THS Jurnal

7/27/2019 THS Jurnal

1/7

doi: 10.1136/jnnp.71.5.5772001 71: 577-582J Neurol Neurosurg Psychiatry

L B Kline and W F HoytThe Tolosa-Hunt syndrome

http://jnnp.bmj.com/content/71/5/577.full.htmlUpdated information and services can be found at:

These include:

References

http://jnnp.bmj.com/content/71/5/577.full.html#related-urlsArticle cited in:

http://jnnp.bmj.com/content/71/5/577.full.html#ref-list-1This article cites 42 articles, 8 of which can be accessed free at:

serviceEmail alerting

box at the top right corner of the online article.Receive free email alerts when new articles cite this article. Sign up in the

CollectionsTopic

(1141 articles)Radiology (diagnostics)(1524 articles)Radiology

(367 articles)Headache (including migraine)(1569 articles)Immunology (including allergy)

(627 articles)Pain (neurology)(719 articles)Ophthalmology(453 articles)Cranial nerves

Articles on similar topics can be found in the following collections

Notes

http://group.bmj.com/group/rights-licensing/permissionsTo request permissions go to:

http://journals.bmj.com/cgi/reprintformTo order reprints go to:

http://group.bmj.com/subscribe/To subscribe to BMJ go to:

group.bmj.comon October 11, 2013 - Published byjnnp.bmj.comDownloaded from
http://jnnp.bmj.com/content/71/5/577.full.htmlhttp://jnnp.bmj.com/content/71/5/577.full.htmlhttp://jnnp.bmj.com/content/71/5/577.full.html#related-urlshttp://jnnp.bmj.com/content/71/5/577.full.html#related-urlshttp://jnnp.bmj.com/content/71/5/577.full.html#ref-list-1http://jnnp.bmj.com/content/71/5/577.full.html#ref-list-1http://jnnp.bmj.com/cgi/collection/radiologyhttp://jnnp.bmj.com/cgi/collection/radiologyhttp://jnnp.bmj.com/cgi/collection/radiologyhttp://jnnp.bmj.com/cgi/collection/radiologyhttp://jnnp.bmj.com/cgi/collection/radiologyhttp://jnnp.bmj.com/cgi/collection/radiology2http://jnnp.bmj.com/cgi/collection/headache_including_migrainehttp://jnnp.bmj.com/cgi/collection/radiology2http://jnnp.bmj.com/cgi/collection/headache_including_migrainehttp://jnnp.bmj.com/cgi/collection/headache_including_migrainehttp://jnnp.bmj.com/cgi/collection/immunology_including_allergyhttp://jnnp.bmj.com/cgi/collection/immunology_including_allergyhttp://jnnp.bmj.com/cgi/collection/immunology_including_allergyhttp://jnnp.bmj.com/cgi/collection/pain2http://jnnp.bmj.com/cgi/collection/pain2http://jnnp.bmj.com/cgi/collection/pain2http://jnnp.bmj.com/cgi/collection/ophthalmologyhttp://jnnp.bmj.com/cgi/collection/ophthalmologyhttp://jnnp.bmj.com/cgi/collection/ophthalmologyhttp://group.bmj.com/group/rights-licensing/permissionshttp://group.bmj.com/group/rights-licensing/permissionshttp://journals.bmj.com/cgi/reprintformhttp://journals.bmj.com/cgi/reprintformhttp://group.bmj.com/subscribe/http://group.bmj.com/http://group.bmj.com/http://group.bmj.com/http://jnnp.bmj.com/http://group.bmj.com/http://jnnp.bmj.com/http://group.bmj.com/subscribe/http://journals.bmj.com/cgi/reprintformhttp://group.bmj.com/group/rights-licensing/permissionshttp://jnnp.bmj.com/cgi/collection/radiologyhttp://jnnp.bmj.com/cgi/collection/radiology2http://jnnp.bmj.com/cgi/collection/headache_including_migrainehttp://jnnp.bmj.com/cgi/collection/immunology_including_allergyhttp://jnnp.bmj.com/cgi/collection/pain2http://jnnp.bmj.com/cgi/collection/ophthalmologyhttp://jnnp.bmj.com/cgi/collection/cranial_nerveshttp://jnnp.bmj.com/content/71/5/577.full.html#related-urlshttp://jnnp.bmj.com/content/71/5/577.full.html#ref-list-1http://jnnp.bmj.com/content/71/5/577.full.html


