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1 This article appeared in a journal published by Elsevier. The attached copy is furnished to the author for internal non-commercial research and education use, including for instruction at the authors institution and sharing with colleagues. Other uses, including reproduction and distribution, or selling or licensing copies, or posting to personal, institutional or third party websites are prohibited. In most cases authors are permitted to post their version of the article (e.g. in Word or Tex form) to their personal website or institutional repository. Authors requiring further information regarding Elsevier s archiving and manuscript policies are encouraged to visit:

Clinical Neurology and Neurosurgery 111 (2009) 699 702 Contents lists available at ScienceDirect Clinical Neurology and Neurosurgery journal homepage: www.elsevier.

Ferrante a,, Ines Arpino b, Alberto Citterio a, Anna Savino c a Department of Neurosciences, Niguarda Cà Granda Hospital, Piazza Ospedale Maggiore 3, 20162 Milan, Italy b Department of

2 Clinical Neurology and Neurosurgery 111 (2009) Contents lists available at ScienceDirect Clinical Neurology and Neurosurgery journal homepage: Case report Coma resulting from spontaneous intracranial hypotension treated with the epidural blood patch in the Trendelenburg position pre-medicated with acetazolamide Enrico Ferrante a,, Ines Arpino b, Alberto Citterio a, Anna Savino c a Department of Neurosciences, Niguarda Cà Granda Hospital, Piazza Ospedale Maggiore 3, Milan, Italy b Department of Anesthesiology, Niguarda Cà Granda Hospital, Milan, Italy c Department of Emergency Room, Niguarda Cà Granda Hospital, Milan, Italy article info abstract Article history: Received 30 July 2008 Received in revised form 31 May 2009 Accepted 2 June 2009 Available online 3 July 2009 Keywords: Headache Spontaneous intracranial hypotension Subdural haematoma Coma Epidural blood patch Acetazolamide A 62-year-old man had a new onset of severe, orthostatic headache which eventually progressed to a stupor and a coma 3 weeks later. A computed tomography (CT) scan showed bilateral chronic subdural haematoma and magnetic resonance imaging (MRI) of the brain showed the typical findings of spontaneous intracranial hypotension (SIH). After pre-medication with acetazolamide, he was treated with three lumbar autologous epidural blood patches (EBPs) and kept in the Trendelenburg position, with full recovery. The first lumbar autologous EBP was ineffective and the second was only partially effective because of incorrect execution of the procedure as shown by spinal neuroimaging examination post- EBP. A spinal neuroimaging examination post-ebp is therefore to be recommended in order to confirm the correct execution of procedure. Pre-medication with acetazolamide and keeping the patient in the Trendelenburg position could reduce the flow of spinal cerebrospinal (CSF) leak favouring sealing of the hole Elsevier B.V. All rights reserved. 1. Introduction 2. Case report Spontaneous intracranial hypotension (SIH) is characterized by orthostatic headaches, low CSF pressure and distinct abnormalities on MRI [1,2]. It generally results from spinal spontaneous CSF leakage, sometimes associated with underlying connective tissue disorders [3]. Treatment ranges from conservative management, such as bed rest, over-hydration and caffeine, to invasive procedures, such as the autologous epidural blood patch (EBP) [4 6], CT-guided fibrin glue injection at the site of the leak [7], and open surgical intervention. EBP has emerged as the treatment of choice for SIH when initial conservative measures fail to bring relief. Sencakova et al. [4] reported 25 cases of SIH treated with EBP, and found that 36% of all patients recovered with one EBP. Berroir et al. [5] observed a 57% success rate in a series of 30 patients with SIH after one EBP. We described 32 patients with SIH and observed a 90% success rate after one EBP [8]. SIH may rarely cause coma, and in these cases the choice of treatment is very challenging [9]. We report a patient with coma from SIH who recovered following repeated lumbar autologous EBPs pre-medicated with acetazolamide. Corresponding author. Tel.: ; fax: address: enricoferrante@libero.it (E. Ferrante). A 62-year-old man, treated with warfarin for a mechanic aortic valve prosthesis, developed severe, diffuse orthostatic headache with nausea and vomiting. There was no history of connective tissue disorders, alcoholism or trauma. After 15 days he became progressively obtunded. Laboratory test results showed INR of 2.3 (normal ). This value was within therapeutic levels, and there were no metabolic disorders. Haematological and biochemical laboratory tests, monitoring of thyroid hormones and inflammation rate were normal. He was admitted to the neurosurgical department. Brain CT revealed small bilateral chronic subdural haematomas (SH), increased attenuation in the sylvian fissures (Fig. 1). Cerebral angiography was normal. He underwent an evacuation of bilateral chronic SH because they were erroneously considered by neurosurgeons to be the cause of obtunding. Two days later the patient became comatose, the Glasgow coma scale score (GCSS) was 5 (eye opening response 1, motor response 3, verbal response 1). He showed respiratory distress and was intubated for 10 h. The patient again developed two episodes of respiratory distress with intubation the third and sixth days after the operation. Afterwards the neurology specialist suspected SIH, advised cerebral RMN with gadolinium. Brain MRI, 12 days after operation, showed diffuse pachymeningeal enhancement and brain sagging (Fig. 2A). These features /$ see front matter 2009 Elsevier B.V. All rights reserved. doi: /j.clineuro

