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REVIEW

ICHD-II diagnostic criteria for TolosaHunt syndrome inidiopathic inflammatory syndromes of the orbit and/or the

cavernous sinusS Colnaghi1,2,3, M Versino1,2,3, E Marchioni3,4, A Pichiecchio5, S Bastianello1,5, V Cosi1,4 & G Nappi3,6,71Department 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 6Scientific Direction, IRCCS Neurological Institute C. Mondino Foundation, Pavia, and7Department of Neurology and ENT, University La Sapienza, Roma, Italy

Colnaghi S, Versino M, Marchioni E, Pichiecchio A, Bastianello S, Cosi V &Nappi G. ICHD-II diagnostic criteria for TolosaHunt syndrome in idiopathicinflammatory syndromes of the orbit and/or the cavernous sinus. Cephalalgia

2008; 577584. London. ISSN 0333-1024A bibliographical search was conducted for papers published between 1999 and2007 to verify the validity of International Classification of Headache Disorders(ICHD)-II criteria for the TolosaHunt syndrome (THS) in terms of (i) the role ofmagnetic resonance imaging (MRI); (ii) which steroid treatment should beconsidered 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% ofthe cases and it normalized after clinical resolution. There was no evidence ofwhich steroid schedule should be considered as adequate; high-dose steroids arelikely 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 signsdid 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 andto 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, [email protected] Received 2 November 2007, accepted 22 December 2007

Introduction

TolosaHunt syndrome (THS) (1, 2) is a rare disor-der characterized by periorbital or hemicranial painaccompanied by diplopia, that may eventuallyrecur. The aetiology is still unknown, but the under-lying pathophysiological mechanism has beenproven to consist of a granulomatous inflammatoryprocess. THS was identified as a nosological entityby the International Headache Society classificationcriteria published in 1988 (3). These criteria weresubstantially revised in the 2004 classification (4)

(Table 1), and the literature was critically reviewedaccordingly (5).

In our opinion, there are several pending ques-tions regarding the appropriateness of the diagnos-tic criteria according to the present InternationalClassification of Headache Disorders (ICHD)-II,especially in terms of the best treatment choice, theduration of symptoms and signs and the anatomicaldistribution of lesions.

The classification criteria mention magnetic reso-nance imaging (MRI) explicitly for the diagnosisand implicitly for the differential diagnosis of THS

doi:10.1111/j.1468-2982.2008.01569.x

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(6, 7), but we think that a positive MRI performedwith appropriate techniques (812) should be man-datory for diagnosis.

The ICHD-II states in point D that adequatesteroid dosage should resolve disease symptomsand signs within 72 h from the treatment onset.

The adequate dosage of steroids and the bestroute of administration are undefined. The consis-tency of the 72-h period, as a time limit for reso-lution of symptoms and signs, does not seem tobe sustained by appropriate evidence in theliterature and, in our opinion, does not fit the realdisease course. Indeed, it seem to represent a criti-cal misleading point in the diagnostic process.Another question pertains to the anatomical dis-tribution of lesions for the diagnosis of THS.According to the ICHD-II criteria, all inflamma-tory 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, mostof the neurological and neuroradiological text-books reserve this diagnosis for those lesionslocated in the cavernous sinus, while they definethe inflammatory processes involving the orbit aspseudotumour. This discrepancy of terms anddefinitions limits the appropriateness of thediagnosis, prognosis and the best treatmentchoices.

Objectives

We set out to compare clinical features and outcomemeasures in discrete subgroups of THS patientsaccording 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 appro-priateness 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 loca-tions of lesions conditioning involvement ofdifferent structures (nerves, muscle or both)in the same paragraphs of the ICHD-II;

to provide useful propositions about the bestpharmacological treatment and the most appro-priate criteria for MRI follow-up.

For these reasons, we reviewed the reports onpatients suffering from THS who underwent MRI

Table 1 ICHD-II classification 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 untreatedB. 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 first

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 fissure or orbit in

some biopsied cases.Careful follow-up is required to exclude other possible causes of painful ophthalmoplegia.

578 S Colnaghi et al.

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examination, but we also considered some condi-tions that mimic, but cannot be classified as THSdue to the localization of the inflammatory process.

Methods

We conducted a bibliographical search on PubMed/Metacrawler and on Embase using the followingKeywords: Tolosa Hunt, Orbital pseudotumor,Idiopathic orbital inflammation, Painful ophthal-moplegia. The limits of the search were the Englishlanguage, and the date of publication between1 January 1999 and 31 January 2007.

To be considered further, the cases had to:

1. have been studied by means of MRI;2. fulfil the ICHD-II diagnostic criteria of THS with

the exception of point D, concerning symptomresolution within 72 h after starting steroidtreatment;

3. be as in point 2, but with an extension of theinflammatory process outside the orbit and thecavernous sinus;

4. be as in point 2, but with inflammation locatedin the ocular muscles only.

