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doi:10.1111/j.1468-2982.2008.01569.

REVIEW

ICHD-II diagnostic criteria for TolosaHunt syndrome in idiopathic inammatory syndromes of the orbit and/or the cavernous sinus
S Colnaghi1,2,3, M Versino1,2,3, E Marchioni3,4, A Pichiecchio5, S Bastianello1,5, V Cosi1,4 & G Nappi3,6,7
Department of Neurology, University of Pavia, 2Department of Neuro-Otology and Neuro-Ophthalmology, IRCCS Neurological Institute C. Mondino Foundation, 3UCADHUniversity Centre for Adaptive Disorders and Headache, University of Pavia, 4Department of Clinical Neurology, 5Department of Neuroradiology and 6Scientic Direction, IRCCS Neurological Institute C. Mondino Foundation, Pavia, and 7 Department of Neurology and ENT, University La Sapienza, Roma, Italy
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Colnaghi S, Versino M, Marchioni E, Pichiecchio A, Bastianello S, Cosi V & Nappi G. ICHD-II diagnostic criteria for TolosaHunt syndrome in idiopathic inammatory syndromes of the orbit and/or the cavernous sinus. Cephalalgia 2008; 577584. London. ISSN 0333-1024 A bibliographical search was conducted for papers published between 1999 and 2007 to verify the validity of International Classication of Headache Disorders (ICHD)-II criteria for the TolosaHunt syndrome (THS) in terms of (i) the role of magnetic resonance imaging (MRI); (ii) which steroid treatment should be considered as adequate; and (iii) the response to treatment. Of 536 articles, 48, reporting on 62 patients, met the inclusion criteria. MRI was positive in 92.1% of the cases and it normalized after clinical resolution. There was no evidence of which steroid schedule should be considered as adequate; high-dose steroids are likely to be more effective both to induce resolution and to avoid recurrences. Pain subsided within the time limit required by the ICHD-II criteria, but signs did not. We conclude that THS diagnostic criteria can be improved on the basis of currently available data. MRI should play a pivotal role both to diagnose and to follow-up THS. Cavernous sinus, headache, ICHD-II, orbital pseudotumour, TolosaHunt syndrome Silvia Colnaghi, Fondazione Istituto Neurologico C. Mondino IRCCSvia Mondino, 2-27100 Pavia, Italy. Tel. + 39 03 8238 0340, fax + 39 03 8238 0286, e-mail si.co@libero.it Received 2 November 2007, accepted 22 December 2007

Introduction
TolosaHunt syndrome (THS) (1, 2) is a rare disorder characterized by periorbital or hemicranial pain accompanied by diplopia, that may eventually recur. The aetiology is still unknown, but the underlying pathophysiological mechanism has been proven to consist of a granulomatous inammatory process. THS was identied as a nosological entity by the International Headache Society classication criteria published in 1988 (3). These criteria were substantially revised in the 2004 classication (4)
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(Table 1), and the literature was critically reviewed accordingly (5). In our opinion, there are several pending questions regarding the appropriateness of the diagnostic criteria according to the present International Classication of Headache Disorders (ICHD)-II, especially in terms of the best treatment choice, the duration of symptoms and signs and the anatomical distribution of lesions. The classication criteria mention magnetic resonance imaging (MRI) explicitly for the diagnosis and implicitly for the differential diagnosis of THS
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Table 1 ICHD-II classication part three. Cranial neuralgias, central and primary facial pain and other headaches 13.16 TolosaHunt syndrome Description: Episodic orbital pain associated with paralysis of one or more of the third, fourth and/or sixth cranial nerves which usually resolves spontaneously but tends to relapse and remit. Diagnostic criteria: A. One or more episodes of unilateral orbital pain persisting for weeks if untreated B. Paresis of one or more of the third, fourth and/or sixth cranial nerves and/or demonstration of granulomas by MRI or biopsy C. Paresis coincides with the onset of pain or follows it within 2 weeks D. Pain and paresis resolve within 72 h when treated adequately with corticosteroids E. Other causes have been excluded by appropriate investigations1 Note: 1. Other causes of painful ophthalmoplegia include tumours, vasculitis, basal meningitis, sarcoid, diabetes mellitus and ophthalmoplegic migraine. Comments: Some reported cases of TolosaHunt syndrome had additional involvement of the trigeminal nerve (commonly the rst division) or optic, facial or acoustic nerves. Sympathetic innervation of the pupil is occasionally affected. The syndrome has been caused by granulomatous material in the cavernous sinus, superior orbital ssure or orbit in some biopsied cases. Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia.

