Painful Ophthalmoplegia (Tolosa-Hunt Variant): Autopsy Findings in a Patient With Necrotizing Intracavernous Carotid Vasculitis and Inflammatory Disease of the Orbit
1987; Elsevier BV; Volume: 62; Issue: 6 Linguagem: Inglês
10.1016/s0025-6196(12)65478-0
ISSN1942-5546
Autores Tópico(s)Ophthalmology and Eye Disorders
ResumoA 79-year-old man with unremitting painful ophthalmoplegia had a necrotizing inflammatory process that involved the intracranial and intracavernous portions of the right internal carotid artery. The condition ultimately resulted in rupture of the carotid vessel and death. At autopsy, thrombophlebitis of the sphenopalatine sinus was also noted. To our knowledge, this is the second reported autopsy study of painful ophthalmoplegia and chronic nongranulomatous inflammation that involved the carotid siphon and the cavernous sinus. A 79-year-old man with unremitting painful ophthalmoplegia had a necrotizing inflammatory process that involved the intracranial and intracavernous portions of the right internal carotid artery. The condition ultimately resulted in rupture of the carotid vessel and death. At autopsy, thrombophlebitis of the sphenopalatine sinus was also noted. To our knowledge, this is the second reported autopsy study of painful ophthalmoplegia and chronic nongranulomatous inflammation that involved the carotid siphon and the cavernous sinus. The symptom complex of painful ophthalmoplegia has varied causes, including neoplasm, inflammation, trauma, aneurysm, and diabetes mellitus.1Kline LB The Tolosa-Hunt syndrome.Surv Ophthalmol. 1982; 27: 79-95Abstract Full Text PDF PubMed Scopus (96) Google Scholar The pathologic process may involve the cavernous sinus or the orbital apex or both. Because of the varied sites of the lesion, several terms have arisen. The eponym Tolosa-Hunt syndrome, coined by Smith and Taxdal,2Smith JL Taxdal DSR Painful ophthalmoplegia: the Tolosa-Hunt syndrome.Am J Ophthalmol. 1966; 61: 1466-1472Abstract Full Text PDF PubMed Scopus (114) Google Scholar has been applied to an inflammatory process of unknown cause that involves the walls of the carotid siphon. This process may be accompanied by arteriographic3Adams AH Warner AM Painful ophthalmoplegia: report of a case with cerebral involvement and psychiatric complications.Bull Los Angeles Neurol Soc. 1975; 40: 49-55PubMed Google Scholar, 4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google Scholar, 5Cohn DF Carasso R Streifler M Painful ophthalmoplegia: the Tolosa-Hunt syndrome.Eur Neurol. 1979; 18: 373-381Crossref PubMed Scopus (21) Google Scholar, 6Dornan TL Espir MLE Gale EAM Tattersall RB Worthington BS Remittent painful ophthalmoplegia: the Tolosa-Hunt syndrome? A report of seven cases and review of the literature.J Neurol Neurosurg Psychiatry. 1979; 42: 270-275Crossref PubMed Scopus (23) Google Scholar, 7Hallpike JF Superior orbital fissure syndrome: some clinical and radiological observations.J Neurol Neurosurg Psychiatry. 1973; 36: 486-490Crossref PubMed Scopus (32) Google Scholar, 8Mathew NT Chandy J Painful ophthalmoplegia.J Neurol Sci. 1970; 11: 243-256Abstract Full Text PDF PubMed Scopus (49) Google Scholar, 9Schatz NJ Farmer P Tolosa-Hunt syndrome: the pathology of painful ophthalmoplegia.in: Smith JL Neuro-ophthalmology: Symposium of the University of Miami and the Bascom Palmer Eye Institute. Vol 6. CV Mosby Company, St. Louis1972: 102-112Google Scholar, 10Sondheimer FK Knapp J Angiographic findings in the Tolosa-Hunt syndrome: painful ophthalmoplegia.Radiology. 1973; 106: 105-112PubMed Google Scholar, 11Spinnler H Painful ophthalmoplegia: the Tolosa-Hunt syndrome.Med J Aust. 1973; 2: 645-646PubMed Google Scholar, 12Takeoka T Gotoh F Fukuuchi Y Inagaki Y Tolosa-Hunt syndrome: arteriographic evidence of improvement in carotid narrowing.Arch Neurol. 1978; 35: 219-223Crossref PubMed Scopus (19) Google Scholar, 13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar and venographic abnormalities.4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google Scholar, 5Cohn DF Carasso R Streifler M Painful ophthalmoplegia: the Tolosa-Hunt syndrome.Eur Neurol. 1979; 18: 373-381Crossref PubMed Scopus (21) Google Scholar, 6Dornan TL Espir MLE Gale EAM Tattersall RB Worthington BS Remittent painful ophthalmoplegia: the Tolosa-Hunt syndrome? A report of seven cases and review of the literature.J Neurol Neurosurg Psychiatry. 1979; 42: 270-275Crossref PubMed Scopus (23) Google Scholar, 7Hallpike JF Superior orbital fissure syndrome: some clinical and radiological observations.J Neurol Neurosurg Psychiatry. 1973; 36: 486-490Crossref PubMed Scopus (32) Google Scholar, 10Sondheimer FK Knapp J Angiographic findings in the Tolosa-Hunt syndrome: painful ophthalmoplegia.Radiology. 1973; 106: 105-112PubMed Google Scholar, 12Takeoka T Gotoh F Fukuuchi Y Inagaki Y Tolosa-Hunt syndrome: arteriographic evidence of improvement in carotid narrowing.Arch Neurol. 1978; 35: 219-223Crossref PubMed Scopus (19) Google Scholar, 14Milstein BA Morretin LB Report of a case of sphenoid fissure syndrome studied by orbital venography.Am J Ophthalmol. 