Abstract
The majority, if not all, of the cases of spontaneous intracranial hypotension result from spontaneous cerebrospinal fluid (CSF) leaks. The disorder has a broad clinical and imaging spectrum with substantial variability in clinical and imaging features, in CSF findings, and in response to treatment. Headache is the most common symptom and is typically orthostatic, but with chronicity the orthostatic features may blur into a chronic, lingering headache. Other clinical features include neck pain, nausea, emesis, interscapular pain, diplopia, dizziness, change in hearing, visual blurring, radicular upper extremity symptoms, and a variety of other, but much less common, manifestations. The most common imaging feature is diffuse pachymeningeal gadolinium enhancement. Other manifestations include imaging evidence of sinking of the brain, subdural fluid collections, enlargement of the pituitary, engorgement of venous sinuses, and engorgement of epidural venous plexus. CSF opening pressure is typically low and CSF analysis may be normal or show increased protein concentration and a primarily lymphocytic pleocytosis. No longer can the entity be simply equated with post-spinal puncture headaches. The pathogenetic core and the independent variable is decrease in CSF volume, whereas clinical imaging and CSF findings, including CSF opening pressures, are all variables dependent on the loss of CSF volume. Many patients respond well to treatment, but some present stubborn therapeutic challenges. A subgroup of patients with orthostatic headaches is gradually recognized who have disorders other than CSF leaks.
Similar content being viewed by others
References and Recommended Reading
Raskin NH: Lumbar puncture headache: a review. Headache 1990, 30:197–200.
Schaltenbrand G: Neure anschauungen zur pathophyiologie der liquorzirkulation. Zentrablbl Neurochir 1938, 3:290–300.
Schaltenbrand, G: Normal and pathological physiology of the cerebrospinal fluid circulation. Lancet 1953, 1:805–808.
Woltman HW: Headache: a consideration of some of the more common types. Med Clin North Am 1940, 24:1159–1170.
Bell WE, Joynt RJ, Sahs AL: Low spinal fluid pressure syndromes. Neurology 1960, 10:512–521.
Lasater GM: Primary intracranial hypotension: the low spinal fluid pressure syndrome. Headache 1970, 10:63–66.
Marcelis J, Silberstein SD: Spontaneous low cerebrospinal fluid pressure headache. Headache 1990, 30:192–196.
Rando TA, Fishman RA: Spontaneous intracranial hypotension: report of two cases and review of the literature. Neurology 1992, 42:481–487.
Silberstein SD, Marcelis J: Headache associated with changes in intracranial pressure. Headache 1992, 32:84–94.
Labadie EL, van Antwerp J, Bamford CR: Abnormal lumbar isotrope cisternography in an unusual case of spontaneous hypoliquoorheic headache. Neurology 1976, 26:135–139.
Molins A, Alvarez J, Sumalla J, et al.: Cisternographic pattern of spontaneous liquoral hypotension. Cephalalgia 1990, 10:59–65.
Weber WE, Heidendahl GA, de Krom MC: Primary intracranial hypotension and abnormal radionuclide cisternography: report of a case and review of the literature. Clin Neurol Neurosurg 1991, 93:55–60.
Mokri B, Krueger BR, Miller GM, Piepgras DG: Meningeal gadolinium enhancement in low-pressure headaches [abstract]. Ann Neurol 1991, 30:294–295.
Sable SG, Ramadan NM: Meningeal enhancement and low CSF pressure headache: an MRI study. Cephalalgia 1991, 11:275–276.
Hochman MS, Naidich TP, Kobertz SA, Fernandez-Maitin A: Spontaneous intracranial hypotension with pachymeningeal enhancement on MRI. Neurology 1992, 42:1628–1630.
Fishman RA, Dillon WP: Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology 1993, 43:609–611.
Pannullo SC, Reich JB, Krol G, et al.: MRI changes in intracranial hypotension. Neurology 1993, 43:919–926.
Mokri B: Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia— evolution of a concept. Mayo Clin Proc 1999, 74:1113–1123.