(6, 7), but we think that a positive MRI performed with appropriate techniques (812) should be mandatory for diagnosis. The ICHD-II states in point D that adequate steroid dosage should resolve disease symptoms and signs within 72 h from the treatment onset. The adequate dosage of steroids and the best route of administration are undened. The consistency of the 72-h period, as a time limit for resolution of symptoms and signs, does not seem to be sustained by appropriate evidence in the literature and, in our opinion, does not t the real disease course. Indeed, it seem to represent a critical misleading point in the diagnostic process. Another question pertains to the anatomical distribution of lesions for the diagnosis of THS. According to the ICHD-II criteria, all inammatory granulomas located in the cavernous sinus, the orbital apex and the orbit, independently of the involved structures (nerves, muscles or both), can justify the diagnosis of THS. In contrast, most of the neurological and neuroradiological textbooks reserve this diagnosis for those lesions located in the cavernous sinus, while they dene the inammatory processes involving the orbit as pseudotumour. This discrepancy of terms and denitions limits the appropriateness of the diagnosis, prognosis and the best treatment choices.

Objectives
We set out to compare clinical features and outcome measures in discrete subgroups of THS patients according to: (a) the distribution of lesions (cavernous sinus, orbital apex, orbit, extraocular muscles); (b) different steroid dosage and route of administration.

Fruitfulness
We also set out

to verify the external consistency and appropriateness of the ICHD-II criteria for THS, paying particular attention to: (a) the time duration of symptoms and signs according to the steroid treatment (b) the inclusion of different anatomical locations of lesions conditioning involvement of different structures (nerves, muscle or both) in the same paragraphs of the ICHD-II; to provide useful propositions about the best pharmacological treatment and the most appropriate criteria for MRI follow-up.

For these reasons, we reviewed the reports on patients suffering from THS who underwent MRI
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ICHD-II diagnostic criteria for IHS examination, but we also considered some conditions that mimic, but cannot be classied as THS due to the localization of the inammatory process.

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Methods
We conducted a bibliographical search on PubMed/ Metacrawler and on Embase using the following Keywords: Tolosa Hunt, Orbital pseudotumor, Idiopathic orbital inammation, Painful ophthalmoplegia. The limits of the search were the English language, and the date of publication between 1 January 1999 and 31 January 2007. To be considered further, the cases had to: 1. have been studied by means of MRI; 2. full the ICHD-II diagnostic criteria of THS with the exception of point D, concerning symptom resolution within 72 h after starting steroid treatment; 3. be as in point 2, but with an extension of the inammatory process outside the orbit and the cavernous sinus; 4. be as in point 2, but with inammation located in the ocular muscles only. For each case we noted the following data: sex, age, side, symptoms (orbital pain, diplopia, visual loss, facial numbness or pain), signs (ocular motor palsy, ocular motor palsy and visual loss, ocular motor palsy and abnormal facial sensation, ocular motor palsy and facial palsy), associated diseases, MRI techniques, location of inammatory tissue as detected by MRI (coded as not detectable, cavernous sinus only, orbit with or without cavernous sinus, cavernous sinus and/or orbit with extension to other intracranial structures, and extraocular muscles), time elapsed between onset of symptoms and starting treatment, duration of clinical followup, duration of MRI follow-up. We also considered the treatment schedules and distinguished three groups: the regular steroid dosage group including patients who were treated with an initial dosage of about 1 mg/kg per day, and the high steroid dosage group including patients who were treated with an initial dosage of 5001000 mg/day for a few days and then usually switched to a regular regimen for a longer period, and treatments other than steroids. Finally, for each subject we noted the following outcome features: pain resolution (coded as more or less than 72 h, and coded as number of days), symptoms resolution (coded as more or less than 72 h, and coded as number of days), time of pathological tissue disappearance on MRI (coded as yes
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or no, and coded as number of days), occurrence of recurrence (coded as yes or no). On the basis of the location of inammatory tissue as detected by MRI, the subjects were divided into three diagnostic groups: 1. the THS group (no inammatory tissue detectable, cavernous sinus only, orbital apex and/or orbit with or without cavernous sinus); 2. the THS plus group (cavernous sinus and/or orbit with extension to other intracranial structures); 3. the orbital myositis (OM) group (extraocular muscles). In the THS group we distinguished a further three subgroups on the basis of the lesion site:

normal MRI subgroup; cavernous sinus subgroup (cavernous sinus only); orbital apex and/or orbit and cavernous sinus subgroup (orbit with or without cavernous sinus).

For each of the three diagnostic groups, the mean and standard deviation (SD) values of each of the above-mentioned variables were computed. We then compared the mean values between groups by means of a KruskalWallis or MannWhitney test for the continuous variables, and by means of a c2 test for the ordinal variables. In the THS group for each of the outcome variables (pain resolution, symptom resolution, time of pathological tissue disappearance at MRI, occurrence of recurrence), we compared the mean values between the cavernous sinus subgroup and the orbit and cavernous sinus subgroup. Finally, considering all the subjects together, the possible relationship between the kind of steroid treatment (regular and high-dose) and the outcome variables (sign disappearance, MRI normalization, and recurrence) was evaluated by means of c2 and MannWhitney tests. The critical P-value was set at P = 0.01.

Results
The bibliographical search revealed 536 articles, 48 of which met the criteria to be included (811, 1356). Overall we included 62 subjects extracted from 48 reports: 38 in the THS group (three of which showed a normal MRI and three were not treated), 16 in the THS plus group and eight in the OM group. In the THS group, inammation was detectable only in the cavernous sinus in 23 (60.5%) patients, in the cavernous sinus and/or in the orbit in 12

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Table 2 For each variable the table shows the mean and the standard deviation (SD) values, and the range for each diagnostic group, and the results of the KruskalWallis test (KW) for mean comparison Diagnostic group Age (years) THS THS plus OM THS THS plus OM THS THS plus OM THS THS plus OM THS THS plus OM THS THS plus OM THS THS plus OM n (%) 37 (97) 16 (100) 8 (100) 25 (66) 10 (63) 8 (100) 31 (82) 14 (9) 8 (100) 21 (55) 11 (69) 3 (38) 6 (16) 2 (13) 2 (25) 26 (68) 7 (44) 3 (38) 13 (34) 9 (56) 3 (38) Mean 40.73 39.69 28.38 17.84 30.30 14.25 352.77 539.14 565.50 276.00 528.00 79.33 47.17 16.50 35.00 100.27 41.71 73.33 352.31 332.00 81.67 SD 16.29 22.19 14.91 39.74 73.90 20.36 577.86 642.83 861.14 641.22 746.46 63.26 94.580 19.09 29.70 281.668 61.73 69.64 769.983 496.84 60.48 Minmax 973 875 951 0180 0240 056 102880 561950 282520 102880 302310 28150 3240 330 1456 71440 3180 14150 102880 301440 35150 KW 2.73 P = 0.25

Treatment delay (days)

1.26 P = 0.53

Clinical follow-up (days)

2.16 P = 0.34

MRI follow-up (days)

2.66 P = 0.26

Pain resolution (days)

1.144 P = 0.564

Signs resolution (days)

1.424 P = 0.491

MRI normalization (days)

0.416 P = 0.812

n is the number of subjects for whom the data were available in each diagnostic group, and in parentheses is the corresponding percentage with respect of the total number of that group. MRI, magnetic resonance imaging; OM, orbital myositis; THS, TolosaHunt syndrome.

