwal VK, Anand S, Ambesh P.
New daily persistent headache: an evolving entity.
Neurol India 2018;66:679–687.
[3] Kranz PG, Tanpitukpongse TP, Choudhury KR, Amrhein TJ, Gray L.
How common is normal cerebrospinal fluid pressure in spontaneous intracranial hypotension? Cephalalgia 2016;36:1209–1217.
[4] Schievink WI.
Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.
JAMA 2006;295:2286–2296.
[5] Kranz PG, Amrhein TJ, Schievink WI, Karikari IO, Gray L.
The “hyperdense paraspinal vein” sign: a marker ofCSF-venous fistula.
AJNRAmJNeuroradiol 2016;37:1379–1381.
[6] Mokri B.
Spontaneous low pressure, low CSF volume headaches:spontaneous CSF leaks.
Headache 2013;53:1034–1053.
[7] Mokri B.
Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia: evolution of a concept.
Mayo Clin Proc 1999;74:1113–1123.
[8 ] Sencakova D, Mokri B, McClelland RL.
The efficacy of epidural blood patch in spontaneous CSF leaks.
Neurology 2001;57:1921–1923.
[9] Schievink WI, Atkinson JL.
Spontaneous intracranial hypotension.
J Neurosurg 1996; 84:151 –152.
[10] Kumar N, Diehn FE, Carr CM, et al.
Spinal CSF venous fistula: a treatable etiology for CSF leaks in craniospinal hypovolemia.
Neurology 2016;86:2310–2312.
[11] Schievink WI, Moser FG , Maya MM, Prasad RS.
Digital subtraction myelography for the identification of spontaneous spinal CSF-venous fistulas.
J Neurosurg Spine 2016; 24:960–964.
[12] Duvall JR, Robertson CE, Cutsforth-Gregory JK, et al.
Headache due to spontaneous spinal cerebrospinal fluid leak secondary to cerebrospinal fluid-venous fistula: Case series.
Cephalalgia Epub 2019 Oct 9.
Original index: Jaclyn R.
Duvall, Carrie E.
Robertson, Mark A.
Whealy and Ivan Garza.
Clinical Reasoning: An etiology of new daily persistent headache.
Neurology January 7, 2020 94:e114-e120.
http://n.
neurology.
org/content/94/1/e114.
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