2/7


3/7

not consistently performed. In addition, veno-graphic abnormalities are not specific forTolosa-Hunt syndrome, and may be foundwith space occupying lesions or other inflam-matory processes in the orbit or parasellarregion. With the advent of CT and MRI directvisualisation of the cavernous sinus is possible.Using these neuroimaging modalities, cavern-ous sinus abnormalities have now been de-scribed in Tolosa-Hunt syndrome (see below).

Clinical profile of Tolosa-Hunt syndromeTolosa-Hunt syndrome can aVect people ofvirtually any age from the 1st to the 8thdecades of life, with no sex predilection. Eitherside may be aVected, with case reports of bilat-eral simultaneous involvement. Uniformly,patients complain of pain, which is a definingsymptom. The pain lasts an average of 8 weeksif untreated. Ocular motor cranial nerve palsiesmay coincide with the onset of pain or follow itwithin a period of up to 2 weeks. 20 It is usuallydescribed as intense, severe, boring,lancinating, or stabbing. It is periorbital inlocation, frequently extending into the retro-

orbital, frontal, and temporal regions.All three ocular motor cranial nerves may beinvolved, in various combinations. Pupillaryreactions may be normal, or there may beeither parasympathetic (oculomotor nerve) orsympathetic (third order neuron Horners syn-drome) involvement.

At times, optic nerve dysfunction has beenreported with Tolosa-Hunt syndrome, indicat-ing that the pathological process may involvethe orbital apex.57 21 22 The optic disc may benormal, swollen, or pale in appearance, andvisual decline may be minimal or lead to blind-ness. Loss of acuity is variable and unpredict-able, and, on occasion, may be permanent.

Infrequently, other cranial nerves not located

within the cavernous sinus/superior orbital fis-sure may be aVected. These include the maxil-lary1 6 1 7 and mandibular branch of the trigemi-nal nerve,6 23 and the facial nerve.11 23

There are no reports of systemic or otherneurological involvement in Tolosa-Hunt syn-drome, with the exception of nausea and vom-iting. These symptoms are probably due to theintense periorbital pain; they promptly resolvewith cessation of pain.

Natural historyAs Hunt outlined in his diagnostic criteria,3

Tolosa-Hunt syndrome follows an unpredict-able course. Usually acute at onset, the

patients symptoms typically last from days toweeks. Before the use of systemic cortico-steroids there was clear evidence that sponta-neous remissions occurred. However, recur-rences are common, taking place in about a halfof reported patients, usually at an interval ofmonths or years from the initial attack.12 24

These recurrences may be ipsilateral, contra-lateral, or rarely, bilateral.

Although Tolosa-Hunt syndrome is a selflimited illness, it does cause considerable mor-bidity. Infrequently, residual cranial nerve pal-sies persist. With the institution of cortico-steroid therapy, the natural course is altered.

Although there is no conclusive evidence toshow that steroids lessen the degree or durationof the ophthalmoplegia, there is dramaticreduction of pain, often within 24 hours.

Diagnostic evaluationWith careful clinical examination, pain associ-ated with typical cranial nerve palsies localisesthe pathological process to the regions of the

cavernous sinus/superior orbital fissure. Asnoted above, current neuroimaging modalitiesallow visualisation of the area of suspectedpathology. Contrast enhanced MRI with multi-ple views, particularly coronal sections, shouldbe the initial diagnostic study performed.Numerous reports have demonstrated an areaof abnormal soft tissue in the region of the cav-ernous sinus in most, but not all, patients withTolosa-Hunt syndrome.2529 Typically, the ab-normality is seen as an intermediate signalintensity on T1 and intermediate weightedimages, consistent with an inflammatory proc-ess. In addition, there is enhancement of theabnormal area after intravenous injection of

paramagnetic contrast. With corticosteroidtherapy, the abnormal area decreases in volumeand signal intensity in most reported cases.