(31.6%) patients and was not detectable in three (7.9%) patients only. The features of the individual reports described in each of the papers included in this Review are available as supplementary on-line material, and Tables 2 and 3 summarize the data. In all the patients for whom these data were available (30/62, 48.4%), the interval between pain and ocular motor palsy onset was always < 2 weeks, as required by the classication criteria. The three diagnostic groups did not differ in most of the variables (Tables 2 and 3), and the variability of the data may partly account for this negative nding. The only exceptions were pain resolution within the 72-h time limit, and recurrence. Pain resolution within the 72-h time limit occurred more frequently in the THS and THS plus than in the OM group (Table 3), and recurrence was more likely in the THS plus and in the OM than in the THS group (Table 3). Although the pain resolved within 72 h after starting treatment, as required by the classication criteria, in most of the THS and THS plus patients, the signs resolved only in one THS plus patient

within this time limit (Table 3). The mean time needed for sign disappearance was not statistically signicantly different in the three diagnostic groups (Table 2). The mean duration of MRI follow-up was not statistically signicantly longer (MannWhitney U 110, z = -0.08, P = 0.933) in patients who showed normalization (n = 16; 230 days) than in those who did not (n = 14; 448 days), but the normalization of MRI always lasted longer than sign resolution in all three diagnostic groups (Table 2). When the outcome variables were compared in the two THS subgroups (the cavernous sinus subgroup and the orbit and cavernous sinus subgroup), no signicant difference was found. Fifty-four (87%) patients underwent steroid treatment, ve (8%) underwent treatment other than steroids (including surgical removal of the inammatory tissue) and three (5%) were not treated. Treatments most often lasted about 2 months, but the duration could range from a few days to several years. By considering all the patients together, the mean time needed for sign disappearance was longer
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ICHD-II diagnostic criteria for IHS


Table 3 For each variable the table shows for each diagnostic group the number of cases and the corresponding percentage with respect of the total number of that group, and the results of the c2 test THS Sex Side Female Male Bilateral Left Right Pain and diplopia Visual loss Facial numbness Facial palsy Regular High dosage Other Yes No Yes No < 72 h > 72 h < 72 h > 72 h Yes No Yes No 17 (45%) 21 (55%) 2 (5%) 18 (48%) 18 (48%) 24 4 9 1 (63%) (10%) (24%) (3%) THS plus 9 (56%) 7 (44%) 2 (12%) 8 (50%) 2 (25%) 11 58 0 0 (69%) (31%) (0%) (0%) OM 6 (75%) 2 (25%) 1 (13%) 2 (25%) 5 (62%) 8 0 0 0 (100%) (0%) (0%) (0%) c2

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2.6 P = 0.27 2.52 P = 0.64 12.27 P = 0.06

Symptoms

Steroid treatment

26 (74%) 9 (26%) 0 (0%) 29 (91%) 3 (9%) 25 (66%) 13 (34%) 18 (78%) 5 (22%) 0 (0%) 28 (100%) 10 (50%) 10 (50%) 8 (21%) 30 (79%)

11 (69%) 2 (12%) 3 (19%) 14 (100%) 0 (0%) 11 (69%) 5 (31%) 5 (62%) 3 (38%) 1 (8%) 12 (92%) 4 (36%) 7 (64%) 11 (69%) 5 (31%)

6 (75%) 0 (0%) 2 (25%) 6 (75%) 2 (25%) 3 (37%) 5 (62%) 0 (0%) 4 (100%) 0 (0%) 5 (100%) 3 (100%) 0 (0%) 4 (59%) 4 (50%)

10.34 P = 0.03 3.79 P = 0.15 2.58 P = 0.27 9.310 P = 0.010 2.595 P = 0.273 3.818 P = 0.148 11.633 P = 0.003

Clinical follow-up MRI follow-up Pain resolution Signs resolution MRI normalization Recurrences

after regular (n = 27; 104 days) than after high-dose steroid treatment (n = 8; 35.5 days), but these two gures did not prove to be statistically signicant (MannWhitney; P = 0.686). The time to reach MRI normalization showed similar behaviour, being longer after regular (n = 20, mean 369.5 days, range 102880 days) than after high-dose treatment (n = 3; mean 119.3 days, range 10320 days), but again this difference was not statistically signicant (Mann Whitney U 17.5, z = 1.14, P = 0.253). Recurrence occurred in 19 (44.2%) patients who underwent regular steroid treatment and in only one (9.1%) of those treated with high-dose steroid (c2 = 4.62, Fishers exact test P = 0.039).