For each case we noted the following data: sex,age, side, symptoms (orbital pain, diplopia, visualloss, facial numbness or pain), signs (ocular motorpalsy, ocular motor palsy and visual loss, ocularmotor palsy and abnormal facial sensation, ocularmotor palsy and facial palsy), associated diseases,

MRI techniques, location of inflammatory tissue asdetected by MRI (coded as not detectable, cavern-ous sinus only, orbit with or without cavernoussinus, cavernous sinus and/or orbit with extensionto other intracranial structures, and extraocularmuscles), time elapsed between onset of symptomsand starting treatment, duration of clinical follow-up, duration of MRI follow-up.

We also considered the treatment schedules anddistinguished three groups: the regular steroiddosage group including patients who were treatedwith 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 of5001000 mg/day for a few days and then usuallyswitched to a regular regimen for a longer period,and treatments other than steroids.

Finally, for each subject we noted the followingoutcome features: pain resolution (coded as more orless than 72 h, and coded as number of days),symptoms resolution (coded as more or less than72 h, and coded as number of days), time of patho-logical tissue disappearance on MRI (coded as yes

or no, and coded as number of days), occurrence ofrecurrence (coded as yes or no).

On the basis of the location of inflammatorytissue as detected by MRI, the subjects weredivided into three diagnostic groups:

1. the THS group (no inflammatory tissue detect-

able, cavernous sinus only, orbital apex and/ororbit with or without cavernous sinus);

2. theTHSplus group (cavernous sinus and/or orbitwith extension to other intracranial structures);

3. the orbital myositis (OM) group (extraocularmuscles).

In the THS group we distinguished a furtherthree subgroups on the basis of the lesion site:

normal MRI subgroup; cavernous sinus subgroup (cavernous sinus

only);

orbital apex and/or orbit and cavernous sinussubgroup (orbit with or without cavernoussinus).

For each of the three diagnostic groups, the meanand standard deviation (SD) values of each of theabove-mentioned variables were computed. Wethen compared the mean values between groups bymeans of a KruskalWallis or MannWhitney testfor the continuous variables, and by means of a c2

test for the ordinal variables.In the THS group for each of the outcome vari-

ables (pain resolution, symptom resolution, time of

pathological tissue disappearance at MRI, occur-rence of recurrence), we compared the mean valuesbetween the cavernous sinus subgroup and theorbit and cavernous sinus subgroup.

Finally, considering all the subjects together, thepossible relationship between the kind of steroidtreatment (regular and high-dose) and the outcomevariables (sign disappearance, MRI normalization,and recurrence) was evaluated by means of c2 andMannWhitney tests.

The critical P-value was set at P = 0.01.

ResultsThe bibliographical search revealed 536 articles, 48 ofwhich met the criteria to be included (811, 1356).Overall we included 62 subjects extracted from 48reports: 38 in the THS group (three of which showeda normal MRI and three were not treated), 16 in theTHS plus group and eight in the OM group.

In the THS group, inflammation was detectableonly in the cavernous sinus in 23 (60.5%) patients,in the cavernous sinus and/or in the orbit in 12

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(31.6%) patients and was not detectable in three

(7.9%) patients only.The features of the individual reports describedin each of the papers included in this Review areavailable as supplementary on-line material, andTables 2 and 3 summarize the data.

In all the patients for whom these data wereavailable (30/62, 48.4%), the interval between painand ocular motor palsy onset was always < 2weeks, as required by the classification criteria.

The three diagnostic groups did not differ inmost of the variables (Tables 2 and 3), and thevariability of the data may partly account for thisnegative finding. The only exceptions were pain

resolution within the 72-h time limit, and recur-rence. Pain resolution within the 72-h time limitoccurred more frequently in the THS and THS plusthan in the OM group (Table 3), and recurrence wasmore likely in the THS plus and in the OM than inthe THS group (Table 3).

Although the pain resolved within 72 h afterstarting treatment, as required by the classificationcriteria, 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 statisticallysignificantly different in the three diagnostic groups(Table 2).

The mean duration of MRI follow-up was notstatistically significantly longer (MannWhitney U110, z = -0.08, P = 0.933) in patients who showednormalization (n = 16; 230 days) than in those whodid not (n = 14; 448 days), but the normalization ofMRI always lasted longer than sign resolution in allthree diagnostic groups (Table 2).

When the outcome variables were compared inthe two THS subgroups (the cavernous sinus sub-group and the orbit and cavernous sinus subgroup),

no significant difference was found.Fifty-four (87%) patients underwent steroid treat-

ment, five (8%) underwent treatment other thansteroids (including surgical removal of the inflam-matory tissue) and three (5%) were not treated.Treatments most often lasted about 2 months, butthe duration could range from a few days to severalyears.