High resolution CT can also demonstratesoft tissue changes in the region of the cavern-ous sinus/superior orbital fissure, but is lesssensitive than MRI.25 30 This is due to lack ofsensitivity to soft tissue change with superim-posed beam hardening and bone streak arti-facts. Thus, even if CT is normal, MRI muststill be performed to appropriately evaluate theregion of the cavernous sinus or superiororbital fissure.

The major limitation of MRI findings inTolosa-Hunt syndrome is their lack of specifi-city. Thus, Yousem et al25 examined 11 patients

and reported pathological MRI findings in thecavernous sinus in nine. In six of these nine theaVected cavernous sinus was enlarged;in five ofnine it had a convex lateral wall. Extension intothe orbital apex was seen in eight patients. Yetthe signal characteristics (hypointense relativeto fat and isointense with muscle on T1weighted images; isointense with fat on T2weighted scans) were also consistent withmeningioma, lymphoma, and sarcoidosis.

Because of this fundamental limitation ofinitial imaging studies, some authorities wouldsuggest that resolution of imaging abnormali-ties after a course of systemic corticosteroidsshould be considered diagnostic of Tolosa-

Hunt syndrome.

29 31

However, caution is ad-vised when assessing the salutary eVects ofsteroids, as improvement both clinically andradiologically may occur with other diseaseprocesses. False positive steroid responsive-ness with a remitting course can be seen withboth malignant processes (for example,lymphoma) as well as more benign disorders(for example, vasculitis).20

Categorically, Tolosa-Hunt syndrome is adiagnosis of exclusion requiring careful patientevaluation (table 1) to rule out tumour, vascu-lar causes, or other forms of inflammation inthe region of the cavernous sinus/superior

578 Kline,Hoyt

www.jnnp.com


4/7

orbital fissure (see diVerential diagnosisbelow).

Occasional reports have documented araised erythrocyte sedimentation rate andleukocytosis in the acute stage of Tolosa-Huntsyndrome.46 17 22 Similarly, positive LE cellpreparation and antinuclear antibody concen-trations5 32 have been documented in patientswith Tolosa-Hunt syndrome, but there is noconvincing evidence that such patients eitherhave or will develop connective tissue disorder.

In general, CSF examination should also beunremarkable, although rarely raised proteinand mild pleocytosis have been reported. How-

ever, if CSF abnormalities persist, the diagno-sis of Tolosa-Hunt syndrome is untenable, andfurther diagnostic evaluations are required.

As noted above, MRI is the initial diagnosticstudy to be performed in patients with a disor-der of the cavernous sinus or superior orbitalfissure. Computed tomography is a very usefuladjunct in detecting bone changes (for exam-ple, erosion, hyperostosis) as well as (perisellar)calcification. Cerebral angiography has de-tected abnormalities in the intracavernouscarotid artery in patients with Tolosa-Huntsyndrome.57 17 18 23 33 These have been de-scribed as segmental narrowing, slightirregularity, or constriction, and will resolve

with corticosteroid therapy. The role of non-invasive vascular MRI techniques (MR angio-graphy and venography) has not been definedin patients with the syndrome but these mayprove to be valuable adjuncts in diagnosticevaluation.

Neurosurgical biopsy is only rarely employedto establish the diagnosis.57 12 17 33 This can betechnically diYcult and should only be per-formed by experienced neurosurgeons. It usu-ally involves biopsy of the dural wall of the cav-ernous sinus. Although generally a procedureof last resort, it should be considered inpatients with rapidly progressive neurological

deficits, lack of steroid responsiveness, orpersistent abnormalities on neuroimagingstudies.