Some points clearly stand out: 1. The classication criteria require that both symptoms and signs resolve within 72 h after starting adequate steroid treatment. Unfortunately, the classication criteria do not state what adequate means, and the literature reports individualized rather than standardized treatment. In addition, in only 2% of the patients did the signs resolve so quickly. 2. MRI showed the presence of inammatory tissue in 92.1% of THS patients: this, and the clinical overlap of THS with other conditions such as those we labelled as THS plus and OM, strongly supports the importance of MRI for the diagnosis of THS. 3. The localization or extension of the inammatory tissue does not seem to be a prognostic factor, and, more specically, the patients from the THS plus group and those from the THS group behave similarly. It may be suggested to amalgamate the two groups. Furthermore, neuroradiological distinction between the THS syndrome (inammatory tissue within the cavernous sinus) and the

Discussion
We were able to consider 62 patients derived from papers about single or a few cases with some unusual features. However, these features varied from one subject to another, and we consider that as a group these patients are likely to be representative of THS, with the only exception that they do not need to full the 72 h diagnostic criteria.
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pseudotumor orbitae needs further investigation to prove its clinical signicance. 4. MRI data show that the disappearance of inammatory tissue takes longer than symptom resolution, and that pathological tissue may not disappear even after several months. Accordingly, it may be suggested that MRI should be used to monitor the disease course and to support the decision of when treatment should be stopped. However, the question of whether THS may have a relapsing course or may become a chronic condition, and whether these two outcomes depend on treatment, is left open. Our review showed that the choice of treatment varied in terms of the kind of steroid, dosage, administration method and duration; but also suggested that recurrence is less likely in patients with high-dose than in those with regular steroid treatment.

Notes
1. NRI should be performed with 3 mm thickness coronal and axial T1-SE and T2-TSE sequences and with T1 fat-suppressed weighted images after godolinium administration at the level of the orbit and cavernous sinus. 2. Other causes of painful ophthalmoplegia include vascular, neoplastic, infectious, systemic granulomatous diseases, basal meningitis, diabetes mellitus, ophthalmopletic migraine, thyroid ophthalmopathy and trauma.

Comments
1. Some reported cases of THS had additional involvement of the trigeminal nerve (commonly the rst division) or optic, facial or acoustic nerves. Sympathetic innervation of the pupil is occasionally affected. 2. The syndrome is caused by granulomatous material in the cavernous sinus, superior orbital ssure or orbit in some biopsied cases; granulomatous material can have an intracranial extension, as demonstrated by MRI. 3. Careful clinical and MRI follow-up is required to conrm the diagnosis and to manage the steroid treatment. It might also be benecial to distinguish between denite THS, when all the criteria are met, and possible THS, when all the criteria but the one of point D are met. These criteria implicitly suggest that THS is an idiopathic disorder whose pathophysiology consists of a granulomatous inammatory process, and that THS could be moved from the cranial neuralgia section to the secondary headache section. In conclusion, further multicentre studies are necessary to improve our knowledge of THS, but the revision of previous papers can suggest some changes to improve the classication criteria.

