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Early epidural blood patch in spontaneous intracranial hypotension

Neurology, 2004
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Early epidural blood patch in spontaneous intracranial hypotension S. Berroir, MD; B. Loisel, MD; A. Ducros, MD; M. Boukobza, MD; C. Tzourio, MD; D. Valade, MD; and M-G. Bousser, MD Abstract—Thirty patients with a typical orthostatic headache were treated by early lumbar epidural blood patch (EBP) without previously performing lumbar puncture or identifying a CSF leak and with or without typical MRI changes. A complete cure was obtained in 77% of patients after one (57%) or two (20%) EBPs. Spontaneous intracranial hypotension with typical orthostatic headache can be diagnosed without lumbar puncture and can be cured by early EBP in a majority of patients. NEUROLOGY 2004;63:1950 –1951 Spontaneous intracranial hypotension (SIH) is an uncommon disabling condition occurring in the ab- sence of an obvious dural tear. Its prominent clinical feature is orthostatic headache, frequently associ- ated with neck pain, nausea, vomiting, diplopia, blurred vision, and distorted hearing. 1-3 MRI abnor- malities include diffuse pachymeningeal gadolinium enhancement (PMGE), subdural hematomas or hy- gromas, and downward displacement of the cranial contents. 1-4 The role of lumbar puncture (LP) to demonstrate low CSF pressure and the timing of further investi- gations to identify a leak are debated particularly because they require a dural puncture that may worsen the patient’s condition. Epidural blood patch (EBP) is the most effective treatment, 2,3,5 but its tim- ing is also debated. We report a consecutive series of 30 patients with SIH and severe orthostatic head- ache treated with early lumbar EBP, even in the absence of typical MRI changes, without previously performing LP or looking for a leak. Methods. Patients were included if they had a typical SIH de- fined as a severe purely orthostatic headache in the absence of obvious causes of dural tear. Headache was defined as severe when it interfered with daily activities and as purely orthostatic when it occurred in 15 minutes in the upright position and disappeared in 15 minutes with recumbency. Brain MRI was performed using a 1.5-T system with unen- hanced T1- and T2-weighted imaging and gadolinium-enhanced T1-weighted imaging in the sagittal and coronal planes. Once SIH diagnosis was established and after full informed consent was obtained, a first EBP was done, followed by a second in case of failure or relapse. After the failure of two to four EBPs, CSF leak was looked for by MRI, CT myelography, and/or radio- isotope cisternography. The same anesthetist performed all EBPs under strict aseptic conditions in an operating room. Up to 40 mL of the patients’ own blood was slowly injected in L3-L4 or L4-L5 spaces and only was stopped in case of severe lumbar pain. The patient remained su- pine for 2 hours and was asked to refrain from strenuous exercise for 3 weeks. Follow-up evaluation was performed at 1 month and yearly thereafter or more frequently if necessary. The duration of the follow-up period was 1 to 4 years. Results. Baseline characteristics. From July 1999 to July 2002, 33 patients (21 women, 12 men; aged 15 to 68 years; mean, 40 years) were consecutively seen with SIH and severe purely orthostatic headache; of these, 21 were newly diagnosed in our Emergency Headache Center. Mean time from onset to diagnosis was 20 15 days. Ten patients reported physical effort as a triggering factor. Four patients had headache exacerbation when coughing or on exertion. Other symptoms included nausea and/or vomiting in 23 patients (70%), neck pain in 16 (48%), hear- ing disturbances in 14 (42%), back pain in 3, and horizon- tal diplopia and drowsiness in 1. Brain MRI (31 patients) showed diffuse PMGE in 19 patients (61%), a sagging brain and subdural collections in 11 (35%), and an isolated sagging brain in 1. MRI was normal in 10 patients (32%). Treatment and outcome. Three patients did not re- ceive EBP because their headache changed rapidly during evaluation. One improved spontaneously in a few days, and two others had sinus thrombosis and were treated with heparin. 6 Among the 30 patients who underwent a lumbar EBP, 27 patients (90%) had immediate relief (90% on a verbal analog scale 0 to 10), and 3 did not improve. No complication was observed. Among the 27 with immediate relief, 17 remained pain free at the end of follow-up period, 1 was lost to follow-up evaluation, and 9 had a relapse within a few days to several weeks. In these 9 patients, the second EBP was followed with immediate and sustained relief in 6 (20%); therefore, 23 (77%) pa- tients were pain free after one or two EBPs (figure). Three patients improved after the second EBP but re- lapsed. Investigations showed a leak at the T9, C7, and T8 levels. One patient was cured after surgery, and another one was cured after five EBPs, including two at the site of the leak. The third patient has a disc herniation at the T8 level for which surgery is still debated. From the Service de Neurologie (Drs. Berroir, Tzourio, and Bousser), Département d’anesthésie reanimation (Dr. Loisel), Centre d’urgence céphalées (Drs. Ducros and Valade), and Service de neuroradiologie (Dr. Boukobza), Lariboisière Hospital, Paris, France. Received April 20, 2004. Accepted in final form July 2, 2004. Address correspondence and reprint requests to Dr. Marie-Germaine Bousser, Service de Neurologie, Hôpital Lariboisière, 2 rue Ambroise Paré, 75475 Paris cedex 10, France; e-mail: mg.bousser@lrb.ap-hop-paris.fr 1950 Copyright © 2004 by AAN Enterprises, Inc.