TreatmentAlmost 40 years ago Hunt first documentedthe beneficial eVect of corticosteroid therapy inTolosa-Hunt syndrome.3 Unfortunately, sincethen there is little new information as tooptimal dosage, duration of treatment, or alter-

native forms of therapy. It is clear that sponta-neous remissions may occur, but there is nodoubt that corticosteroids markedly reduce theperiorbital pain. No data are available as towhether treatment hastens recovery of theassociated cranial nerve palsies. Although ster-oids are generally tapered over weeks tomonths, in some cases prolonged therapy maybe necessary. As cautioned previously, positiveresponse to steroids has been reported in vari-ous parasellar neoplasms, including chordoma,giant cell tumour, lymphoma, and epider-moid.24

Pathology

Tolosa-Hunt syndrome is caused by an inflam-matory process. Tolosa2 originally describednon-specific, chronic inflammation with prolif-eration of fibroblasts and infiltration of thesepta and wall of the cavernous sinus with lym-phocytes and plasma cells. Hunt et al3 corrobo-rated these findings, emphasising the lack ofnecrosis and pointed out that . . .such inflam-matory changes, in a tight connective tissue,may exert pressure upon the penetratingnerves. Subsequent reports have showngranulomatous inflammation, with epithelioidcells and occasional giant cells.6 12 Necrosismay also be seen. There have been no reportsof an infectious organism associated withTolosa-Hunt syndrome.

AetiologyThe aetiology of Tolosa-Hunt syndrome re-mains unknown. No information is available asto what triggers the inflammatory process inthe region of the cavernous sinus/superiororbital fissure. It seems that the syndrome fallswithin the range of idiopathic orbital inflam-mation (pseudotumour).34 Thus, non-specific inflammation typically causes anacute orbitopathy. If located more posteriorly,the inflammatory process involves the cavern-ous sinus/superior orbital fissure, producingthe Tolosa-Hunt syndrome. Rarely, the inflam-mation may spread intracranially.3537

DiVerential diagnosisBecause the diagnosis of Tolosa-Hunt syn-drome can been made only after other diseaseprocesses have been excluded, it behoves theclinician to be familiar with the diVerentialdiagnosis of painful ophthalmoplegia. In fact,during the initial patient evaluation there areoften no clues in the history or physical exam-ination to distinguish Tolosa-Hunt syndromefrom other causes of painful ophthalmoplegia.Therefore, the clinician should be aware of: (1)causes of parasellar syndrome and (2) otherentities producing painful ophthalmoplegia.

Table 1 Diagnostic evaluation of Tolosa- Hunt syndrome

Haematological tests:Complete blood countSerum chemistry (glucose, electrolytes, liver and renalfunction)Erythrocyte sedimentation rateC reactive proteinHaemoglobin A1CFluorescent treponemal antibody testAntinuclear antibodyAnti-dsDNA antibodyAnti-sm antibody

Serum protein electrophoresisAntinuclear cytoplasmic antibodyAngiotensin converting enzyme

Cerebrospinal fluid:Opening pressureCell count and diVerentialProteinGlucoseCulture: bacterial, fungal, mycobacterialSerologyAngiotensin converting enzymeCytology

Neuroradiologial studiesMRICTCerebral angiography

Biopsy:NasopharynxCavernous sinus

The Tolosa-Hunt syndrome 579

www.jnnp.com


5/7

CAUSES OF PARASELLAR SYNDROME (TABLE 2)Craniocerebral trauma may produce painfulophthalmoplegia in various ways: basilar skull

fracture with ocular motor nerve damage,intracavernous carotid artery injury with sub-sequent aneurysm formation, or carotid-cavernous fistula.

Various vascular causes may produce painfulophthalmoplegia, the most frequent being anintracavernous carotid artery aneurysm.38 39