700 E. Ferrante et al. / Clinical Neurology and Neurosurgery 111 (2009) 699 702 Fig. 1. Axial CT scan without contrast shows small chronic subdural haematomas interesting both frontal regions (white arrows).

A multislice spiral spinal CT post-lumbar autologous EBP showed small quantity of air in the epidural space from L5 to D11 (Fig.

3 700 E. Ferrante et al. / Clinical Neurology and Neurosurgery 111 (2009) Fig. 1. Axial CT scan without contrast shows small chronic subdural haematomas interesting both frontal regions (white arrows). blood mixed with 5 ml of iopamidol with patient lying in prone position. A multislice spiral spinal CT post-lumbar autologous EBP showed small quantity of air in the epidural space from L5 to D11 (Fig. 3B) and blood mixed with contrast medium was localized only in paraspinal musculature. The patient progressively improved over the next 15 days. He then again became progressively stuporous, asthenic, and was affected by disphagia. We performed another lumbar autologous EBP at L2 L3 level under fluoroscopy guidance (27 days after the second EBP) with 30 ml of blood mixed with 5 ml of iopamidol. Spinal CT post-ebp showed blood in epidural space from L1 to C7 D1 level (Fig. 3C). After 24 h the headache disappeared and he improved. He had rapid recovery of function soon after the third EBP. Oral anti-coagulant therapy with warfarin was stopped until INR was normal and before EBP treatment. Meantime enoxeparine 0.4 ml was administrated subcutaneously, twice per day. Warfarin treatment was resumed after EBPs. The patient was treated with a pre-medication with acetazolamide at dosage of 500 mg 12 h before giving lumbar autologous EBPs. He was kept in a 30 Trendelenburg position since an hour before the procedure, during the procedure and 24 h after the EBPs. Bilateral chronic SH were resolved completely after the first surgery. Brain MRI was normal after 4 months (Fig. 2B). After 24 months of follow-up the patient was in good health, he had no clinical sequelae and limitations related to the episode. He was fully functional, he was on warfarin and he had no recurrences. were considered to be consistent with SIH and severe resultant diencephalic compression. Spinal and myelo-mri failed to prove a CSF leak. He was placed in the Trendelenburg position at about 30 and awoke within 8 h, GCSS 15 [E4 M6 V5]. The next day the patient underwent a lumbar autologous EBP with 35 ml of blood mixed with 1 ml of gadolinium. The EBP was performed at L4 L5 level with a 16-g Tuohy needle via a midline approach with the patient lying on his left side. The epidural space was identified with the loss of resistance technique using air. The injection was stopped when the patient reported an increased pressure sensation in the neck. Spinal MRI post-lumbar autologous EBP showed that only a small quantity of blood went into epidural space at L5 S1 level whereas considerable blood was localized into paraspinal musculature (Fig. 3A). Twenty-four hours after lumbar autologous EBP, when the patient was in anti-trendelenburg position at about 45, he again became stuporous and inattentive. Another lumbar autologous EBP at L4 L5 level was therefore given under fluoroscopy guidance with 30 ml of 3. Discussion In this case bilateral chronic SH were caused by SIH by tearing of bridging veins resulting from the downward displacement of the brain, the coma by severe sagging of the brain leading to diencephalic deformation and dysphagia by bulbar weakness to deep midline structure [10]. SH could been attributed solely to the warfarin use and the obtunding, incorrectly, to the SH, but the INR on therapeutic range and the positional feature of the headache suggested the diagnosis of SIH. The SH in SIH are frequently small and may be asymptomatic, but sometimes become large and symptomatic, cause notable mass effect and shift the midline structures. Readers may wonder whether the fact that the patient was on warfarin may have contributed to the bleed and although this must always be a consideration, we do not think this was a contributing factor in this case. In fact, we had previously observed six patients with SIH complicated with bilateral chronic SH with mass effect Fig. 2. Sagittal T1-weighted brain MRI with gadolinium showing diffuse pachymeningeal enhancement (black arrows), pituitary gland enlargement (white arrow), flattening of the pons with partial obliteration of the basilar cisterns (black open arrowheads), descent of the hypothalamic structures (asterisk) (A); sagittal T1-weighted brain MRI with contrast acquired 4 months after the EBP procedure showing resolution of the previously described signs of SIH (B).