Discussion. Thirty patients with SIH and severe purely orthostatic headache received early lumbar EBP, performed whenever the typical headache per- sisted after the end of the clinical and MRI evalua- tion period; 23 (77%) were cured after one (57%) or two (20%) EBPs, with a follow-up period of 1 to 4 years. The fact that these 33 patients were recruited during a 3-year period suggests that SIH occurs more frequently than classically thought, particu- larly because the present series, based on a severe purely orthostatic headache, excluded unusual pre- sentations of SIH, such as nonpositional, 7 exer- tional, 8 or even absent headache. 9 Baseline characteristics of our patients are essen- tially similar to those reported in other large series: 1,9 female preponderance, mean age of ~40 years, physical effort as triggering factor, and frequent as- sociated nausea, neck pain, tinnitus, or hearing dis- turbances. The lower rate of typical MRI changes (68%) compared with other series (80% 1,3,10 ) may be because of the greater number of recent cases. There is no consensus regarding the management of SIH. In mild forms, conservative measures are usually sufficient. In severe cases, such as ours, there is little debate about the indication of EBP 2,3,5 when PMGE is present on MRI, 5 but when MRI is normal, it is usually recommended to proceed with additional diagnostic studies. 1,5 However, because these studies imply a dural puncture that may worsen the patient’s condition, we choose to first per- form one or two EBPs even in patients with normal MRI and to postpone additional investigations. The overall success rate after one or two lumbar EBPs (77%) is less than the 90% observed in post- lumbar puncture headache, probably because the leaks, when present, are mostly thoracic and thus distant from the EBP level. Our 77% success rate is higher than the 56% observed in a Mayo Clinic series of 25 patients, possibly because their patients were more severe cases, had a documented CSF leak (im- plying a dural puncture), and received a smaller quantity of blood (10 to 20 mL) in contrast to 20 to 40 mL in our series. References 1. Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc 1997;72:400 – 413. 2. Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypo- tension to cerebrospinal fluid hypovolemia-evolution of a concept. Mayo Clin Proc 1999;74:1113–1123. 3. Mokri B, Posner JB. Spontaneous intracranial hypotension. The broad- ening spectrum of CSF leaks. Neurology 2000;55:1771–1772. 4. Brightbill TC, Goodwin RS, Ford RG. Magnetic resonance imaging of intracranial hypotension syndrome with pathophysiological correlation. Headache 2000;40:292–299. 5. Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood patch in CSF leaks. Neurology 2001;57:1921–1923. 6. Berroir S, Grabli D, Héran F, Bakouche P, Bousser MG. Cerebral sinus venous thrombosis in two patients with spontaneous intracranial hypo- tension. Cerebrovasc Dis 2004;17:9 –12. 7. Schievink WI, Smith KA. Nonpositional headache caused by spontane- ous intracranial hypotension. Neurology 1998;51:1768 –1769. 8. Mokri B. Spontaneous CSF leaks mimicking benign exertional head- aches. Cephalalgia 2002;22:780 –783. 9. Mokri B, Atkinson JLD, Piepgras DG. Absent headache despite CSF volume depletion (intracranial hypotension). Neurology 2000;55:1722– 1724. 10. Chung SJ, Kim JS, Lee M. Syndrome of cerebral fluid hypovolemia. Clinical and imaging features and outcome. Neurology 2000;55:1321– 1327. Figure. Flow chart of 30 patients with spontaneous intra- cranial hypotension treated using epidural blood patch. November (2 of 2) 2004 NEUROLOGY 63 1951
Early epidural blood patch in spontaneous intracranial hypotension S. Berroir, MD; B. Loisel, MD; A. Ducros, MD; M. Boukobza, MD; C. Tzourio, MD; D. Valade, MD; and M-G. Bousser, MD Abstract—Thirty patients with a typical orthostatic headache were treated by early lumbar epidural blood patch (EBP) without previously performing lumbar puncture or identifying a CSF leak and with or without typical MRI changes. A complete cure was obtained in 77% of patients after one (57%) or two (20%) EBPs. Spontaneous intracranial hypotension with typical orthostatic headache can be diagnosed without lumbar puncture and can be cured by early EBP in a majority of patients. NEUROLOGY 2004;63:1950 –1951 Spontaneous intracranial hypotension (SIH) is an uncommon disabling condition occurring in the absence of an obvious dural tear. Its prominent clinical feature is orthostatic headache, frequently associated with neck pain, nausea, vomiting, diplopia, blurred vision, and distorted hearing.1-3 MRI abnormalities include diffuse pachymeningeal gadolinium enhancement (PMGE), subdural hematomas or hygromas, and downward displacement of the cranial contents.1-4 The role of lumbar puncture (LP) to demonstrate low CSF pressure and the timing of further investigations to identify a leak are debated particularly because they require a dural puncture that may worsen the patient’s condition. Epidural blood patch (EBP) is the most effective treatment,2,3,5 but its timing is also debated. We report a consecutive series of 30 patients with SIH and severe orthostatic headache treated with early lumbar EBP, even in the absence of typical MRI changes, without previously performing LP or looking for a leak. Methods. Patients were included if they had a typical SIH defined as a severe purely orthostatic headache in the absence of obvious causes of dural tear. Headache was defined as severe when it interfered with daily activities and as purely orthostatic when it occurred in ⬍15 minutes in the upright position and disappeared in ⬍15 minutes with recumbency. Brain MRI was performed using a 1.5-T system with unenhanced T1- and T2-weighted imaging and gadolinium-enhanced T1-weighted imaging in the sagittal and coronal planes. Once SIH diagnosis was established and after full informed consent was obtained, a first EBP was done, followed by a second in case of failure or relapse. After the failure of two to four EBPs, CSF leak was looked for by MRI, CT myelography, and/or radioisotope cisternography. The same anesthetist performed all EBPs under strict aseptic conditions in an operating room. Up to 40 mL of the patients’ own blood was slowly injected in L3-L4 or L4-L5 spaces and only was stopped in case of severe lumbar pain. The patient remained supine for 2 hours and was asked to refrain from strenuous exercise for 3 weeks. Follow-up evaluation was performed at 1 month and yearly thereafter or more frequently if necessary. The duration of the follow-up period was 1 to 4 years. Results. Baseline characteristics. From July 1999 to July 2002, 33 patients (21 women, 12 men; aged 15 to 68 years; mean, 40 years) were consecutively seen with SIH and severe purely orthostatic headache; of these, 21 were newly diagnosed in our Emergency Headache Center. Mean time from onset to diagnosis was 20 ⫾ 15 days. Ten patients reported physical effort as a triggering factor. Four patients had headache exacerbation when coughing or on exertion. Other symptoms included nausea and/or vomiting in 23 patients (70%), neck pain in 16 (48%), hearing disturbances in 14 (42%), back pain in 3, and horizontal diplopia and drowsiness in 1. Brain MRI (31 patients) showed diffuse PMGE in 19 patients (61%), a sagging brain and subdural collections in 11 (35%), and an isolated sagging brain in 1. MRI was normal in 10 patients (32%). Treatment and outcome. Three patients did not receive EBP because their headache changed rapidly during evaluation. One improved spontaneously in a few days, and two others had sinus thrombosis and were treated with heparin.6 Among the 30 patients who underwent a lumbar EBP, 27 patients (90%) had immediate relief (⬎90% on a verbal analog scale 0 to 10), and 3 did not improve. No complication was observed. Among the 27 with immediate relief, 17 remained pain free at the end of follow-up period, 1 was lost to follow-up evaluation, and 9 had a relapse within a few days to several weeks. In these 9 patients, the second EBP was followed with immediate and sustained relief in 6 (20%); therefore, 23 (77%) patients were pain free after one or two EBPs (figure). Three patients improved after the second EBP but relapsed. Investigations showed a leak at the T9, C7, and T8 levels. One patient was cured after surgery, and another one was cured after five EBPs, including two at the site of the leak. The third patient has a disc herniation at the T8 level for which surgery is still debated. From the Service de Neurologie (Drs. Berroir, Tzourio, and Bousser), Département d’anesthésie reanimation (Dr. Loisel), Centre d’urgence céphalées (Drs. Ducros and Valade), and Service de neuroradiologie (Dr. Boukobza), Lariboisière Hospital, Paris, France. Received April 20, 2004. Accepted in final form July 2, 2004. Address correspondence and reprint requests to Dr. Marie-Germaine Bousser, Service de Neurologie, Hôpital Lariboisière, 2 rue Ambroise Paré, 75475 Paris cedex 10, France; e-mail: mg.bousser@lrb.ap-hop-paris.fr 1950 Copyright © 2004 by AAN Enterprises, Inc. sentations of SIH, such as nonpositional,7 exertional,8 or even absent headache.9 Baseline characteristics of our patients are essentially similar to those reported in other large series: 1,9 female preponderance, mean age of ~40 years, physical effort as triggering factor, and frequent associated nausea, neck pain, tinnitus, or hearing disturbances. The lower rate of typical MRI changes (68%) compared with other series (⬎80%1,3,10) may be because of the greater number of recent cases. There is no consensus regarding