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Spontaneous Cerebrospinal Fluid (CSF) Leaks 

Guest Editor: Dr. Ivan Garza

 

Dr. Ivan Garza is an Assistant Professor of Neurology at the Mayo Clinic College of Medicine and a Consultant at the Mayo Clinic’s Department of Neurology in Rochester, MN. He is board certified in Neurology by the American Board of Psychiatry and Neurology (ABPN) and holds subspecialty certification in Headache Medicine by the United Council of Neurologic Subspecialties (UCNS).

Spontaneous Cerebrospinal Fluid (CSF) Leaks
Ivan Garza, MD

Spontaneous cerebrospinal fluid (CSF) leaks typically present with orthostatic headaches. Less commonly, spontaneous CSF leaks can present with other headache types. Nausea, vomiting, hearing disturbances, diplopia, back pain, and dizziness are not uncommon associated symptoms. Although the exact cause of CSF leaks often remains uncertain, some patients may be predisposed due to disorders of connective tissue or spinal meningeal anomalies. When a spontaneous CSF leak is suspected, head MRI with contrast is ordinarily the first study to obtain. Common abnormalities seen include diffuse dural enhancement, subdural fluid collections, engorged cerebral venous sinuses, and cerebral descent. Important to know is the fact that despite typical clinical features, head MRI may occasionally be unremarkable. In such situations, ancillary studies may help answer whether a CSF leak is present. Radioisotope cisternography and spine MRI are helpful tools in such occurrences. Presently, CT-myelography remains the most reliable test to find the exact spinal CSF leak site. Often, however, the exact CSF leak site is not found.

Regarding management, no concrete guideline is available. When conservative measures such as rest and caffeine have failed, autologous epidural blood patch (EBP) is the treatment of choice. A lumbar “blind” EBP can be tried if the exact leak site is unknown although a targeted one when the site of the leak is known is preferred. The latter increases the odds of treatment success. Not uncommonly, patients with spontaneous CSF leaks may require multiple EBP. Epidural injection of fibrin glue may be considered in non-responders and surgery is a reasonable option for those who have failed less invasive measures as long as the site of the CSF leak has been definitely established.

Manuscripts published in Headache pertaining to the syndrome of spontaneous CSF leaks are outlined below.

ILLUSTRATIVE CASES AND OVERVIEW
Levin, M. Resident and Fellow Section: Postural headache, Lavigne C, McGeeney BE. Headache. 2009 Jun;49(6):938-40.

Ferrante E, Savino A, Sances G, Nappi G. Spontaneous intracranial hypotension syndrome: report of twelve cases. Headache. 2004 Jun;44(6):615-22.

Arjona A, Carneado J, López-Lozano JJ, Blasco MR. Orthostatic headache. Headache. 1998 Feb;38(2):142-3.

Marcelis J, Silberstein SD. Spontaneous low cerebrospinal fluid pressure headache. Headache. 1990 Mar;30(4):192-6.

Lasater GM. Primary intracranial hypotension. The low spinal fluid pressure syndrome. Headache. 1970 Jul;10(2):63-6.


POSSIBLE RISK FACTORS
Ahn AH, Berman BD, Dillon WP. Spontaneous intracranial hypotension-hypovolemia associated with tacrolimus. Headache. 2010 Sep;50(8):1386-9. Epub 2010 May 31.

Albayram S, Baş A, Ozer H, Dikici S, Gulertan SY, Yuksel A. Spontaneous intracranial hypotension syndrome in a patient with Marfan syndrome and autosomal dominant polycystic kidney disease. Headache. 2008 Apr;48(4):632-6.

Mokri B. Familial occurrence of spontaneous spinal CSF leaks: underlying connective tissue disorder. Headache. 2008 Jan;48(1):146-9.

Patrick A, McAuley D, Roxburgh R. Spontaneous low-pressure headache complicating bagpiping. Headache. 2007 Apr;47(4):611-3.

Chung SJ, Ki CS, Lee MC, Lee JH. Fibrillin-1 gene analysis of Korean patients with spontaneous CSF hypovolemia. Headache. 2007 Jan;47(1):111-5.