Mokri B: Headache associated with abnormalities in intracranial structure or function: low cerebrospinal fluid pressure headache. In Wolff’s Headache. Edited by Silberstein SD, Lipton RB, Dalessio AR. Oxford: Oxford University Press; 2001:417–433. A comprehensive review of the subject.
Schrijver I, Schievink WI, Godfrey M, et al.: Spontaneous spinal cerebrospinal leak and minor skeletal features of Marfan syndrome: a fibrinillopathy. J Neurosurg 2002, 96:483–489. This study brings to attention the abnormalities of fibrillin in some of the patients with spontaneous CSF leaks.
Mokri B, Maher CO, Sencakova D: Spontaneous CSF leaks: underlying disorder of connective tissue. Neurology 2002, 58:814–816. This paper brings attention to underlying disorders of connective tissue, in particular fibrillin and elastin, in some of the patients with spontaneous CSF leaks.
Mokri B, Low PA: Orthostatic headaches without CSF leak in postural tachycardia syndrome. Neurology 2003, 61:980–982.
Chung SJ, Kim JS, Lee MC: Syndrome of cerebral spinal fluid hypovolemia. Neurology 2000, 55:1321–1327. A comprehensive review of 30 patients with CSF hypovolemia.
Mokri B: Spontaneous intracranial hypotension. Curr Neurol Neurosci Rep 2001, 1:109–117.
Mokri B, Piepgras DG, Miller GM: Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc 1997, 72:400–413.
Schievnik WI, Wijdicks EF, Meyer FB, Sonntag VK: Spontaneous intracranial hypotension mimicking aneurysmal subarachnoid hemorrhage. Neurosurgery 2001, 48:513–517.
Mokri B: Spontaneous CSF leaks mimicking benign exertional headaches. Cephalalgia 2002, 22:780–783.
Hochman MS, Naidich TP: Diffuse meningeal enhancement in patients with overdraining long-standing ventricular shunts. Neurology 1999, 52:406–409.
Mokri B, Atkinson JL, Piepgras DG: Absent headaches despite CSF volume depletion (intracranial hypotension). Neurology 2000, 55:573–575.
Horton JC, Fishman RA: Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak. Ophthalmology 1994, 101:244–251.
Brady-McCreery K, Spiedel S, Hussein MA, Coats DK: Spontaneous intracranial hypotension with unique strabismus due to third and fourth cranial neuropathies. Binocul Vis Strabismus Q 2002, 17:43–48.
Follens I, Evans PA, Tassignon MJ: Combined fourth and sixth cranial nerve palsy after lumbar puncture: a rare complication. Bull Soc Belge Ophthalmol 2001, 281:29–33.
Warner GT: Spontaneous intracranial hypotension causing a partial third cranial nerve palsy: a novel observation. Cephalalgia 2002, 22:822–823.
Ferrante E, Savino A, Briuschia A, et al.: Transient oculomotor cranial nerve palsy in spontaneous intracranial hypotension. J Neurosurg Sci 1998, 42:177–179.
Beck CE, Rizk NW, Kiger LT, et al.: Intracranial hypotension presenting with severe encephalopathy. Case report. J Neurosurg 1998, 89:470–473.
Pleasure SJ, Abosch A, Friedman J, et al.: Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology 1998, 50:1854–1857.
Evans RW, Mokri B: Spontaneous intracranial hypotension resulting in coma. Headache 2002, 42:159–160.
Hong M, Shah GV, Adams KM, et al.: Spontaneous intracranial hypotension causing reversible frontotemporal dementia. Neurology 2002, 58:1285–1287.
Pakiam AS, Lee C, Lang AE: Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol 1999, 56:869–872.
Yamamoto M, Suehiro T, Nakata H, et al.: Primary low cerebrospinal fluid pressure syndrome associated with galactorrhea. Intern Med 1993, 32:228–231.
Portier F, de Minteguiaga C, Racy E, et al.: Spontaneous intracranial hypotension: a rare cause of labyrinthine hydrops. Ann Otol Rhinol Laryngol 2002, 111:817–820.