Rarely, giant aneurysms of the posterior circu-lation, including the posterior cerebral artery,may mimic Tolosa-Hunt syndrome.40 Carotid-cavernous fistula and cavernous sinus throm-bosis also have acute onset of symptoms andsigns, and must be included in the diVerentialdiagnosis. Patients with carotid-cavernous fis-tulae rarely complain of severe pain and

typically have dramatic ophthalmological signsincluding proptosis, arterialised conjunctivalvessels, chemosis, increased intraocular pres-sure, and retinal vascular abnormalities. Abruit is often present, cerebral angiography isthe diagnostic procedure of choice, and inter-ventional angiographic techniques are usuallycurative. Cavernous sinus thrombosis may beseptic or aseptic in origin. In the first,there maybe associated signs of sinusitis, otitis, gingivitis,or orbital cellulitis. Patients are febrile, have aleukocytosis, and if the infectious processspreads intracranially, seizures and alteredmental states may occur. Aseptic cavernous

sinus thrombosis may be associated withvarious conditions including polycythemia,sickle cell disease, vasculitis, pregnancy, dehy-dration, trauma, and intracranial surgery.Regardless of aetiology, cavernous sinusthrombosis is characterised by orbital conges-tion, proptosis, eyelid swelling, chemosis, lacri-mation, and ophthalmoparesis. Pain around orbehind the eye is common. Treatment ofcavernous sinus thrombosis usually involves

anticoagulant drugs, at times thrombolyticagents, and in septic cases, appropriate anti-biotic therapy.

As outlined in table 2, painful ophthalmople-gia may be caused by either contiguous ormetastatic spread of a neoplasm. Metastaticinvolvement of the cavernous sinus/superiororbital fissure is generally due to haematog-enous dissemination of neoplastic cells.41 Occa-sionally, intracranial neoplastic invasion mayoccur by intraneural or perineural spread, as inthe case of squamous cell carcinoma producingpainful ophthalmoplegia many months afterlocal excision of a facial skin tumour.42

Two aspects of neoplastic involvement of the

cavernous sinus/superior orbital fissure requireemphasis. Firstly, it is important to realise thatmode of onset and clinical course are notindicative of the type of lesion causing painfulophthalmoplegia. In their review of parasellarsyndrome, Thomas and Yoss43 state that Asudden onset of symptoms does not weigh infavor of aneurysmal or against neoplasticorigin, and gradual onset is not necessarilyindicative of a neoplasm . . .spontaneous singleor multiple remissions of symptoms, even ofyears duration, are unreliable in predicting thenature of the underlying process. Secondly, asnoted above, high dose corticosteroid therapymay initially improve signs and symptoms dueto neoplasm.

Inflammatory causes of painful ophthalmo-plegia include those due to a specific infectiousagent. It is essential that careful CSF examina-tion be done and that cultures (bacterial,fungal, mycobacterial) be obtained. The poten-tial role of a paranasal sinus as a cause of pain-ful ophthalmoplegia requires attention. Sinusdisease may lead to cavernous sinus involve-ment, either via contiguous spread of infectionor due to sphenoid sinus mucocele.

OTHER CAUSES OF PAINFUL OPHTHALMOPLEGIA

Table 3 summarises other aetiologies of painfulophthalmoplegia in which there is no involve-ment of the cavernous sinus/superior orbital

fissure.Various orbital diseases cause painful oph-thalmoplegia. Typically, the patient presentswith orbital signs, including proptosis, con-junctival injection, chemosis, and resistance toretrodisplacement of the globe. In addition, theeye may be displaced within the orbit, andthere may be abnormalities of the ocularadnexa (for example, lids, lacrimal gland).

Diabetic ophthalmoplegia typically producesan acute, often painful mononeuropathy ineither a known or previously undiagnosed dia-betic person. Invariably there is recovery ofocular motor cranial nerve function, usually

Table 2 Causes of parasellar syndrome producing painfulophthalmoplegia

(1) Trauma:(2) Vascular:

Intracavernous carotid artery aneurysmPosterior cerebral artery aneurysmCarotid-cavernous fistulaCarotid-cavernous thrombosis

(3) Neoplasm:Primary intracranial tumour

Pituitary adenomaMeningioma

CraniopharyngiomaSarcomaNeurofibromaGasserian ganglion neuromaEpidermoid

Primary cranial tumourChordomaChondromaGiant cell tumour

Local metastasesNasopharyngeal tumourCylindromaAdamantinomaSquamous cell carcinoma