the management of SIH. In mild forms, conservative measures are usually sufficient. In severe cases, such as ours, there is little debate about the indication of EBP2,3,5 when PMGE is present on MRI,5 but when MRI is normal, it is usually recommended to proceed with additional diagnostic studies.1,5 However, because these studies imply a dural puncture that may worsen the patient’s condition, we choose to first perform one or two EBPs even in patients with normal MRI and to postpone additional investigations. The overall success rate after one or two lumbar EBPs (77%) is less than the 90% observed in postlumbar puncture headache, probably because the leaks, when present, are mostly thoracic and thus distant from the EBP level. Our 77% success rate is higher than the 56% observed in a Mayo Clinic series of 25 patients, possibly because their patients were more severe cases, had a documented CSF leak (implying a dural puncture), and received a smaller quantity of blood (10 to 20 mL) in contrast to 20 to 40 mL in our series. References Figure. Flow chart of 30 patients with spontaneous intracranial hypotension treated using epidural blood patch. Discussion. Thirty patients with SIH and severe purely orthostatic headache received early lumbar EBP, performed whenever the typical headache persisted after the end of the clinical and MRI evaluation period; 23 (77%) were cured after one (57%) or two (20%) EBPs, with a follow-up period of 1 to 4 years. The fact that these 33 patients were recruited during a 3-year period suggests that SIH occurs more frequently than classically thought, particularly because the present series, based on a severe purely orthostatic headache, excluded unusual pre- 1. Mokri B, Piepgras DG, Miller GM. Syndrome of orthostatic headaches and diffuse pachymeningeal gadolinium enhancement. Mayo Clin Proc 1997;72:400 – 413. 2. Mokri B. Spontaneous cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia-evolution of a concept. Mayo Clin Proc 1999;74:1113–1123. 3. Mokri B, Posner JB. Spontaneous intracranial hypotension. The broadening spectrum of CSF leaks. Neurology 2000;55:1771–1772. 4. Brightbill TC, Goodwin RS, Ford RG. Magnetic resonance imaging of intracranial hypotension syndrome with pathophysiological correlation. Headache 2000;40:292–299. 5. Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood patch in CSF leaks. Neurology 2001;57:1921–1923. 6. Berroir S, Grabli D, Héran F, Bakouche P, Bousser MG. Cerebral sinus venous thrombosis in two patients with spontaneous intracranial hypotension. Cerebrovasc Dis 2004;17:9 –12. 7. Schievink WI, Smith KA. Nonpositional headache caused by spontaneous intracranial hypotension. Neurology 1998;51:1768 –1769. 8. Mokri B. Spontaneous CSF leaks mimicking benign exertional headaches. Cephalalgia 2002;22:780 –783. 9. Mokri B, Atkinson JLD, Piepgras DG. Absent headache despite CSF volume depletion (intracranial hypotension). Neurology 2000;55:1722– 1724. 10. Chung SJ, Kim JS, Lee M. Syndrome of cerebral fluid hypovolemia. Clinical and imaging features and outcome. Neurology 2000;55:1321– 1327. November (2 of 2) 2004 NEUROLOGY 63 1951 Early epidural blood patch in spontaneous intracranial hypotension S. Berroir, B. Loisel, A. Ducros, et al. Neurology 2004;63;1950-1951 DOI 10.1212/01.WNL.0000144339.34733.E9 This information is current as of November 22, 2004 Updated Information & Services including high resolution figures, can be found at: http://www.neurology.org/content/63/10/1950.full.html References This article cites 10 articles, 6 of which you can access for free at: http://www.neurology.org/content/63/10/1950.full.html##ref-list-1 Citations This article has been cited by 13 HighWire-hosted articles: http://www.neurology.org/content/63/10/1950.full.html##otherarticles Subspecialty Collections This article, along with others on similar topics, appears in the following collection(s): All Clinical trials http://www.neurology.org//cgi/collection/all_clinical_trials Clinical trials Observational study (Cohort, Case control) http://www.neurology.org//cgi/collection/clinical_trials_observational_ study_cohort_case_control Low pressure syndrome http://www.neurology.org//cgi/collection/low_pressure_syndrome Permissions & Licensing Information about reproducing this article in parts (figures,tables) or in its entirety can be found online at: http://www.neurology.org/misc/about.xhtml#permissions Reprints Information about ordering reprints can be found online: http://www.neurology.org/misc/addir.xhtml#reprintsus Neurology ® is the official journal of the American Academy of Neurology. Published continuously since 1951, it is now a weekly with 48 issues per year. Copyright . All rights reserved. Print ISSN: 0028-3878. Online ISSN: 1526-632X.
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