Nowak DA, Radiek SO, Zinner J, et al.: Broadening of the clinical spectrum: unusual presentation of spontaneous cerebrospinal fluid hypovolemia. Case report. J Neurosurg 2003, 98:903–907.
Albayram S, Wasserman BA, Yousem DM, Wityk R: Intracranial hypotension as a cause of radiculopathy from cervical epidural venous engorgement. Case report. Am J Neuroradiol 2002, 23:618–621.
Schievink WI, Meyer FB, Atkinson JL, Mokri B: Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. J Neurosurg 1999, 84:598–605.
Mokri B, Parisi JE, Scheithauer BW, et al.: Meningeal biopsy in intracranial hypotension: meningeal enhancement on MRI. Neurology 1995, 45:1801–1807.
Benamer M, Tainturier C, Graveleau P, Peirot L: Radionuclide cisternography in spontaneous intracranial hypotension. Clin Nucl Med 1998, 23:150–151.
Bai J, Yokoyama K, Kinuya S, et al.: Radionuclide cisternography in intracranial hypotension syndrome. Ann Nucl Med 2002, 16:75–78.
Spelle L, Boulin A, Tainturier C, et al.: Neuroimaging features of spontaneous intracranial hypotension. Neuroradiology 2001, 43:622–627.
Lin WC, Lirng JF, Fuh JL, et al.: MR findings of spontaneous intracranial hypotension. Acta Radiol 2002, 43:249–255.
Atkinson JL, Weinshenker BG, Miller GM, et al.: Acquired Chiari I malformation secondary to spontaneous spinal cerebrospinal fluid leakage and chronic intracranial hypotension syndrome in seven cases. J Neurosurg 1988, 88:237–242.
Mokri B: Headache caused by decreased intracranial pressure: diagnosis and management. Curr Opin Neurol 2003, 16:319–326. An updated review of CSF leaks and associated headaches and other clinical manifestations as well as imaging abnormalities and treatments.
Aoki N, Sakai T, Oikawa K: Spontaneous intracranial hypotension associated with subdural hematoma: diagnostic usefulness of percutaneous subdural tapping and magnetic resonance imaging. Acta Neuro Chir 1998, 140:47–49.
Sato Y, Honda Y, Maruoka H, et al.: Recurrence of spontaneous intracranial hypotension with subdural hematomas. Cephalalgia 1997, 17:42–45.
de Noronha RJ, Sharrack B, Hadjivassiliou M, Romanowski CA: Subdural hematoma: a potentially serious consequence of spontaneous intracranial hypotension. J Neurol Neurosurg Psychiatry 2003, 74:752–755.
Augustin J, Proust F, Vendure L, et al.: Hematoma sous-dural chronique bilateral: une hypotension intracranienne spontanee [French]. Neurochirurgie 2003, 49:47–50.
Shimazun N, Oba H, Aoki S, et al.: Pituitary enlargement in spontaneous intracranial hypotension on MRI. Nippon Acta Radiologica 1998, 58:349–352.
Mokri B, Atkinson JL: False pituitary tumor in CSF leaks. Neurology 2000, 55:573–575.
Alvarez-Linera J, Escribano J, Benito-Leun J, et al.: Pituitary enlargement in patients with intracranial hypotension syndrome. Neurology 2000, 55:1895–1897. A study of pituitary enlargement in intracranial hypotension.
Bakshi R, Mechtler LL, Kamran S, et al.: MRI findings in lumbar puncture headache syndrome: abnormal duralmeningeal and dural venous sinus enhancement. Clin Imaging 1999, 23:73–76.
Roll JD, Larson TC, Soriano R: Cerebral angiographic findings in spontaneous intracranial hypotension. Am J Neuroradiol 2003, 24:707–708.
Moayeri NN, Henson JW, Schaefer PW, Zervas NT: Spinal dural enhancement on magnetic resonance imaging associated with spontaneous intracranial hypotension. J Neurosurg 1998, 88:912–918.