Distant metastasesLymphomaMultiple myelomaCarcinomatous metastases

(4) Inflammation:Bacterial

Sinusitis

MucocelePeriostitis

ViralHerpes zoster

FungalMucormycosis

SpirochetalTreponema pallidum

MycobacterialMycobacterium tuberculosis

Unknown cause:SarcoidosisWegeners gra nulomatosisEosinophilic granulomaTolosa-Hunt s yndrome

580 Kline,Hoyt

www.jnnp.com


6/7

within 3 months. In addition, there are reportsof diabetic patients with simultaneous paralysisof multiple ocular motor nerves.44 45 Fre-quently, these episodes are painful, oftenrecurrent, and not particularly responsive tocorticosteroid therapy.

Posterior fossa aneurysms may produceeither acute, painful ophthalmoplegia, or maypresent in a more subacute or chronic fashion.The acute presentation is most often due to an

aneurysm in the anterior circulation, typicallyat the junction of the internal carotid-posteriorcommunicating arteries, whereas the subacutepresentation is caused by basilar artery aneu-rysms. In both, cerebral angiography is diag-nostic.

Giant cell arteritis may also produce painfulophthalmoplegia. The clinical picture may beone of single or multiple ocular motor nervepalsies. In the only pathological study ofophthalmoplegia occurring in giant cell arteri-tis, ischaemic necrosis of the extraocular mus-cles was demonstrated. Examination of theocular and motor cranial nerves was unremark-able.46

Ophthalmoplegic migraine is a poorly un-

derstood form of complicated migraine.47

Typically, this clinical syndrome occurs in achild or young adult with periodic headache,who develops an ocular motor cranial nervepalsy at the height of an attack of cephalgia,which is primarily unilateral and in the orbitalregion. Most often involved is the oculomotornerve, occasionally the abducens nerve, andrarely the trochlear nerve. The paresis lasts fordays to weeks after cessation of a headache;recovery is gradual and tends to be lesscomplete after repeated attacks. A familyhistory of migraine is usually obtained. Thereare reports of enhancement of the extra-axialportion of the oculomotor nerves of patients

with ophthalmoplegic migraine when evalu-ated with contrast-enhanced MRI.4850

ConclusionAlthough the pathogenetic basis of Tolosa-Hunt syndrome remains unknown, from apractical clinical standpoint it can be regardedas a distinct entity which may be simulated byvarious other disorders. It cannot be empha-sised too strongly that patients suspected ofhaving the syndrome require careful evalua-tion, appropriate treatment, and scrupulousfollow up observation.

The work is supported by an unrestricted grant from Researchto Prevent Blindness Inc, New York, USA.

1 Smith JL, Taxdal DSR. Painful ophthalmoplegia. theTolosa-Hunt syndrome. Am J Ophthalmol 1966;61:146672.

2 Tolosa E. Periarteritic lesions of the carotid siphon with theclinical features of a carotid infraclinoid aneurysm. J Neu-rol Neurosurg Psychiatry 1954;17:3002.

3 Hunt WE, Meagher JN, Le Fever HE, et al. Painful ophthal-moplegia. Its relation to indolent inflammation of the cav-ernous sinus. Neurology 1961;11:5662.

4 Lakke JPWF. Superior orbital fissure syndrome. Report of acase caused by local pachymeningitis. Arch Neurol 1962;7:289300.

5 Mathew NT, Chandy J. Painful ophthalmoplegia. J NeurolSci 1970;11:24356.

6 Schatz NJ,Farmer P: Tolosa-Hunt syndrome: the pathologyof painful ophthalmoplegia. In: Smith JL, ed. Neuro-ophthalmology.Symposium of the University of Miami and theBascom Palmer Eye Institute. Vol VI. St Louis: Mosby, 1972:10212.

7 Spinnler H. Painful ophthalmoplegia; the Tolosa-Hunt syn-drome. Med J Aust1973;2:6456.

8 Roca PD. Painful ophthalmoplegia: the Tolosa-Huntsyndrome. Ann Ophthalmol 1975;7:82834.

9 Cohn DF, Carasso R, Streifler M. Painful ophthalmoplegia.Eur Neurol 1979;18:37381.

10 Muhletaler CA, Gerlock AJ. Orbital venography in painfulophthalmoplegia (Tolosa-Hunt syndrome). Am J Radiol1979;133:314.

