Clinical Features of and Prognosis Factors for Spontaneous Intracranial Hypotension_West China Medical Journal

Authors：

 HaoXiaoting ¹ , WangBin ² , ChenNing ¹ , YanBo ¹ , HeLi ¹ ,  ZhouDong ¹

1. Department of Neurology, West China Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;
2. Department of Neurology, Chengdu No.6 People's Hospital, Chengdu, Sichuan 610051, P. R. China;

Corresponding author：

ZhouDong, Email: zhoudong66@yahoo.de

Keywords：

Spontaneous intracranial hypotension; Clinical features; Treatment; Prognosis

DOI：

10.7507/1002-0179.20160057

Video：

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Objective To summarize the clinical features of and prognosis factors for spontaneous intracranial hypotension (SIH). Methods We continuously registered hospitalized patients diagnosed with SIH from December 1st, 2010 to February 1st, 2014. Etiology information and clinical features were collected at the first day of admission. Routine blood test and lumbar puncture were done as soon as possible. Every patient got position and fluid infusion therapy. During the one-week follow-up, headache level was evaluated with Visual Analogue Scale (VAS). Results There were 110 patients included, and among them, 39(35.5%) were male and 71(64.55%) were female. The age of onset was between 17 and 91 years old with a mean onset age of (42.0±12.4). Besides postural headache, common signs were nausea (68 cases, 61.8%), vomiting (63 cases, 57.3%), dizziness (40 cases, 36.4%), neck pain (27 cases, 24.5%), and tinnitus (23 cases, 20.9%). VAS at baseline was (7.46±0.86), and at the last follow-up, VAS was (3.45±2.17), with an average improvement of 53.75%. Patients with ANA marker positive had better prognosis. Conclusion SIH can accompany serious brain stem and cerebellum signs, and even meningeal irritation. Cerebrospinal fluid (CSF) changes are similar to virus infection with negative serum virus screening. If CSF leak cannot be found on imaging, patients can improve through fluid infusion therapy and postural treatment.

Citation： HaoXiaoting, WangBin, ChenNing, YanBo, HeLi, ZhouDong. Clinical Features of and Prognosis Factors for Spontaneous Intracranial Hypotension. West China Medical Journal, 2016, 31(2): 203-207. doi: 10.7507/1002-0179.20160057 Copy

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2.	Mokri B. Spontaneous low cerebrospinal pressure/volume headaches[J]. Curr Neurol Neurosci Rep, 2004, 4(2):117-124.
3.	Mokri B. Syndrome of cerebral spinal fluid hypovolemia:clinical and imaging features and outcome[J]. Neurology, 2001, 56(11):1607-1608.
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8.	Mea E, Chiapparini L, Savoiardo M, et al. Headache attributed to spontaneous intracranial hypotension[J]. Neurol Sci, 2008, 29(Suppl 1):S164-S165.
9.	Leung GK, Ho J, Pu JK. Pituitary enlargement in spontaneous intracranial hypotension——a diagnostic pitfall[J]. Acta Neurochir (Wien), 2011, 153(12):2445-2446.
10.	Tsui EY, Ng SH, Cheung YK, et al. Spontaneous intracranial hypotension with diffuse dural enhancement of the spinal canal and transient enlargement of the pituitary gland[J]. Eur J Radiol, 2001, 38(1):59-63.
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13.	Shima K, Ishihara S, Tomura S. Pathophysiology and diagnosis of spontaneous intracranial hypotension[J]. Acta Neurochir Suppl, 2008, 102:153-156.
14.	Liu CH, Lai SC, Lin CS, et al. Spontaneous intracranial hypotension with cerebrospinal fluid leakage at 2 sites treated by epidural blood patch[J]. Acta Neurol Taiwan, 2011, 20(4):272-277.
15.	Mea E, Chiapparini L, Savoiardo M, et al. Application of IHS criteria to headache attributed to spontaneous intracranial hypotension in a large population[J]. Cephalalgia, 2009, 29(4):418-422.
16.	江云, 王谨, 龚向阳, 等. 42例自发性脊髓脑脊液漏所致颅内低压的临床特点分析[J]. 浙江大学学报:医学版, 2014, 43(1):83-88.
17.	Shephard MK, Macgregor EA, Zakrzewska JM. Orofacial pain:a guide for the headache physician[J]. Headache, 2014, 54(1):22-39.
18.	Schievink WI, Schwartz MS, Maya MM, et al. Lack of causal association between spontaneous intracranial hypotension and cranial cerebrospinal fluid leaks[J]. J Neurosurg, 2012, 116(4):749-754.
19.	Bachmann-Mennenga B, Philipps J, Haukamp F, et al. Spontaneous intracranial hypotension. A rare syndrome with good treatment options[J]. Anaesthesist, 2004, 53(1):45-52.
20.	Khalid S, Das PG, Mohammad H, et al. Spontaneous intracranial hypotension in a patient with Marfan's syndrome treated with epidural blood patch——a case report[J]. Middle East J Anaesthesiol, 2012, 21(4):635-638.
21.	Azevedo Júnior D. Spontaneous intracranial hypotension. Report of a case[J]. Arq Neuropsiquiatr, 1995, 53(3-B):676-679.
22.	Kranz PG, Gray L, Taylor JN. CT-guided epidural blood patching of directly observed or potential leak sites for the targeted treatment of spontaneous intracranial hypotension[J]. AJNR Am J Neuroradiol, 2011, 32(5):832-838.
23.	Ferrante E, Arpino I, Citterio A, et al. Epidural blood patch in Trendelenburg position pre-medicated with acetazolamide to treat spontaneous intracranial hypotension[J]. Eur J Neurol, 2010, 17(5):715-719.
24.	Sainani NI, Lawande MA, Pungavkar SA, et al. Spontaneous intracranial hypotension:a study of six cases with Mr findings and literature review[J]. Australas Radiol, 2006, 50(5):419-423.
25.	Kranz PG, Stinnett SS, Huang KT, et al. Spinal meningeal diverticula in spontaneous intracranial hypotension:analysis of prevalence and myelographic appearance[J]. AJNR Am J Neuroradiol, 2013, 34(6):1284-1289.

