MOHAMED LOTFY, M.D.*; MOATAZ A. EL-AWADY, M.D.**; ASHRAF E. ZAGHLOUL, M.D.** and TAREK NEHAD, M.D.***

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1 Med. J. Cairo Univ., Vol. 84, No. 2, December: , Effect of Therapeutic Lumbar Puncture on the Visual Outcome and the Further Need for Surgery in Patients with Idiopathic Intracranial Hypertension with Grade II Papilloedema MOHAMED LOTFY, M.D.*; MOATAZ A. EL-AWADY, M.D.**; ASHRAF E. ZAGHLOUL, M.D.** and TAREK NEHAD, M.D.*** The Department of Neurosurgery, Faculty of Medicine, Cairo* & Benha** Universities and the Department of Ophthalmology, Faculty of Medicine, Benha University***, Egypt Abstract Background: Idiopathic Intracranial Hypertension (IIH) is a disorder characterized by increased intracranial pressure (ICP) of unknown cause, predominantly seen in women of childbearing age and associated with a history of recent weight gain. There is little published evidence comparing lumbar puncture with medication or other treatment modalities. Objective: To study the effect of introduction of therapeutic lumbar puncture on the visual outcome and the further need for surgery in patients with IIH (grade II papilloedema). Patient and Methods: This prospective study included all the patients with IIH diagnosed according to the modified Dandy criteria with grade II papilloedema, managed at the Neurosurgical Department, Benha University between January 2009 and January The patients were randomly assigned into two Groups A and B according to the inclusion of therapeutic lumbar puncture. Results: The mean time at which headache decreased to nearly half the initial was 8.1 days (Group A) and 4.7 days (Group B). Five patients in Group A and three patients in Group B progressed to grade III papilloedema and lumboperitoneal CSF diversion was planned. In the first monthlyvisit, 12 patients in Group A and 13 patients in Group B showed gradual improvement to grade I while 5 patients in Group A and 6 patients in Group B showed gradual improvement to grade 0 and at the end of follow-up period all these patients reached grade 0 papilloedema. The rate of success of treatment was 77.2% in Group A and 86.3% in Group B. Conclusion: The results of this study favor the introduction of therapeutic lumbar CSF puncture together with the other non-surgical maneuvers in the management of IIH with grade II papilloedema. Key Words: Idiopathic intracranial hypertension Papilloedema Visual outcome. Correspondence to: Dr. Moataz A. El-Awady, moataz_elawady@yahoo.com motaz.youssef@fmed.bu.edu.eg Introduction IDIOPATHIC Intracranial Hypertension (IIH) is a disorder characterized by increased intracranial pressure (ICP) of unknown cause, predominantly seen in women of childbearing age and associated with a history of recent weight gain [20]. Pseudo Tumor Cerebri (PTC) is a term that was first used by Nonne to describe raised intracranial pressure in the absence of a space occupying lesion [12]. In his article he refered to other conditions causing ICP with no intracranial tumor, such as communicating hydrocephalus, which are no more classified as Pseudo tumor cerebri. that the term 'benign intracranial hypertension' became more commonly used and it was used interchangeably with PTC [19]. In comparison with patients with tumors it was considered 'benign' [13], but also regarding the possibility of becoming blind if not well treated in up to 25% of cases means we cannot consider this condition benign when vision is a concern. That is why now the term idiopathic intracranial hypertension is used in general [19]. There are different available treatment options for treating this condition including carbonic anhydrase inhibitors as acetazolamide, intermittent therapeutic lumbar puncture, lumbo-peritoneal and ventriculo-peritoneal shunts, and to choose the line of treatment we should consider, the possibility of losing vision or of significant headache if we don't have immediate intracranial pressure control, versus long-term possible complications of the different interventions [18]. No much published evidence that compares lumbar puncture with medication or other therapeutic modalities [12]. 301