E. Ferrante et al. / Clinical Neurology and Neurosurgery 111 (2009) 699 702 701 Fig. 3.

4 E. Ferrante et al. / Clinical Neurology and Neurosurgery 111 (2009) Fig. 3. Sagittal T1-weighted spinal MRI with fat suppression, after the first EBP showing presence of a small amount of blood mixed with gadolinium inside the epidural space (white arrow) and of large amount of the injected material inside the paraspinal muscles (white arrowhead) (A). Electronic reconstruction on sagittal plane of spiral CT, from D11 D12 to L4 L5, after the second lumbar EBP showing the presence of air in the posterior epidural space (black arrow) and in the paraspinal muscles (white arrow) (B). Electronic reconstruction on sagittal plane of spiral CT, from C2 to L1, after the third EBP showing enhancing of the posterior epidural spaces extending from L1 to C7 D1 (black arrows) (C). causing intracranial hypertension who underwent an evacuation of their subdural haematomas. Notably, these patients had no risk factor for bleeding, including the absence of anti-coagulant therapy. Therefore, SIH by itself may determine intracranial bleeding. The subdural haematomas are present in SIH in about 40% of cases [11]. The findings of the neurosurgeons regarding the parenchyma after evacuation of the haematoma were of an atypical picture, because the parenchymal brain pulsed less and expanded very slowly. Nevertheless nobody suspected a SIH. Our case suggests that placing a patient with rostrocaudal herniation by SIH in a 30 Trendelenburg position can be life-saving and that the Trendelenburg position can favour the spread of blood into epidural space from the lumbar to cervico-dorsal level. This may be very useful when the site of CSF leak remained unknown, as in our case, or when the leak site is at the cervical level [12]. In this case we did not perform an invasive spinal neuroimaging examination, as CT myelography, to identify the CSF leak site because we believe that EBP targeted at the CSF leak site is not mandatory, also because this site often remains unknown, even after performing several invasive examinations. Furthermore, the case shows that lumbar autologous EBP is effective in treating cases of SIH resulting in coma. It can sometimes appear ineffective because of the incorrect execution of this procedure, as shown in our case, by neuroimaging examination after the first and second lumbar autologous EBPs. During the first and second EBP, probably, initially, the Tuohy needle was in the epidural space, but by technical error moved outside the epidural space. So we can explain why majority of blood, was found in the lumbar paravertebral space. It is therefore necessary to perform a neuroimaging examination post-ebp to confirm the correct execution of EBP. Acetazolamide is a carbonic anhydrase inhibitor with half-life of 6 9 h [13]. It is, principally used to treat acute mountain sickness [14], increasing ventilation, glaucoma decreasing aqueous humour production [15] and idiopathic intracranial hypertension, reducing CSF formation and therefore the intracranial pressure [16]. It is employed in these pathologies with good results. Principal side effects of this drug are fatigue, anorexia, depression and distal paresthesias. We chose the use of acetazolamide and the Trendelenburg position during EBP performance from the neurosurgical procedure for the spinal liquoral leakage repair. In fact, they place a catheter to drain CSF reducing the spinal pressure, thus closing the CSF fistula. Probably pre-medication with acetazolamide associated with keeping the patient in TP could reduce the flow of spinal CSF leak and cause collapse of dura madre favouring the approach of borders of the dural hole producing the seal of hole. Furthermore, pre-medication with acetazolamide could avoid the rebound intracranial hypertension sometimes reported after EBP [17]. Until now we treated 42 patients and none had a rebound of intracranial hypertension after EBP. So it is probable that the low dose of acetazolamide is sufficient for our patients to prevent the rebound of intracranial hypertension, perhaps because they are more responsive to this drug. This, to our knowledge, is the first report that describes the use of acetazolamide in the treatment of SIH. References [1] Mokri B. Low cerebrospinal fluid pressure syndromes. Neurol Clin N Am 2004;22: [2] Schievink WI. Spontaneous spinal cerebrospinal fluid leaks. Cephalalgia 2008;28: [3] Schievink WI, Gordon OK, Tourje J. Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study. Neurosurgery 2004;54: [4] Sencakova D, Mokri B, McClelland RL. The efficacy of epidural blood patch in spontaneous CSF leaks. Neurology 2001;57: [5] Berroir S, Loisel B, Ducros A, Boukobza M, Tzourio C, Valade D, et al. Early epidural blood patch in spontaneous intracranial hypotension. Neurology 2004;63:

5 702 E. Ferrante et al. / Clinical Neurology and Neurosurgery 111 (2009) [6] Ferrante E, Arpino I, Citterio A, Savino A, Sterzi R. Lumbar epidural blood patch in Trendelenburg position to treat the headache by spontaneous CSF leak. Neurology 2007;68(Suppl. 1):A [7] Schievink WI, Moser FG, Pikul BK. Reversal of coma with an injection of glue. Lancet 2007;369:1402. [8] Ferrante E, Arpino I, Citterio A, Wetzl R, Savino A, Sterzi R. Spontaneous intracranial hypotension treated by lumbar epidural blood patch in Trendelenburg position pre-medicated with acetazolamide. Neurol Sci 2008;29(Suppl.):S24. [9] Pleasure SJ, Abosch A, Friedman J, Ko NU, Barbaro N, Dillon W, et al. Spontaneous intracranial hypotension resulting in stupor caused by diencephalic compression. Neurology 1998;50: [10] Pakiam A, Lee C, Lang A. Intracranial hypotension with Parkinsonism, ataxia, and bulbar weakness. Arch Neurol 1998;56: [11] Schievink WI, Maya MM, Moser FG, Tourje J. Spectrum of subdural fluid collection in spontaneous intracranial hypotension. J Neurosurg 2005;103: [12] Ferrante E, Arpino I, Citterio A. Is it a rational choice to treat with lumbar epidural blood patch headache caused by spontaneous cervical CSF leak? Cephalalgia 2006;26: [13] Jackson EK. Diuretics: inihibitors of carbonic anhydrase. In: Goodman and Gilman s The pharmacological basic of therapeutics. 10th ed. McGraw-Hill; p [14] Kayser B, Hulsebosch R, Bosch F. Low-dose acetylsalicylic analog and acetazolamide for prevention of acute mountain sickness. High Alt Med Biol 2008;9: [15] Ritland JS, Egge K, Lydersen S, Juul R, Semh SO. Comparison of survival of exfoliative glaucoma patients and primary open-angle glaucoma patients: impact of acetazolamide use. Acta Ophthalmol Scand 2004;82: [16] Kesler A, Hadayer A, Goldhammer Y, Almog Y, Korczyn AD. Idiopathic intracranial hypertension risk of recurrences. Neurology 2004;63: [17] Mokri B. Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks. Mayo Clinic Proc 2002;77:

Treatment of spontaneous intracranial hypotension: evolution of the therapeutic and diagnostic modalities

DOI 10.1007/s10072-013-1364-2 BRIEF COMMUNICATION Treatment of spontaneous intracranial hypotension: evolution of the therapeutic and diagnostic modalities Angelo Franzini G. Messina L. Chiapparini G.