Conclusion and proposal


Overall, in our opinion the classication criteria for THS syndrome could benet from the points discussed above: (i) there is no evidence of what kind of steroid treatment is adequate; (ii) the 72-h time limit should be applied for pain resolution only; (iii) MRI plays a key role in diagnosis; and (iv) inammatory tissue can extend beyond the cavernous sinus and the orbit. Points (iii) and (iv) imply that we need to use MRI techniques specically aimed at showing the presence of inammatory tissue, as suggested by Cakirer (8), La Mantia (5) and ourselves (9). The diagnosis of THS cannot rely only on MRI data, but these should be used in conjunction with clinical ndings both to make the diagnosis and to follow-up patients suffering from THS and, in some cases, a biopsy will still be needed to ascertain the nature of the pathological tissue. The classication criteria could be modied as follows: A. One or more episodes of unilateral orbital pain persisting for weeks if untreated B. Paresis of one or more of the third, fourth and/or sixth cranial nerves and demonstration of granulomatous material in the cavernous sinus, superior orbital ssure or orbit by MRI C. Paresis coincides with the onset of pain or follows it within 2 weeks D. Pain resolves within 72 h when treated with corticosteroids E. Other causes have been excluded by appropriate investigations.

References
1 Hunt WE. TolosaHunt syndrome: one cause of painful ophthalmoplegia. J Neurosurg 1976; 44:5449. 2 Tolosa E. Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm. J Neurol Neurosurg Psychiatry 1954; 17:3002. 3 Headache Classication Committee of the International Headache Society. Classication and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Cephalalgia 1988; 8 (Suppl. 7):196. 4 Sarchielli PXI. Congress of the International Headache

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Society. September 1316, 2003, Rome, Italy. Expert Opin Pharmacother 2004; 5:95975. La Mantia L, Curone M, Rapoport AM, Bussone G. TolosaHunt syndrome: critical literature review based on IHS 2004 criteria. Cephalalgia 2006; 26:77281. Brazis PW, Lee AG, Stewart M, Capobianco D. Clinical review: the differential diagnosis of pain in the quiet eye. Neurologist 2002; 8:82100. Gladstone JP, Dodick DW. Painful ophthalmoplegia: overview with a focus on TolosaHunt syndrome. Curr Pain Headache Rep 2004; 8:3219. Cakirer S. MRI ndings in TolosaHunt syndrome before and after systemic corticosteroid therapy. Eur J Radiol 2003; 45:8390. Colnaghi S, Pichiecchio A, Bastianello S, Versino M. SPIR MRI usefulness for steroid treatment management in TolosaHunt syndrome. Neurol Sci 2006; 27:1379. de Arcaya AA, Cerezal L, Canga A, Polo JM, Berciano J, Pascual J. Neuroimaging diagnosis of TolosaHunt syndrome: MRI contribution. Headache 1999; 39:3215. Desai SP, Carter J, Jinkins JR. Contrast-enhanced MR imaging of TolosaHunt syndrome: a case report. Am J Neuroradiol 1991; 12:1823. Pascual J, Cerezal L, Canga A, Alvarez de Arcaya A, Polo JM, Berciano J. TolosaHunt syndrome: focus on MRI diagnosis. Cephalalgia 1999; 19 (Suppl. 25):368. Adams AB, Kazim M, Lehman TJ. Treatment of orbital myositis with adalimumab (Humira). J Rheumatol 2005; 32:13745. Alioglu Z, Akbas A, Sari A, Erdol H, Ozmenoglu M. Tolosa Hunt syndrome: a case report. Clinical and magnetic resonance imaging ndings. J Neuroradiol 1999; 26:6872. Borruat FX, Vuilleumier P, Ducrey N, Fankhauser H, Janzer RC, Regli F. Idiopathic orbital inammation (orbital inammatory pseudotumour): an unusual cause of transient ischaemic attack. J Neurol Neurosurg Psychiatry 1995; 58:8890. Brown JS, Moster ML, Kenning JA, Ronis ML. The TolosaHunt syndrome: a case report. Otolaryngol Head Neck Surg 1990; 102:4024. del Toro M, Macaya A, Vazquez E, Roig M. Painful ophthalmoplegia with reversible carotid stenosis in a child. Pediatr Neurol 2001; 24:31719. Ersahin Y. Orbital pseudotumor. Pediatr Neurosurg 1999; 31:53. Falcini F, Simonini G, Resti M, Cimaz R. Recurrent orbital pain and diplopia in a 12 year old boy. Ann Rheum Dis 2002; 61:934. Foroozan R. Combined central retinal artery and vein occlusion from orbital inammatory pseudotumour. Clin Exp Ophthalmol 2004; 32:4357. Foubert-Samier A, Sibon I, Maire JP, Tison F. Long-term cure of TolosaHunt syndrome after low-dose focal radiotherapy. Headache 2005; 45:38991. Ganesan V, Lin JP, Chong WK, Kirkham FJ, Surtees RA. Painful and painless ophthalmoplegia with cavernous sinus pseudotumour. Arch Dis Child 1996; 75:239 41. Garrity JA, Coleman AW, Matteson EL, Eggenberger ER, Waitzman DM. Treatment of recalcitrant idiopathic