Prasad S, El-Haddad G, Zhuang H, Khella S. Intracranial hypotension following chiropractic spinal manipulation. Headache. 2006 Oct;46(9):1456-8.

University of South Alabama Headache Center. Acute and persistent nonpositional headache induced by dural trauma and cerebrospinal fluid leak. Headache. 2004 Feb;44(2):189.

Ontachi Y, Sakajiri K, Higashi S, Yokoyama K, Suzuki M, Asakura H, Nakao S. Congenital predisposition to spontaneous intracranial hypotension: a case report. Headache. 2003 Jun;43(6):678-80.

Fukutake T, Sakakibara R, Mori M, Araki M, Hattori T. Chronic intractable headache in a patient with Marfan's syndrome. Headache. 1997 May;37(5):291-5.


CLINICAL MANIFESTATIONS
Famularo G, Minisola G, Gigli R. Thunderclap headache and spontaneous intracranial hypotension. Headache. 2005 Apr;45(4):392-3; author reply 393.

Batsis JA, Phy MP. Intracranial hypotension: aggravation of headache at high altitude. Headache. 2005 Apr;45(4):380-4.

Evans RW, Boes CJ. Spontaneous low cerebrospinal fluid pressure syndrome can mimic primary cough headache. Headache. 2005 Apr;45(4):374-7.

Tsai PH, Wang SJ, Lirng JF, Fuh JL. Spontaneous intracranial hypotension presenting as mental deterioration. Headache. 2005 Jan;45(1):76-80.

Ferrante E, Savino A. Nonpostural headache by spontaneous intracranial hypotension. Headache. 2003 Feb;43(2):127-9.

Kawase Y, Ikeda K, Murata K, Iwamoto K, Ito H, Iwasaki Y. Nonpostural headache in spontaneous intracranial hypotension. Headache. 2008 Apr;48(4):641-2.

Kong DS, Park K, Nam DH, Lee JI, Kim ES, Kim JS, Hong SC, Shin HJ, Eoh W, Kim JH. Atypical spontaneous intracranial hypotension (SIH) with nonorthostatic headache. Headache. 2007 Feb;47(2):199-203.

Horikoshi T. Patulous eustachian tube in spontaneous intracranial hypotension syndrome - a response. Headache. 2009 Mar;49(3):482-3.

Albayram S, Bas A. Patulous eustachian tube in spontaneous intracranial hypotension syndrome. Headache. 2008 Feb;48(2):300.

Horikoshi T, Imamura S, Matsuzaki Z, Umeda T, Uchida M, Mitsuka K, Kinouchi H. Patulous Eustachian tube in spontaneous intracranial hypotension syndrome. Headache. 2007 Jan;47(1):131-5.


RARE AND UNUSUAL CASES
Seok HY, Eun MY, Choi JY, Roh JH, Park MH, Park KW, Kwon DY. Atypical spontaneous intracranial hypotension with a head-shaking headache. Headache. 2010 Jul;50(7):1211-4.

Cheshire WP Jr, Wharen RE Jr. Trigeminal neuralgia in a patient with spontaneous intracranial hypotension. Headache. 2009 May;49(5):770-3.

Schievink WI, Maya MM, Chow W, Louy C. Reversible cerebral vasoconstriction in spontaneous intracranial hypotension. Headache. 2007 Feb;47(2):284-7.


DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS
Schievink WI, Dodick DW, Mokri B, Silberstein S, Bousser MG, Goadsby PJ. Diagnostic criteria for headache due to spontaneous intracranial hypotension: a perspective. Headache. 2011 Oct;51(9):1442-4.

Rozen T, Swidan S, Hamel R, Saper J. Trendelenburg position: a tool to screen for the presence of a low CSF pressure syndrome in daily headache patients. Headache. 2008 Oct;48(9):1366-71.

Schievink WI, Akopov SE. Filum ependymoma mimicking spontaneous intracranial hypotension. Headache. 2005 May;45(5):607-9.


NEUROIMAGING FINDINGS
Albayram S, Asik M, Hasiloglu ZI, Dikici AS, Erdemli HE, Altintas A. Pathological contrast enhancement of the oculomotor and trigeminal nerves caused by intracranial hypotension syndrome. Headache. 2011 May;51(5):804-8.

