Abstract
Spontaneous intracranial hypotension (SIH) is typically manifested by orthostatic headaches that may be associated with one or more of several other symptoms, including pain or stiffness of the neck, nausea, emesis, horizontal diplopia, dizziness, change in hearing, visual blurring or visual field cuts, photophobia, interscapular pain, and occasionally face numbness or weakness or radicular upper-limb symptoms. Cerebrospinal fluid (CSF) pressures, by definition, are quite low. SIH almost invariably results from a spontaneous CSF leak. Only very infrequently is this leak at the skull base (cribriform plate). In the overwhelming majority of patients, the leak is at the level of the spine, particularly the thoracic spine and cervicothoracic junction. Sometimes, documented leaks and typical clinical and imaging findings of SIH are associated with CSF pressures that are consistently within limits of normal. Magnetic resonance imaging of the head typically shows diffuse pachymeningeal gadolinium enhancement, often with imaging evidence of sinking of the brain, and less frequently with subdural fluid collections, engorged cerebral venous sinuses, enlarged pituitary gland, or decreased size of the ventricles. Radioisotope cisternography typically shows absence of activity over the cerebral convexities, even at 24 or 48 hours, and early appearance of activity in the kidneys and urinary bladder, and may sometimes reveal the level of the leak. Although various treatment modalities have been implemented, epidural blood patch is probably the treatment of choice in patients who have failed an initial trial of conservative management. When adequate trials of epidural blood patches fail, surgery can offer encouraging results in selected cases in which the site of the leak has been identified. Some of the spontaneous CSF leaks are related to weakness of the meningeal sac, likely in connection with a connective tissue abnormality.
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References and Recommended Reading
Mokri B, Krueger BR, Miller GM, Peipgras DG: Meningeal gadolinium enhancement in low pressure headaches [abstract]. Ann Neurol 1991, 30:294–295.
Mokri B: Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia—evolution of a concept. Mayo Clin Proc 1999, 74:1113–1123. A comprehensive review of the subject including historical aspects, clinical findings, and cerebrospinal fluid (CSF) and imaging abnormalities. The emerging clinical-imaging syndromes related to CSF leaks or volume loss are addressed along with illustrative cases.
Dillon WP, Fishman RA: Some lessons learned about the diagnosis and treatment of spontaneous intracranial hypotension [editorial]. Am J Neuroradiol 1998, 9:1001–1002. An informative editorial by two experienced experts in the field containing interesting pearls and keen observations.
Silberstein SD, Marcelis J: Headache associated with changes in intracranial pressure. Headache 1992, 32:84.
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.
Labadie EL, van AntwerpJ, Bamford CR: Abnormal lumbar isotrope cisternography in an unusual case of spontaneous hypoliquorrheic headache. Neurology 1976, 26:135–139.
Molins A, Alvarez J, Sumalla J, et al.: Cisternographic pattern of spontaneous liquoral hypotension. Cephalalgia 1990, 10:5–65.
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 Neruosurg 1991, 93:55–60.
Fishman RA, Dillon WP: Dural enhancement and cerebral displacement secondary to intracranial hypotension. Neurology 1993, 43:609–611.
Hochman MS, Naidich TP, Kobetz SA, Fernandez-Maitin A: Spontaneous intracranial hypotension with pachymeningeal enhancement on MRI. Neurology 1992, 42:1628–1630.
Pannullo SC, Reich JB, Krol G, et al.: MRI changes in intracranial hypotension. Neurology 1993, 43:919–926.
Sable SG, Ramadan NM: Meningeal enhancement and low CSF pressure headache: an MRI study. Cephalalgia 1991, 11:275–276.
Davenport RJ, Chataway SJ, Warlow CP: Spontaneous intracranial hypotension from a CSF leak in a patient with Marfan’s syndrome. J Neurol Neurosurg Psychiatry 1995, 59:516–519.
Mokri B, Piepgras DG, Miller GM: Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc 1997, 72:400–413. Study of 26 consecutive patients with orthostatic headaches and diffuse pacymeningeal enhancement resulting from cerebrospinal fluid (CSF) leak or CSF shunt overdrainage. Detailed review of clinical manifestations, CSF, and magnetic resonance imaging abnormalities, as well as cisternographic and myelographic findings.
Fattori R, Nienaber CA, Descovich B, et al.: Importance of dural ectasia in phenotypic assessment of Marfan’s syndrome. Lancet 1999, 354:910–913.
Fukutake T, Sakakibara R, Mori M, et al.: Chronic intractable headache in a patient with Marfan’s syndrome. Headache 1997, 37:291–295.
Schievink WI, Torres VE: Spinal meningeal diverticula in autosomal dominant polycystic kidney disease. Lancet 1997, 349:1223–1224.
Vishteh AG, Schievink WI, Baskin JJ, Sonntag VK: Cervical bony spur presenting with spontaneous intracranial hypotension. Case report. J Neurosurg 1998, 89:483–484.
Horton JC, Fishman RA: Neurovisual findings in the syndrome of spontaneous intracranial hypotension from dural cerebrospinal fluid leak. Ophthalmology 1994, 101:244–251.