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10

11

12

13

14

15

16

17

18 19

20

21

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orbital inammation (chronic orbital myositis) with iniximab. Am J Ophthalmol 2004; 138:92530. 24 Gonzales GR. Pain in TolosaHunt syndrome. J Pain Symptom Manage 1998; 16:199204. 25 Goto Y, Hosokawa S, Goto I, Hirakata R, Hasuo K. Abnormality in the cavernous sinus in three patients with TolosaHunt syndrome: MRI and CT ndings. J Neurol Neurosurg Psychiatry 1990; 53:2314. 26 Guaschino E, Zandrini C, Minonzio G, Mancioli A, Broggini M, Bono G. Relapsing-remitting painful ophthalmoplegia due to orbital myositis. J Headache Pain 2005; 6:4712. 27 Haque TL, Miki Y, Kashii S, Yamamoto A, Kanagaki M, Takahashi T et al. Dynamic MR imaging in TolosaHunt syndrome. Eur J Radiol 2004; 51:20917. 28 Hardman JA, Halpin SFS, Mars S, Hourihan MD, Lane CM. MRI of idiopathic orbital inammatory syndrome using fat saturation and Gd-DTPA. Neuroradiology 1995; 37:4758. 29 Imai F, Kiya N, Ogura Y, Nomura M, Gireesh K, Sano H, Kanno T. TolosaHunt syndrome with unusual clinical coursestwo case reports. Neurol Med Chir (Tokyo) 1995; 35:2831. 30 Ishikawa S, Yahikozawa H, Yamazaki M, Kikkawa M, Ikeda SI, Hanyu N. Recurrent steroid-responsive trismus and painful ophthalmoplegia. J Neurol Sci 1999; 170:69 71. 31 Kaji T, Arisaka M, Arisaka H, Shimada A, Miyamoto K, Nojiri K et al. TolosaHunt syndrome complicated with chronic hemodialysis. Nephron 1993; 63:3712. 32 Kang H, Park KJ, Son S, Choi DS, Ryoo JW, Kwon OY et al. MRI in TolosaHunt syndrome associated with facial nerve palsy. Headache 2006; 46:3369. 33 Khan MA, Hashmi SM, Prinsley PR, Premachandra DJ. Reidels thyroiditis and TolosaHunt syndrome, a rare association. J Laryngol Otol 2004; 118:15961. 34 Koul R, Jain R. TolosaHunt syndrome: MRI before and after treatment. Neurol India 2003; 51:137. 35 Lai YH, Wang HZ, Tsai RK, Hoyt WF, Lee BF. Bilateral orbital pseudotumor with suprasellar and pulmonary involvement: report of a case. J Neuroophthalmol 2000; 20:26872. 36 Lee EJ, Jung SL, Kim BS, Ahn KJ, Kim YJ, Jung AK et al. MR imaging of orbital inammatory pseudotumors with extraorbital extension. Korean J Radiol 2005; 6:828. 37. Levin N, Karussis D. Inuence of progesterone and clomiphene on TolosaHunt syndrome. Neurology 2002; 59:16612. 38 Magrini L, Rotiroti G, Conti F, Viganego F, Alessandri C, Picardo V, Valesini G. Orbital myositis in a patient with primary biliary cirrhosis: successful treatment with methotrexate and corticosteroids. Isr Med Assoc J 2003; 5:8256. 39 Mark AS, Blake P, Atlas SW, Ross M, Brown D, Kolsky M. Gd-DTPA enhancement of the cisternal portion of the oculomotor nerve on MR imaging. Am J Neuroradiol 1992; 13:146370. 40 Mormont E, Laloux P, Vauthier J, Ossemann M. Radiotherapy in a case of TolosaHunt syndrome. Cephalalgia 2000; 20:9313. 41 Nabili S, McCarey DW, Browne B, Capell HA. A case of