Decontee J, Joel C, John R. Images from headache: intracranial hypotension complicating Marfan's syndrome. Headache. 2009 Nov-Dec;49(10):1534-5.

Balgera R, Rigamonti A, Agostoni E. Images from headache: spontaneous intracranial hypotension presenting as "pseudo" subarachnoid hemorrhage. Headache. 2008 Apr;48(4):614-5.

Whyte CA, Eshkar NS. Spontaneous intracranial hypotension. Headache. 2008 Jan;48(1):140-1.

Rothrock JF. Low pressure headache and pseudosubdural hematomas. Headache. 2003 Oct;43(9):1009.

Eross EJ, Dodick DW. Prolonged neurologic complication and MRI abnormalities consequent to intracranial hypotension. Headache. 2003 Apr;43(4):415.

Bruera OC, Bonamico L, Giglio JA, Sinay V, Leston JA, Figuerola ML. Intracranial hypotension: the nonspecific nature of MRI findings. Headache. 2000 Nov-Dec;40(10):848-52.

Brightbill TC, Goodwin RS, Ford RG. Magnetic resonance imaging of intracranial hypotension syndrome with pathophysiological correlation. Headache. 2000 Apr;40(4):292-9.

Kasner SE, Rosenfeld J, Farber RE. Spontaneous intracranial hypotension: headache with a reversible Arnold-Chiari malformation. Headache. 1995 Oct;35(9):557-9.


NEUROIMAGING MODALITIES REQUIRING FURTHER STUDY
Hasiloglu ZI, Albayram S, Gorucu Y, Selcuk H, Cagil E, Erdemli HE, Adaletli I. Assessment of CSF Flow Dynamics Using PC-MRI in Spontaneous Intracranial Hypotension. Headache. 2012 May;52(5):808-819.

Rozen TD, Beams JL, Lock JH Jr. Is contrast-enhanced 3D spoiled gradient echo MRI a superior way to image pachymeningeal enhancement in intracranial hypotension? Headache. 2012 Jan;52(1):140-1.

Schievink WI, Tourje J. Upright MRI in spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. Headache. 2007 Oct;47(9):1345-6.

Albayram S, Gunduz A, Saip S, Ozer H, Gulsen F, Kocer N, Islak C. Intrathecal gadolinium-enhanced MR-cisternography in spontaneous intracranial hypotension associated with Behcet's syndrome. Headache. 2007 Apr;47(4):613-6.


COMPLICATIONS
Schievink WI, Maya MM. Cerebral venous thrombosis in spontaneous intracranial hypotension. Headache. 2008 Nov-Dec;48(10):1511-9.

Tan K, Venketasubramanian N, Hwang CY, Lim CC. My headache does not get better when I lie down: spontaneous intracranial hypotension complicated by venous thrombosis. Headache. 2008 Jan;48(1):149-52.

Evan RW, Mokri B. Spontaneous intracranial hypotension resulting in coma. Headache. 2002 Feb;42(2):159-60.

Sung V. Acute respiratory failure complicating intracranial hypotension. Headache. 2007 Oct;47(9):1340-1.


TREATMENT
Schievink WI. A novel technique for treatment of intractable spontaneous intracranial hypotension: lumbar dural reduction surgery. Headache. 2009 Jul;49(7):1047-51. Epub 2009 May 15.

Hannerz J, Dahlgren G, Irestedt L, Meyerson B, Ericson K. Treatment of idiopathic intracranial hypotension: cervicothoracic and lumbar blood patch and peroral steroid treatment. Headache. 2006 Mar;46(3):508-11.

Pascual LF, Santos S, Escalza I, Iñiguez C, Morales-Asín F. Spontaneous intracranial hypotension: quick clinical and magnetic resonance imaging response to corticosteroids. A case report. Headache. 2002 May;42(5):359-61.

Kamada M, Fujita Y, Ishii R, Endoh S. Spontaneous intracranial hypotension successfully treated by epidural patching with fibrin glue. Headache. 2000 Nov-Dec;40(10):844-7.

Virtual Issue compiled 13 June 2012

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