By considering all the patients together, the meantime needed for sign disappearance was longer

Table 2 For each variable the table shows the mean and the standard deviation (SD) values, and the range for eachdiagnostic group, and the results of the KruskalWallis test (KW) for mean comparison

Diagnostic group n (%) Mean SD Minmax KW

Age (years) THS 37 (97) 40.73 16.29 973 2.73 P = 0.25

THS plus 16 (100) 39.69 22.19 875

OM 8 (100) 28.38 14.91 951

Treatment delay (days) THS 25 (66) 17.84 39.74 0180 1.26 P = 0.53

THS plus 10 (63) 30.30 73.90 0240

OM 8 (100) 14.25 20.36 056

Clinical follow-up (days) THS 31 (82) 352.77 577.86 102880 2.16 P = 0.34

THS plus 14 (9) 539.14 642.83 561950

OM 8 (100) 565.50 861.14 282520

MRI follow-up (days) THS 21 (55) 276.00 641.22 102880 2.66 P = 0.26

THS plus 11 (69) 528.00 746.46 302310

OM 3 (38) 79.33 63.26 28150

Pain resolution (days) THS 6 (16) 47.17 94.580 3240 1.144 P = 0.564

THS plus 2 (13) 16.50 19.09 330

OM 2 (25) 35.00 29.70 1456

Signs resolution (days) THS 26 (68) 100.27 281.668 71440 1.424 P = 0.491THS plus 7 (44) 41.71 61.73 3180

OM 3 (38) 73.33 69.64 14150

MRI normalization (days) THS 13 (34) 352.31 769.983 102880 0.416 P = 0.812

THS plus 9 (56) 332.00 496.84 301440

OM 3 (38) 81.67 60.48 35150

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.



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after regular (n = 27; 104 days) than after high-dose

steroid treatment (n = 8; 35.5 days), but these twofigures did not prove to be statistically significant(MannWhitney; P = 0.686). The time to reach MRInormalization showed similar behaviour, beinglonger after regular (n = 20, mean 369.5 days, range102880 days) than after high-dose treatment (n = 3;mean 119.3 days, range 10320 days), but again thisdifference was not statistically significant (MannWhitney U 17.5, z = 1.14, P = 0.253).

Recurrence occurred in 19 (44.2%) patients whounderwent regular steroid treatment and in onlyone (9.1%) of those treated with high-dose steroid

(c2

= 4.62, Fishers exact test P = 0.039).

Discussion

We were able to consider 62 patients derived frompapers about single or a few cases with someunusual features. However, these features variedfrom one subject to another, and we consider that asa group these patients are likely to be representativeof THS, with the only exception that they do notneed to fulfil the 72 h diagnostic criteria.

Some points clearly stand out:

1. The classification criteria require that both symp-toms and signs resolve within 72 h after start-ing adequate steroid treatment. Unfortunately,the classification criteria do not state whatadequate means, and the literature reports indi-vidualized rather than standardized treatment.In addition, in only 2% of the patients did thesigns resolve so quickly.

2. MRI showed the presence of inflammatory tissuein 92.1% of THS patients: this, and the clinicaloverlap of THS with other conditions such asthose we labelled as THS plus and OM, strongly

supports the importance of MRI for the diagno-sis of THS.

3. The localization or extension of the inflammatorytissue does not seem to be a prognostic factor,and, more specifically, the patients from the THSplus group and those from the THS group behavesimilarly. It may be suggested to amalgamate thetwo groups. Furthermore, neuroradiological dis-tinction between the THS syndrome (inflamma-tory tissue within the cavernous sinus) and the

Table 3 For each variable the table shows for each diagnostic group the number of cases and the correspondingpercentage with respect of the total number of that group, and the results of the c2 test

THS THS plus OM c2

Sex Female 17 (45%) 9 (56%) 6 (75%) 2.6

Male 21 (55%) 7 (44%) 2 (25%) P = 0.27

Side Bilateral 2 (5%) 2 (12%) 1 (13%) 2.52Left 18 (48%) 8 (50%) 2 (25%) P = 0.64

Right 18 (48%) 2 (25%) 5 (62%)

Symptoms Pain and diplopia 24 (63%) 11 (69%) 8 (100%) 12.27

Visual loss 4 (10%) 58 (31%) 0 (0%) P = 0.06

Facial numbness 9 (24%) 0 (0%) 0 (0%)

Facial palsy 1 (3%) 0 (0%) 0 (0%)

Steroid treatment Regular 26 (74%) 11 (69%) 6 (75%) 10.34

High dosage 9 (26%) 2 (12%) 0 (0%) P = 0.03

Other 0 (0%) 3 (19%) 2 (25%)