11 Inzafari D, Sita D, Marconi GP, et al. The Tolosa-Huntsyndrome: further clinical and pathogenetic considerationsbased on the study of eight cases. J Neurol1981;224:2218.

12 Aron-Rosa D, Doyon D, Sallamon G, et al. Tolosa-Hunt

syndrome. Ann Ophthalmol 1978;10:11618.13 Goadsby PJ, Lance JW. Clinicopathological correlation in acase of painful ophthalmoplegia: Tolosa-Hunt syndrome. J

Neurol Neurosurg Psychiatry 1989;52:12903.14 Hunt WE. Tolosa-Hunt syndrome: one cause of painful

ophthalmoplegia. J Neurosurg 1976;44:5449.15 Van Dalen JTW, Blecker GM. The Tolosa-Hunt syndrome.

Doc Ophthalmol 1977;44:16772.16 Milstein BA, Morretia LB. Report of a case of sphenoid fis-

sure syndrome studied by orbital venography. Am JOphthalmol 1971;72:6003.

17 Sondheimer FK, Knapp J. Angiographic findings in theTolosa-Hunt syndrome: painful ophthalmopleg ia. Radiol-ogy 1973;106:10512.

18 Julien J, Vallat JM, Vallat M, et al. Orbital phlebography inthe diagnosis of painful ophthalmoplegia. Neuroradiology1975;9:1636.

19 Hannetz J. Recurrent Tolosa-Hunt syndrome: a report of 10new cases. Cephalgia 1999;19 (suppl 25):335.

20 Frderreuther S, Straube A. The criteria of the Inter-national Headache Society for Tolosa-Hunt syndromeneed to be revised. Neurol1999;246:3717.

21 Anderson BI. Unusual course of painful ophthalmoplegia.

Report of a case. Acta Ophthalmol 1980;58:8418.22 Zafrulla KM, Dhir SP, Jain IS. Tolosa-Hunt syndrome. IndJ Opht halmol 1977;25:216.

23 Adams AH, Warner AW. Painful ophthalmoplegia: report ofa case with cerebral involvement and psychiatric complica-tions. Bull Los Angeles Neurol Soc 1975;40:4955.

24 Kline LB. The Tolosa-Hunt syndrome. Surv Ophthalmol1982;27:7995.

25 Yousem DM, Atlas SW, Grossman RI, et al. MR imaging ofTolosa-Hunt syndrome. AJNR Am J Neuroradiol 1990;10:11814.

26 Gato Y, Hosokawa S, Goto I, et al. Abnormality in the cav-ernous sinus in three patients with Tolosa-Hunt syndrome:MRI and CT findings. J Neurol Neurosurg Psychiatry 1990;53:2314.

27 Desai SP, Carter J, Jinkins JR. Contrast-enhanced MRimaging of Tolosa-Hunt syndrome: a case report. AJNR

Am J Neuroradiol 1991;12:1823.28 Obadasi Z, Gkeil Z, Atilla S, et al. The value of MRI in a

case of Tolosa-Hunt syndrome. Clin Neurol Neurosurg1997;99:1514.

29 Areaya AA, Cerezal L, Canga A, e t al . Neuroimagingdiagnosis of Tolosa-Hunt syndrome: MRI contribution.

Headache 1999;39:3215.30 Kwan ESK, Walpert SM, Hedges TR, et al. Tolosa-Huntrevisited: not necessarily a diagnosis of exclusion. AJNR

Am J Neuroradiol 1987;8:106772.31 Pascual J,Cerezal L,Canga A, et al. Tolosa-Hunt syndrome:

focus on MRI diagnosis. Cephalgia 1999;19(suppl 25):368.