2 302 Effect of Therapeutic Lumbar Puncture on the Visual Outcome The aim of this work was to study the effect of introduction of therapeutic lumbar puncture on the visual outcome and the further need for surgery in patients with IIH with grade II papilloedema. Patients and Methods Inclusion criteria: This prospective study included all the patients with IIH diagnosed according to the modified Dandy criteria (Table 1) with grade II papilloedema, managed at the Neurosurgical Department, Benha University between January 2009 and January The patients were randomly assigned into two Groups A and B according to the inclusion of therapeutic lumbar puncture. Table (1): Modified Dandy criteria. Symptoms of raised intracranial pressure headache, nausea, vomiting, transient visual obscurations or papilloedema. No localizing signs with the exception of abducent (CN VI) palsy. The patient is awake and alert. Normal CT and MRI findings. Lumbar puncture opening pressure >25cmH 2 O with normal CSF biochemical and cytological composition. No other explanation for raised intracranial pressure. Adapted from Friedman and Jacobson [4]. Exclusion criteria: The patients with IIH diagnosed according to the modified Dandy criteria and they had other grades of papilloedema were excluded from our study. All patients were examined neurologically and ophthalmological. Neurological visits: Complete neurological examination and review of the radiographic images (C.T and MRI brain were done for all patients) done by the authors. The degree of headache was assessed using the Visual Analogue Score (VAS) for pain. The patients are directed to record the degree of headache daily Ophthalmological visits: Ophthalmological examinations were done by the same person and included the visual acuity and fundus examination. The examinations were conducted at diagnosis and monthly thereafter. Papilledema was graded using the Frisén scale grade [6]. Grouping: Group A: Lumbar puncture was done for only diagnostic purpose (to prove that opening CSF pressure is higher than 25cm CSF and to get a sample for biochemical and cytological examination) and was repeated after one, three and six months. Group B: Lumbar puncture was done for diagnostic and therapeutic purposes. diagnosis, in therapeutic lumbar puncture the CSF was drained so that the CSF pressure was reduced to half the value of the opening pressure. Another lumbar puncture was done 24 hours after the therapeutic one to measure CSF pressure and diagnostic lumbar puncture was repeated after one, three and six months. All patients were treated in the form of weight reduction program and acetazolamide regimen of 10mg/kg body weight/day; orally divided into two or three doses per day. All patients were followed-up weekly for the degree of headache and monthly for the visual acuity and degree of papilloedema on fundus examination. Ophthalmological follow-up was repeated also in cases when there was severe progressive headache or progressive deterioration of vision. Medical treatment with lumbar puncture was considered successful when headache and papilloedema resolved with no recurrence during sixmonth period of follow-up. Progression of papilloedma despite these maneuvers was the indication for surgery. The surgery performed was lumboperitoneal CSF diversion. Statistical analysis: The collected data were tabulated and analyzed using SPSS version 16.0 for Windows. Categorical data were presented as number and percentages while quantitative data were expressed as mean ± Standard Deviation (SD). The Student's t-test was used to compare between means of two groups of numerical (parametric) data. Inter-group comparison of categorical data was performed by using Fisher's Exact Test as appropriate. Statistical significance was accepted at p-value <0.05. Results 44 patients with IIH (grade II papilloedema on fundus examination) included in this study. They were 35 females and 9 males with age ranging between 16 and 45 years (mean ± SD=27.1 ±9.3). Tables (2,3) showed the CSF pressure in both groups during the study period. Neurological results: Headache: The mean time at which headache decreased to nearly half the initial was 8.1 days in Group A and 4.7 days in Group B. Abducent palsy: Only one patient in Group B had abducent palsy. Initial OP was 60cm CSF,

3 Mohamed Lotfy, et al. 303 headache did not improve and papilloedema progressed so lumboperitoneal CSF diversion was arranged later on. Ophthalmological results: Five patients in Group A and three patients in Group B progressed to grade III papilloedema and lumbo-peritoneal CSF diversion was planned. In the first monthlyvisit, 12 patients in Group A and 13 patients in Group B showed gradual improvement to grade I while 5 patients in Group A and 6 patients in Group B showed gradual improvement to grade 0 and at the end of follow-up period all these patients reached grade 0. Table (4) showed the patient criteria and results of the study. Comparing the success of both groups were non-statistically