Clinical follow-up Yes 29 (91%) 14 (100%) 6 (75%) 3.79

No 3 (9%) 0 (0%) 2 (25%) P = 0.15

MRI follow-up Yes 25 (66%) 11 (69%) 3 (37%) 2.58

No 13 (34%) 5 (31%) 5 (62%) P = 0.27

Pain resolution < 72 h 18 (78%) 5 (62%) 0 (0%) 9.310

> 72 h 5 (22%) 3 (38%) 4 (100%) P = 0.010

Signs resolution < 72 h 0 (0%) 1 (8%) 0 (0%) 2.595

> 72 h 28 (100%) 12 (92%) 5 (100%) P = 0.273

MRI normalization Yes 10 (50%) 4 (36%) 3 (100%) 3.818

No 10 (50%) 7 (64%) 0 (0%) P = 0.148

Recurrences Yes 8 (21%) 11 (69%) 4 (59%) 11.633

No 30 (79%) 5 (31%) 4 (50%) P = 0.003



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pseudotumor orbitae needs further investiga-tion to prove its clinical significance.

4. MRI data show that the disappearance of in-flammatory tissue takes longer than symptomresolution, and that pathological tissue may notdisappear even after several months. Accordingly,it may be suggested that MRI should be used tomonitor the disease course and to support thedecision of when treatment should be stopped.

However, the question of whether THS may havea relapsing course or may become a chronic condi-tion, and whether these two outcomes depend ontreatment, is left open.

Our review showed that the choice of treatmentvaried in terms of the kind of steroid, dosage,administration method and duration; but alsosuggested that recurrence is less likely in patientswith high-dose than in those with regular steroidtreatment.

Conclusion and proposal

Overall, in our opinion the classification criteria forTHS syndrome could benefit from the points dis-cussed above: (i) there is no evidence of what kindof steroid treatment is adequate; (ii) the 72-h timelimit should be applied for pain resolution only; (iii)MRI plays a key role in diagnosis; and (iv) inflam-matory tissue can extend beyond the cavernoussinus and the orbit. Points (iii) and (iv) imply thatwe need to use MRI techniques specifically aimed

at showing the presence of inflammatory tissue, assuggested by Cakirer (8), La Mantia (5) and our-selves (9). The diagnosis of THS cannot rely only onMRI data, but these should be used in conjunctionwith clinical findings both to make the diagnosisand to follow-up patients suffering from THS and,in some cases, a biopsy will still be needed toascertain the nature of the pathological tissue.

The classification criteria could be modified asfollows:

A. One or more episodes of unilateral orbital painpersisting for weeks if untreated

B. Paresis of one or more of the third, fourthand/or sixth cranial nerves and demonstrationof granulomatous material in the cavernoussinus, superior orbital fissure or orbit by MRI

C. Paresis coincides with the onset of pain orfollows it within 2 weeks

D. Pain resolves within 72 h when treated withcorticosteroids

E. Other causes have been excluded by appropriateinvestigations.

Notes

1. NRI should be performed with 3 mm thicknesscoronal and axial T1-SE and T2-TSE sequencesand with T1 fat-suppressed weighted imagesafter godolinium administration at the level ofthe orbit and cavernous sinus.

2. Other causes of painful ophthalmoplegia includevascular, neoplastic, infectious, systemic granu-lomatous diseases, basal meningitis, diabetesmellitus, ophthalmopletic migraine, thyroid oph-thalmopathy and trauma.

Comments

1. Some reported cases of THS had additionalinvolvement of the trigeminal nerve (commonlythe first division) or optic, facial or acoustic

nerves. Sympathetic innervation of the pupil isoccasionally affected.2. The syndrome is caused by granulomatous

material in the cavernous sinus, superior orbitalfissure or orbit in some biopsied cases; granulo-matous material can have an intracranial exten-sion, as demonstrated by MRI.

3. Careful clinical and MRI follow-up is required toconfirm the diagnosis and to manage the steroidtreatment.

It might also be beneficial to distinguish betweendefinite THS, when all the criteria are met, and

possible THS, when all the criteria but the one ofpoint D are met.

These criteria implicitly suggest that THS is anidiopathic disorder whose pathophysiology consistsof a granulomatous inflammatory process, and thatTHS could be moved from the cranial neuralgiasection to the secondary headache section.

In conclusion, further multicentre studies arenecessary to improve our knowledge of THS, butthe revision of previous papers can suggest somechanges to improve the classification criteria.

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Supplementary material

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A table showing the main data for each of the subjectsfrom all the studies considered in this review.

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Please note: Blackwell Publishing is not responsible forthe content or functionality of any supplementary materialssupplied by the authors. Any queries (other than missingmaterial) should be directed to the corresponding authorfor the article.


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