Major point
  1. “Spontaneous intracranial hypotension” (SIH) is also known as “headache attributed to spontaneous low cerebrospinal fluids (CSF) pressure”.
  2. Spontaneous intracranial hypotension is a headache disorder due to the leakage of CSF.
  3. The most common symptom of SIH is a severe orthostatic headache, which means that the symptoms may worsen during erect position.
  4. When SIH occurred, the symptom can be relieved by lying supine, drinking more water, and absorb more caffeine products.
  5. Current evidence supports that “multiple epidural blood patch (EBP)” as early as possible, can hasten the recovery.
  6. If there is an accompanied subdural hematoma (SDH), surgical drainage may be advised according to your clinical condition.
I. What is spontaneous intracranial hypotension (SIH)?
Spontaneous intracranial hypotension (SIH) is a headache disorder that is caused by cerebrospinal fluid (CSF) leakage. To diagnose this disease, physicians will utilize magnetic resonance images (MRI) encompassing brain and spinal region (using the novel Heavily T2-weighted myelography) to detect the site of CSF leakage. This disease is most popular among the young adults, and female has a higher incidence than male. For spontaneous cases, there is no specific identified predisposing factors. However, some patients may encounter a similar symptom that is caused by the injury of spinal dura (a membrane covering the CSF) after lumbar puncture, spinal anesthesia, after strenuous exercise or lifting heavy objects.

II. What are the common symptoms of SIH?
  1. Orthostatic headache: Patients may encounter a severe, disabling headache when getting from supine to erect position, and the pain may be relieved largely after lying down again. The location of the pain can be frontal, occipital or holocephalic.
  2. Neck pain or stiffness: The symptoms can extend downward, event to the lower back region.
  3. Some patient may present nausea, vomiting, tinnitus, hearing impairment or visual problems.
Having the aforementioned symptoms does NOT necessarily mean that you have SIH. Therefore, a detailed clinical inspection and examinations were warranted before the correct diagnosis, you can go to the neurology clinic for evaluation.
 
III. What are the common therapeutic strategies for SIH?
  1. Conservative treatments: Some SIH patients may recover spontaneously within weeks to months. The mainstay principle of treatment is to remain bedrest, intake adequate water or caffeinated beverages. These strategies can help maintain the CSF fluid and avoid from further leakage.
  2. Epidural blood patch (EBP): Current evidence have suggested that performing multiple EBPs as soon as possible can help decline the headache days and the rate of developing complications such as SDH. This treatment is performed by a well-trained physician. The physician will draw 20-30 mL of your own blood from the veins, and inject into the leakage site. When the blood clots, the leakage site can be healed.
  3. Surgical dural repair: If conservative treatment and EBP failed, your physician may offer you surgical repair.
  4. Subdural hematoma (SDH) drainage surgery: SDH is not a common complication. However, when the hematoma is large enough to cause cerebral compression, it may be life-threatening. Therefore, your physician may discuss the timing of drainage surgery if hematoma presented.
IV. How do we care for ourselves if diagnosed with SIH?
  1. Daily care:
    1. Remaining in a supine position can avoid from headache aggravation.
    2. Avoid strenuous maneuvers such as holding breath, coughing or lifting heavy objects.
    3. Keep hydrated: Daily intake of at least 1500 to 3000 mL is optimal for relieving the pain.
    4. Intake caffeinated beverages to increase the CSF pressure.
  2.  Post-Epidural blood patch care:
    1. Remain absolute bedrest for 2 days.
    2. Refrain from bending, turning, or stretching your neck or back.
    3. After two days of resting, before the first attempt to get off the bed, please be careful and move slowly. You can lean your body to one side holding the handrail, and gradually sit straight. Keep monitoring your symptoms during activity.
    4. Keep the injection site clean, and observe if there’s redness, swelling or signs likely to be an inflammatory or infection process.
    5. If you encounter any discomfort, especially limbs tingling sensation, weakness, more severe headache or other discomfort, please inform our medical staffs.
Reference
  1. D’Antona, L., Merchan, M. A. J., Vassiliou, A., Watkins, L. D., Davagnanam, I., Toma, A. K., & Matharu, M. S. (2021). Clinical presentation, investigation findings, and treatment outcomes of spontaneous intracranial hypotension syndrome: a systematic review and meta-analysis. JAMA neurology, 78(3), 329-337. https://doi.org/10.1001/jamaneurol.2020.4799
  2. Lin, P. T., Wang, Y. F., Hseu, S. S., Fuh, J. L., Lirng, J. F., Wu, J. W., ... & Wang, S. J. (2023). The SIH-EBP Score: A grading scale to predict the response to the first epidural blood patch in spontaneous intracranial hypotension. Cephalalgia, 43(3), 03331024221147488. https://doi.org/10.1177/03331024221147488
  3. Tsai, P. H., Fuh, J. L., Lirng, J. F., & Wang, S. J. (2007). Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case—control study. Cephalalgia, 27(8), 929-934. https://doi.org/10.1111/j.1468-2982.2007.01376.x 
  4. Tsai, P. H., Fuh, J. L., Lirng, J. F., & Wang, S. J. (2008). Comparisons between heavily T2-weighted MR and CT myelography studies in two patients with spontaneous intracranial hypotension. Cephalalgia, 28(6), 653-657. https://doi.org/10.1111/j.1468-2982.2008.01562.x
  5. Wu, J. W., Hseu, S. S., Fuh, J. L., Lirng, J. F., Wang, Y. F., Chen, W. T., ... & Wang, S. J. (2017). Factors predicting response to the first epidural blood patch in spontaneous intracranial hypotension. Brain, 140(2), 344-352. https://doi.org/10.1093/brain/aww328
  6. Wu, J. W., Wang, Y. F., Fuh, J. L., Lirng, J. F., Chen, S. P., Hseu, S. S., & Wang, S. J. (2018). Correlations among brain and spinal MRI findings in spontaneous intracranial hypotension. Cephalalgia, 38(14), 1998-2005. https://doi.org/10.1177/0333102418804161
  7. Wu, J. W., Wang, Y. F., Hseu, S. S., Chen, S. T., Chen, Y. L., Wu, Y. T., ... & Wang, S. J. (2021). Brain volume changes in spontaneous intracranial hypotension: Revisiting the Monro-Kellie doctrine. Cephalalgia, 41(1), 58-68. https://doi.org/ 10.1177/0333102420950385
  8. Wang S. J. (2021) Spontaneous Intracranial Hypotension. Continuum , 27(3),746-766. https://doi.org/10.1212/CON.0000000000000979 
  9. Wang, Y. F., Lirng, J. F., Fuh, J. L., Hseu, S. S., & Wang, S. J. (2009). Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension. Neurology, 73(22), 1892-1898. https://doi.org/10.1212/WNL.0b013e3181c3fd99
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