32 Lesser R, Jampol LM. Tolosa-Hunt syndrome and anti-nuclear factor. Am J Ophthalmol1974;77:7324.

33 Hallpike JF. Superior orbital fissure syndrome. J NeurolNeurosurg Psychiatr y 1973;36:48690.

34 Levy IS, Wright JE, Lloyd GAS. Orbital and retro-orbitalpseudo-tumor. Modern Problems of Ophthalmology 1975;14:3647.

35 Kaye AH, Hahn JF, Craciun A, et al. Intracranial extensionof inflammatory pseudotumor of the orbit. J Neurosurg1984;60:6259.

36 Frohman LP, Kupersmith MJ, Lang J, et al. Intracranialextension and bone destruction in orbital pseudotumor.

Arch Ophthalmol 1986;104:3804.

Table 3 Additional causes of painful ophthalmoplegia

Orbital disease:Idiopathic orbital inflammation (pseudotumour)Contiguous sinusitisMucormycosis or other fungal infectionMetastatic tumourLymphoma/leukaemia

Diabetic ophthalmoplegia:MononeuropathyMultiple cranial nerve palsies

Posterior fossa aneurysm:

Posterior communicating arteryBasilar artery

Giant cell arteritis:

Ophthalmoplegic migraine:

The Tolosa-Hunt syndrome 581

www.jnnp.com


7/7

37 Bencheif B, Zovaoui A, Chedid G, et al. Intracranial exten-sion of idiopathic orbital inflammatory pseudotumor.

AJNR Am J Neuroradiol 1993;14:1814.38 Meadows SP. Intracavernous aneurysms of the internal

carotid artery. Arch Ophthalmol 1959;65:56674.39 Trobe JD, Glaser JS, Post MJD. Meningiomas and

aneurysms of the cavernous sinus. Arch Ophthalmol1978;96:45767.

40 Coppedo JR, HoVman H. Tolosa-Hunt syndrome withproptosis mimicked by giant aneurysm of the posterior cer-ebral artery. Arch Neurol 1981;38:545.

41 Post MJD, Mendez DR, Kline LB, et al. Metastatic diseaseto the cavernous sinus.J Comput Assist Tomogr1985;9:11520.

42 Trobe JD, Hood CI, Parsons JT, et al. Intracranial spread of

squamous carcinoma along the trigeminal nerve. ArchOphthalmol 1982;100:60811.

43 Thomas JE,Yoss RE. The parasellar syndrome: problems indetermining etiology. Mayo Clin Proc 1978;45:61723.

44 Larson DL, Auchincloss JH. Multiple symmetric bilateralcranial nerve palsies in patients with unregulated diabetesmellitus: report of three cases. Ann Intern Med 1950;85:26571.

45 Eshbaugh CG, Siatkowski RM, Smith JL, et al. Simultane-ous, multiple cranial neuropathies in diabetes mellitus. J

Neuroophthalmol1995;15:21924.46 Barricks ME, Traviesa DB, Glaser JS, et al. Ophthalmople-

gia in cranial arteritis. Brain 1977;100:20921.47 Friedman AP, Hunter GH, Merritt HH. Ophthalmoplegic

migraine. Arch Neurol 1962;7:3207.48 Mark AS, Blake P, Atlas SW, et al. Gd-DTPA enhancement

of the cisternal portion of the oculomotor nerve on MRimaging. AJNR Am J Neuroradiol 1992;13:146370.

49 Stommel EW, Ward TN, Harris RD. MRI findings in a case

of ophthalmoplegic migraine. Headache 1993;33:2347.50 stergaard JR, Moller HV, Christensen T. Recurrent

ophthalmoplegia in childhood: diagnostic and etiologicconsiderations. Cephalgia 1996;17:703.

w w w . j n n p . c o m

S e e w h i c h a r t i c l e s h a v e j u s t b e e n a c c e p t e d f o r p u b l i c a t i o n a n d

p r e v i e w t h e t a b l e o f c o n t e n t s f o r t h e n e x t i s s u e a m o n t h b e f o r e i t i s p u b l i s h e d

F u t u r e c o n t e n t

W h a t ' s i n t h e n e x t i s s u e

582 Kline,Hoyt

www.jnnp.com

THS Jurnal

Documents

Transcript of THS Jurnal