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atypical idiopathic orbital myositis mimicking neurological disease. J Clin Neurosci 2001; 8:1645. Wasmeier C, Pfadenhauer K, Rosler A. Idiopathic inammatory pseudotumor of the orbit and TolosaHunt syndromeare they the same disease? J Neurol 2002; 249:123741. Watanabe A, Ishii R, Okamura H, Shirabe T. Magnetic resonance imaging of non-specic inammatory granulation involving the skull basetwo case reports. Neurol Med Chir (Tokyo) 1998; 38:1046. Yeung MC, Kwong KL, Wong YC, Wong SN. Paediatric TolosaHunt syndrome. J Paediatr Child Health 2004; 40:41013. Zournas C, Trakadas S, Kapaki E, Doris S, Gatzonis S, Gouliamos A, Papageorgiou C. Gadopentetate dimeglumine-enhanced MR in the diagnosis of the TolosaHunt syndrome. Am J Neuroradiol 1995; 16:9424.

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orbital myositis associated with rheumatoid arthritis. Ann Rheum Dis 2002; 61:9389. Nezu A, Kimura S, Osaka H. TolosaHunt syndrome with pseudotumor cerebri. Report of an unusual case. Brain Dev 1995; 17:21618. Odabasi Z, Gokcil Z, Atilla S, Pabuscu Y, Vural O, Yardim M. The value of MRI in a case of TolosaHunt syndrome. Clin Neurol Neurosurg 1997; 99:1514. Ohnishi J. Visual evoked potentials and magnetic resonance imagings of TolosaHunt syndrome before and after steroid therapy. Acta Neurol Scand 2002; 105: 2401. Ozawa T, Minakawa T, Saito A, Yoneoka Y, Yoshimura J, Arai H. MRA demonstration of periarteritis in Tolosa Hunt syndrome. Acta Neurochir (Wien) 2001; 143:30912. Palacios E, Valvassori G, Bresler M. TolosaHunt syndrome. Ear Nose Throat J 1999; 78:150. Panlio CB, Hernandez-Cossio O, Hernandez-Fustes OJ. Orbital myositis and rheumatoid arthritis: case report. Arq Neuropsiquiatr 2000; 58:1747. Singh NP, Garg S, Kumar S, Gulati S. Multiple cranial nerve palsies associated with type 2 diabetes mellitus. Singapore Med J 2006; 47:71215. Tatsumi H, Takeuchi Y, Hanaoka M, Okubo M, Kamata K. TolosaHunt syndrome in uraemic patients undergoing maintenance haemodialysis. Nephrol Dial Transplant 1998; 13:23702. Tessitore E, Tessitore A. TolosaHunt syndrome preceded by facial palsy. Headache 2000; 40:3936. Uehara F, Ohba N. Diagnostic imaging in patients with orbital cellulitis and inammatory pseudotumor. Int Ophthalmol Clin 2002; 42:13342. Ugur HC, Tascilar N, Atilla H, Yucemen N. A case of

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Supplementary material
The following supplementary material is available for this article online: A table showing the main data for each of the subjects from all the studies considered in this review. This material is available as part of the online article from http:/ /www.blackwell-synergy.com Please note: Blackwell Publishing is not responsible for the content or functionality of any supplementary materials supplied by the authors. Any queries (other than missing material) should be directed to the corresponding author for the article.

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