Yamamoto M, Suehiro T, Nakata H, et al.: Primary low cerebrospinal fluid pressure syndrome associated with galactorrhea. Intern Med 1993, 32:228–231.
Pleasure SJ, Abosch A, Friedman J, et al.: Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology 1998, 50:1854–1857.
Pakiam AS, Lee C, Lang AE: Intracranial hypotension with parkinsonism, ataxia, and bulbar weakness. Arch Neurol 1999, 56:869–872.
Beck CE, Rizk NW, Kiger LT, et al.: Intracranial hypotension presenting with severe encephalopathy. Case report. J Neurosurg 1998, 89:470–473.
Fay T: Mechanism of headache. Arch Neurol Psychiatry 1937, 37:471–474.
Mokri B, Hunter SF, Atkinson JLD, Piepgras DG: Orthostatic headaches caused by CSF leak but with normal CSF pressures. Neurology 1998, 51:786–790.
Mokri B, Parisi JE, Scheithauer BW, et al.: Meningeal biopsy in intracranial hypotension: meningeal enhancement on MRI. Neurology 1995, 45:1801–1807.
Pavlin DJ, McDonald JS, Child B, Rusch V: Acute subdural hematoma—an unusual sequela to lumbar puncture. Anesthesiology 1979, 51:338–340.
Sipe JC, Zyroff J, Waltz TA: Primary intracranial hypotension and bilateral isodense subdural hematomas. Neurology 1981, 31:334–337.
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 1998, 88:237–242.
Mokri B, Atkinson JLD: False pituitary tumor in spontaneous cerebrospinal fluid leak [abstract]. Ann Neurol 1999, 46:475.
Bakshi R, Mechtler LL, Kamran S, et al.: MRI findings in lumbar puncture headache syndrome: abnormal dural-meningeal and dural venous sinus enhancement. Clin Imag 1999, 23:73–76.
Rabin BM, Roychowdhury S, Meyer JR, et al.: Spontaneous intracranial hypotension: spinal MRI findings. Am J Neuroradiol 1998, 19:1034–1039.
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.
Haines DE, Harkey HL, Al-Mefty O: The “subdural” space: a new look at an outdated concept. Neurosurgery 1993, 32:111–120.
Tourtellotte WW, Haerer AF, Heller GL, Somers JE: Post-lumbar puncture headaches. Springfield, IL: Charles C Thomas Publisher, 1964.
Grant R, Condon B, Patterson J, et al.: Changes in cranial CSF volume during hypercapnia and hypocapnia. J Neurol Neurosurg Psychiatry 1989, 52:218–222.
Rice GG, Dabbs CH: The use of peridural and subarachnoid injections of saline solution in the treatment of severe postspinal headaches. Anesthesoiology 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 anesthesia 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, 68:789–791.
Gormley JB: Current comment: treatment of postspinal headache. Anesthesiology 1960, 21:565–566.
DiGiovanni AJ, Galbert MW, Wahle WM: Epidural injection of autologous blood for postlumbar-puncture headache. II. Additional clinical experiences and laboratory investigation. Anesth Analg 1972, 51:226–232.
Crawford JS: Epidural blood patch. Anaesthesia 1985, 40:381.
Rosenberg PH, Heavner JE: In vitro study of the effect of epidural blood patch on leakage through a dural puncture. Anesth Analg 1985, 64:501–504.
Szeinfeld M, Ihmeidan IH, Moser MM, et al.: Epidural blood patch: evaluation of the volume and spread of blood injected into the epidural space. Anesthesiology 1986, 64:820–822.
Seebacher J, Ribeiro V, LeGuillou JL, et al.: Epidural blood patch in the treatment of post-dural puncture headache: a double blind study. Headache 1989, 29:630–632.
Vakharia SB, Thomas PS, Rosenbaum AE, et al.: Magnetic resonance imaging of cerebrospinal fluid leak and tamponade effect of blood patch in post-dural puncture headache. Anesth Analg 1997, 84:585–590.
Schievink WI, Morreale VM, Atkinson JL, et al.: Surgical treatment of spontaneous spinal cerebrospinal fluid leaks. J Neurosurg 1998, 88:243–246. This article addresses the surgical procedures and observations in 10 patients with cerebrospinal fluid leaks who had been treated surgically.
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, Van Dongen RT, Crul BJP: Epidural fibrin glue injection stops persistent cerebrospinal fluid leak during long-term intrathecal catheterization. Anesth Analg 1997, 84:1140–1141.
Mokri B, Atkinson JL, Piepgras DG: Absent headache despite CSF volume depletion (intracranial hypotension). Neurology 2000, 55:573–575.
Mokri B, Atkinson JL, Dodick DW, et al.: Absent pachymeningeal gadolinium enhancement on cranial MRI despite symptomatic CSF leak. Neurology 1999, 53:402–404.
Mokri B: Cerebrospinal fluid volume depletion and its emerging clinical/imaging syndromes. Neurosurg Focus 2000, 9:Article 6.
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Mokri, B. Spontaneous intracranial hypotension. Curr Neurol Neurosci Rep 1, 109–117 (2001). https://doi.org/10.1007/s11910-001-0005-y
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DOI: https://doi.org/10.1007/s11910-001-0005-y