significant (p>0.05 for both the success rate and the proportion of patients who required surgery). Complications of the lumbar puncture: Transient radiculopathy occurred in 3 patients in Group A (13.6%) and in 5 patients in Group B (22.7%) but disappeared in few days. Table (2): Opening CSF pressure after each lumbar puncture in Group A. Patient Age Sex Initial LP 1 month 1 22 F month 6 month 2 28 F F F M F F F M F F M F F F M F F F F F F Table (3): Opening CSF pressure after each lumbar puncture in Group B. Patient Age Sex Initial LP 24h 1 3 month months 1 30 F months 2 33 F M F F F M F F M F F M F F F F M F F F F Table (4): Patient criteria and results of the study. Total number Age (years) mean ± SD Group A Group B Test p-value (±8.5) Sex: Females FET=0 1 Males (±7.2) t= Success of non-surgical treatment 17 (77.2%) 19 (86.3%) FET= Patients who required surgery 5 (22.8%) 3 (13.7%) FET=

4 304 Effect of Therapeutic Lumbar Puncture on the Visual Outcome Fig. (1): Bilateral optic disc edema at presentation (Group B). Fig. (2): Resolution of optic disc edema after six-months of the same patient in Fig. (1). Discussion Restoring acuity of vision and resolving papilledema are the principal objectives of treating idiopathic intracranial hypertension and the main landmark of relative success of a treatment option [5]. When the patients have IIH with no visual changes as the main presentation, medication and reduction of body weight as conservative measures are routinely recommended. Time may be needed for these conservative measures to become effective; so, we advise frequent follow-up, at intervals of 1 month [11]. In this study, all patients were treated in the form of weight reduction program and acetazolamide regimen of 10mg/kg body weight/day, orally two or three times per day. It is thought that reduction of body weight can address one of the principal risk factors for having idiopathic intracranial hypertension recurrence and may also reducing the risk of idiopathic intracranial hypertension and concomitant visual deteri- oration by central venous pressure reduction or through endocrinologic mechanism [17]. Medications that are Carbonic Anhydrase Inhibitors (CAI), were found to decrease the CSF formation rate, and so addressing one of the proposed mechanisms of idiopathic intracranial hypertension and typically are used to treat and prevent recurrence of this condition [20]. Of carbonic anhydrase inhibitors, the most commonly used medication to treat IIH is Acetazolamide, but topiramate shown to be of equal effect as a result of partial CAI activity augmented by its analgesic action against headache and also body weight reduction as a fortuitous side effect [14]. Using corticosteroids may cause temporary remission of idiopathic intracranial hypertension, but the risks of chronic use of steroid in obese patients preclude their use [1]. Another common treatment option is therapeutic lumbar puncture that is addressing directly the elevated CSF pressure by reducing it immediately when the patient has rapidly declining vision. One

5 Mohamed Lotfy, et al. 305 older study suggesting that the effect is of short duration; and within just an hour a return to initial pressure takes place [8]. This was proved to be untrue in our study according to the values of CSF pressure after 24 hours. In the past serial lumbar punctures (e.g., twice or three times weekly) have been proposed as an alternative to surgery for patients with papilledema, when the disease cannot be controlled medically [4]. In this study we aimed at studying the effect of introduction of therapeutic lumbar puncture on the visual outcome in patients with grade II papilloedema on the Frisen scale [6]. Lower grades may be self-limited or improve with short term medications [13] and the higher grades usually need rapid surgical interventions [14,15]. The introduction of therapeutic lumbar CSF puncture together with the other non-surgical maneuvers in the management of IIH. Elevated the rate of success of treatment from 77.2% in Group A to 86.3% in Group B and decreased the percentage of patients that need further more aggressive surgery from 22.8% in Group A to 13.7% in Group B. The two groups were comparable regarding mean age and sex. The only variable to which the change in success of treatment could be attributed is the introduction of therapeutic lumbar CSF puncture. The natural history of this condition is unknown [7]. In some cases, it is a self-limited, while in others intracranial pressure may remain elevated for years even if the patient systemic and visual symptoms has