1. Park ES, Kim E. Spontaneous intracranial hypotension:clinical presentation, imaging features and treatment[J]. J Korean Neurosurg Soc, 2009, 45(1):1-4.
2. Mokri B. Spontaneous low cerebrospinal pressure/volume headaches[J]. Curr Neurol Neurosci Rep, 2004, 4(2):117-124.
3. Mokri B. Syndrome of cerebral spinal fluid hypovolemia:clinical and imaging features and outcome[J]. Neurology, 2001, 56(11):1607-1608.
4. Chung SJ, Kim JS, Lee MC. Syndrome of cerebral spinal fluid hypovolemia:clinical and imaging features and outcome[J]. Neurology, 2000, 55(9):1321-1327.
5. Moreno MJ, Fernandez Pena CM, Escriche D, et al. Cervical pain as a clinical sign of spontaneous intracraneal hypotension[J]. Neurologia, 1996, 11(6):236-238.
6. Porta-Etessam J, Di Capua D, Jorquera M, et al. Orthostatic headache and bilateral abducens palsy secondary to spontaneous intracranial hypotension[J]. J Headache Pain, 2011, 12(1):109-111.
7. Albayram S, Ozer H, Kara B. Diagnostic criteria for spontaneous spinal CSF leaks and intracranial hypotension[J]. AJNR Am J Neuroradiol, 2008, 29(10):e94.
8. Mea E, Chiapparini L, Savoiardo M, et al. Headache attributed to spontaneous intracranial hypotension[J]. Neurol Sci, 2008, 29(Suppl 1):S164-S165.
9. Leung GK, Ho J, Pu JK. Pituitary enlargement in spontaneous intracranial hypotension——a diagnostic pitfall[J]. Acta Neurochir (Wien), 2011, 153(12):2445-2446.
10. Tsui EY, Ng SH, Cheung YK, et al. Spontaneous intracranial hypotension with diffuse dural enhancement of the spinal canal and transient enlargement of the pituitary gland[J]. Eur J Radiol, 2001, 38(1):59-63.
11. Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension[J]. JAMA, 2006, 295(19):2286-2296.
12. Bassani L, Graffeo CS, Behrooz N, et al. noninvasive diagnosis and management of spontaneous intracranial hypotension in patients with marfan syndrome:case report and review of the literature[J]. Surg Neurol Int, 2014, 5:8.
13. Shima K, Ishihara S, Tomura S. Pathophysiology and diagnosis of spontaneous intracranial hypotension[J]. Acta Neurochir Suppl, 2008, 102:153-156.
14. Liu CH, Lai SC, Lin CS, et al. Spontaneous intracranial hypotension with cerebrospinal fluid leakage at 2 sites treated by epidural blood patch[J]. Acta Neurol Taiwan, 2011, 20(4):272-277.
15. Mea E, Chiapparini L, Savoiardo M, et al. Application of IHS criteria to headache attributed to spontaneous intracranial hypotension in a large population[J]. Cephalalgia, 2009, 29(4):418-422.
16. 江云, 王谨, 龚向阳, 等. 42例自发性脊髓脑脊液漏所致颅内低压的临床特点分析[J]. 浙江大学学报:医学版, 2014, 43(1):83-88.
17. Shephard MK, Macgregor EA, Zakrzewska JM. Orofacial pain:a guide for the headache physician[J]. Headache, 2014, 54(1):22-39.
18. Schievink WI, Schwartz MS, Maya MM, et al. Lack of causal association between spontaneous intracranial hypotension and cranial cerebrospinal fluid leaks[J]. J Neurosurg, 2012, 116(4):749-754.
19. Bachmann-Mennenga B, Philipps J, Haukamp F, et al. Spontaneous intracranial hypotension. A rare syndrome with good treatment options[J]. Anaesthesist, 2004, 53(1):45-52.
20. Khalid S, Das PG, Mohammad H, et al. Spontaneous intracranial hypotension in a patient with Marfan's syndrome treated with epidural blood patch——a case report[J]. Middle East J Anaesthesiol, 2012, 21(4):635-638.
21. Azevedo Júnior D. Spontaneous intracranial hypotension. Report of a case[J]. Arq Neuropsiquiatr, 1995, 53(3-B):676-679.
22. Kranz PG, Gray L, Taylor JN. CT-guided epidural blood patching of directly observed or potential leak sites for the targeted treatment of spontaneous intracranial hypotension[J]. AJNR Am J Neuroradiol, 2011, 32(5):832-838.
23. Ferrante E, Arpino I, Citterio A, et al. Epidural blood patch in Trendelenburg position pre-medicated with acetazolamide to treat spontaneous intracranial hypotension[J]. Eur J Neurol, 2010, 17(5):715-719.
24. Sainani NI, Lawande MA, Pungavkar SA, et al. Spontaneous intracranial hypotension:a study of six cases with Mr findings and literature review[J]. Australas Radiol, 2006, 50(5):419-423.
25. Kranz PG, Stinnett SS, Huang KT, et al. Spinal meningeal diverticula in spontaneous intracranial hypotension:analysis of prevalence and myelographic appearance[J]. AJNR Am J Neuroradiol, 2013, 34(6):1284-1289.

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Clinical Features of and Prognosis Factors for Spontaneous Intracranial Hypotension

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