Rabin BM, Roychowdhury S, Meyer JR, et al.: Spontaneous intracranial hypotension: spinal MRI findings. Am J Neuroradiol 1998, 19:1034–1039.
Yousry I, Forderreuther S, Mariggl B, et al.: Cervical imaging in postural headache: MR signs and pathophysiologic implications. Am J Neuroradiol 2001, 22:1239–1250.
Chiapparini L, Farina L, D’Incerti L, et al.: Spinal radiological findings in nine patients with spontaneous intracranial hypotension. Neuroradiology 2002, 44:143–150.
Luetemer PH, Mokri B: Dynamic CT myelography: a novel technique for localizing high-flow spinal cerebrospinal fluid leaks. Am J Neuroradiol 2003, 24:1711–1714. This study introduces a new technique for detection of the site of CSF egress in high-flow leaks or leaks from multiple sites.
Mokri B: The Monro-Kellie hypothesis. Applications in CSF volume depletion. Neurology 2001, 56:1746–1748.
Mokri B: Cerebrospinal fluid volume depletion and its emerging clinical/imaging syndromes. Neurosurg Focus 2000, 9:(article 6).
Fay T: Mechanism of headache. Arch Neurol Psychiatry 1937, 37:471–474.
Rice GG, Dabbs CH: The use of peridural and subarachnoid injections of saline solution in the treatment of severe postspinal headaches. Anesthesiology 1950, 11:17–23.
Usubiaga JE, Usubiaga LE, Brea LM, Goyena R: Effect of saline injections on epidural and subarachnoid space pressures and relation to postspinal anesthesias headache. Anesth Analg 1967, 46:293–296.
Gibson BE, Wedel DJ, Faust RJ, Petersen RC: Continuous epidural saline infusion for the treatment of low CSF pressure headache. Anesthesiology 1988, 48:789–791.
Aldrete JA: Persistent post-dural-puncture headache treated with epidural infusion of Dextran. Headache 1994, 34:265–267.
Duffy PJ, Crosby ET: The epidural blood patch. Resolving the controversies. Can J Anesth 1999, 46:878–886.
Sencakova D, Mokri B, McClelland RL: The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology 2001, 57:1921–1923. A study addressing the efficacy of epidural blood patch in spontaneous CSF leaks.
Vilming ST, Titus F: Low cerebrospinal fluid pressure. In The Headache. Edited by Olefsen J, Tpelt-Hanson P, Welch KM. New York: Raven Press; 1993:687–695.
Crul BJ, Gerritse BM, Van Dongen RT, Schoonderwaldt HC: Epidural fibrin glue injection stops persistent postdural puncture headache. Anesthesiology 1999, 91:576–577.
Gerriste BM, Von Dongen RT, Crul BJ: Epidural fibrin glue injection stops persistent cerebrospinal fluid leak during long-term intrathecal catheterization. Anesth Analg 1997, 84:1140–1141.
Schievink WI, Morreale VM, Atkinson JL, et al.: Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg 1998, 88:243–246.
Maher CO, Meyer FR, Mokri B: Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. Neurosurg Focus 2000, 9:(article 7).
Mokri B: Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks. Mayo Clin Proc 2002, 77:1241–1246. This paper brings to attention the development of a rebound intracranial hypertension after treatment of intracranial hypotension from spontaneous CSF leaks.
Mokri B, Hunter SF, Atkinson JL, Piepgras DG: Orthostatic headaches caused by CSF leak but with normal CSF pressures. Neurology 1998, 51:786–790.
Mokri B, Atkinson JL, Dodick DW, et al.: Absent pachymeningeal gadolinium enhancement on cranial MRI despite symptomatic CSF leak. Neurology 1999, 53:402–404.
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Mokri, B. Spontaneous low cerebrospinal pressure/volume headaches. Curr Neurol Neurosci Rep 4, 117–124 (2004). https://doi.org/10.1007/s11910-004-0025-5
Issue Date:
DOI: https://doi.org/10.1007/s11910-004-0025-5