resolved. In some patients, the process may last from months to years. Patients with mild to moderate loss of vision tend to recover vision after medical treatment. Papilledema usually needs a few weeks or months to resolve, but many patients are left with some residual disc elevation, especially nasally [16]. Severe visual impairment could be permanent and serious complication of idiopathic intracranial hypertension. The condition produces marked visual impairment in nearly 25% of cases. In idiopathic intracranial hypertension, the risk of losing vision in the pediatric and adults are similar [3]. Recurrent symptoms reported in 8 to 37% of cases, even years after diagnosis [3]. Deterioration of vision in these cases is usually gradual, but other patients may have fulminant papilledema and blindness may appear more rapidly. In Corbett (2004) follow-up study of 5-41 years after the initial diagnosis of 57 patients, revealed severe visual impairment in 14 patients (24.6%) [2]. In Kesler et al., experience the long term prognosis and visual outcome of 54 patients with IIH observed over a period of 6.2 years showed that recurrences occurred in almost 40% of the cases and recurrence was frequently associated with weight gain [10]. Conclusion: The results of this study favor the introduction of therapeutic lumbar CSF puncture together with the other non-surgical maneuvers in the management of IIH with grade II papilloedma. Meanwhile further studies with larger number of patients are still needed to elucidate this conflicting point. Acknowledgement: & References 1- BRAZIS P.W.: Pseudotumorcerebri. Curr. Neurol. Neurosci. Rep., 4: , CORBETT J.J.: Increased intracranial pressure: Idiopathic and otherwise. Journal of Neuro-Ophthalmology, Vol. 24, No. 2, (June 2004), pp , CORBETT J.J.: The first Jacobson Lecture. Familial idiopathic intracranial hypertension. Journal of Neuroophthalmology, Vol. 28, No. 4, (December 2008), pp , FRIEDMAN D.I. and JACOBSON D.M.: Diagnostic criteria for idiopathic intracranial hypertension. Neurology, 59: , FRIEDMAN D.I., McDERMOTT M.P. and KIEBURTZ K.: The Idiopathic Intracranial Hypertension Treatment Trial: Design Considerations and Methods J. Neuro- Ophthalmol., 34: , FRISÉN L.: Swelling of the optic nerve head: A staging scheme. J. Neurol. Neurosurg. Psychiatry., 45: 13-8, GEORGE A.E.: Idiopathic intracranial hypertension: Pathogenesis and the role of MR imaging. Radiology, 170: 21-2, JOHNSTON I. and PATERSON A.: Benign intracranial hypertension. II. CSF pressure and circulation. Brain, 97: , KAMMERDIENER L. and FRIEDMAN D.I.: Patient interviews and focus groups to determine outcome measures for idiopathic intracranial hypertension. Paper presented at: North American Neuro-ophthalmology Society; Tucson, AZ, KESLER A., HADAYER A., GOLDHAMMER Y., AL- MOG Y. and KORCZYN A.D.: Idiopathic intracranial hypertension: Risk of recurrences. Neurology, Vol. 63, No. 9, (November 2004), pp , LUECK C.J. and McILWAINE G.G.: Idiopathic intracranial hypertension. Pract. Neurol., , 2002.

6 306 Effect of Therapeutic Lumbar Puncture on the Visual Outcome 12- NONNE M.: Über Fällevom Symptomenkomplex "Tumor cerebri" mitausgang in Heilung (Pseudotumorcerebri). ÜberletalverlaufeneFälle von "Pseudotumorcerebri" mit- Sektionsbefund. J. Neurol., 27: , PEARCE J.M.: From pseudotumourcerebri to idiopathic intracranial hypertension. Pract. Neurol., 9: 353-6, RANDHAWA S. and VAN STAVERN G.P.: Idiopathic intracranial hypertension (pseudotumorcerebri). Curr. Opin. Ophthalmol., 19: , REBOLLEDA G. and MUNOZ-NEGRETE F.J.: Followup of mild papilledema in idiopathic intracranial hypertension with optical coherence tomography. Investigative Ophthalmology & Visual Science, Vol. 50, No. 11, (November 2009), pp , STONE M.B.: Ultrasound diagnosis of papilledema and increased intracranial pressure in pseudotumorcerebri. American Journal of Emergency Medicine, Vol. 27, No. 3, (March 2009), pp. e1-376, SUGERMAN H.J., DeMARIA E.J., FELTON W.L., et al.: Increased intra-abdominal pressure and cardiac filling pressures in obesity-associated pseudotumorcerebri. Neurology, 49: , TANG R.A., DOROTHEO E.U., SCHIFFMAN J.S. and BAHRANI H.M.: Medical and surgical management of idiopathic intracranial hypertension in pregnancy. Current Neurology and Neuroscience Reports, Vol. 4, No. 5, (September 2004), pp , TIBUSSEK D., SCHNEIDER D.T., VANDEMEULEBRO- ECKE N., et al.: Clinical spectrum of the pseudotumorcerebri complex in children. Child's Nervous System, Vol. 26, No. 3, (March 2010), pp , WALL M.: Idiopathic intracranial hypertension. Neurol. Clin., 28: , 2010.