1971; 72: 600-603PubMed Scopus (21) Google Scholar, 15Muhletaler CA Gerlock Jr, AJ Orbital venography in painful ophthalmoplegia (Tolosa-Hunt syndrome).AJR. 1979; 133: 31-34Crossref PubMed Scopus (31) Google Scholar Several authors4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google Scholar, 10Sondheimer FK Knapp J Angiographic findings in the Tolosa-Hunt syndrome: painful ophthalmoplegia.Radiology. 1973; 106: 105-112PubMed Google Scholar, 15Muhletaler CA Gerlock Jr, AJ Orbital venography in painful ophthalmoplegia (Tolosa-Hunt syndrome).AJR. 1979; 133: 31-34Crossref PubMed Scopus (31) Google Scholar, 16Donin JF Borit A Orbital myositis: its relationship to the Tolosa-Hunt syndrome.in: Smith JL Neuro-ophthalmology Update. Masson Publishing, New York1977: 99-103Google Scholar, 17Levy IS Wright JE Lloyd GAS Orbital and retro-orbital pseudo-tumours.Mod Probl Ophthalmol. 1975; 14: 364-367PubMed Google Scholar have suggested a kinship of this entity with the so-called orbital inflammatory pseudotumor, an enigma in itself, and at least one patient with a combined inflammatory process of the cavernous sinus and orbital tissue has been described.4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google Scholar, 16Donin JF Borit A Orbital myositis: its relationship to the Tolosa-Hunt syndrome.in: Smith JL Neuro-ophthalmology Update. Masson Publishing, New York1977: 99-103Google Scholar, 17Levy IS Wright JE Lloyd GAS Orbital and retro-orbital pseudo-tumours.Mod Probl Ophthalmol. 1975; 14: 364-367PubMed Google Scholar, 18Rosenbaum DH Davis MJ Song IS The syndrome of painful ophthalmoplegia: a case with intraorbital mass and hypervascularity.Arch Neurol. 1979; 36: 41-43Crossref PubMed Scopus (14) Google Scholar In some instances, the cause of the inflammation has been determined;19Finlay CE Two cases of syphilitic lesions situated at the sphenoidal fissure: sphenoidal syndrome.South Med J. 1930; 23: 51-54Crossref Scopus (3) Google Scholar, 20Rochon-Duvigneaud Quelques cas de paralysie de tous les nerfs orbitaires (ophtalmoplégie totale avec amaurose et anesthésie dans le domaine de l'ophtalmique), d'origine syphilitique.Arch Ophtalmol (Paris). 1896; 16: 746-760Google Scholar in others, the nature of the presumed inflammation and its source remain in doubt. A few patients have had an accompanying sinusitis.6Dornan TL Espir MLE Gale EAM Tattersall RB Worthington BS Remittent painful ophthalmoplegia: the Tolosa-Hunt syndrome? A report of seven cases and review of the literature.J Neurol Neurosurg Psychiatry. 1979; 42: 270-275Crossref PubMed Scopus (23) Google Scholar, 21Trantas A Ophtalmoplégie totale et autres complications oculaires dans les polysinusites.Arch Ophtalmol (Paris). 1893; 13: 358-362Google Scholar, 22Kjoer I A case of orbital apex syndrome in collateral pansinusitis.Acta Ophthalmol (Copenh). 1945; 23: 357-366Crossref Scopus (23) Google Scholar, 23Kretzschmar S Jacot P Des symptōmes précoces et d'une étiologie souvent méconnue du syndrome de la fente sphénoidale (à propos de quatre cas personnels).Schweiz Med Wochenschr. 1939; 69: 1103-1107Google Scholar Reports of pathologic examination of tissue from patients with a suspected inflammatory process of the cavernous sinus have been limited. Two previous autopsy studies are recorded,13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar, 16Donin JF Borit A Orbital myositis: its relationship to the Tolosa-Hunt syndrome.in: Smith JL Neuro-ophthalmology Update. Masson Publishing, New York1977: 99-103Google Scholar but only that by Tolosa13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar demonstrated an abnormality at the site of the cavernous sinus. Histologic examination of biopsy material from the cavernous sinus or orbit is reported to have shown “granulomatous” inflammation in some cases4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google Scholar, 9Schatz NJ Farmer P Tolosa-Hunt syndrome: the pathology of painful ophthalmoplegia.in: Smith JL Neuro-ophthalmology: Symposium of the University of Miami and the Bascom Palmer Eye Institute. Vol 6. CV Mosby Company, St. Louis1972: 102-112Google Scholar, 24Berg E Gay AJ Tolosa-Hunt syndrome.Eye Ear Nose Throat Digest. 1967; 29: 51-52Google Scholar and nonspecific chronic inflammation in others.13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar, 17Levy IS Wright JE Lloyd GAS Orbital and retro-orbital pseudo-tumours.Mod Probl Ophthalmol. 1975; 14: 364-367PubMed Google Scholar, 25Lakke JPWF Superior orbital fissure syndrome: report of a case caused by local pachymeningitis.Arch Neurol. 1962; 7: 289-300Crossref PubMed Scopus (79) Google Scholar, 26Spector RH Fiandaca MS The “sinister” Tolosa-Hunt syndrome.Neurology. 1986; 36: 198-203Crossref PubMed Google Scholar The site and nature of the histopathologic findings in this case report closely resemble those described by Tolosa.13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar A 79-year-old man who had had a subdural hematoma in 1976 underwent a total hip arthroplasty on Nov. 14, 1983. The immediate postoperative period was uneventful. Three days postoperatively, persistent frontal headache, right maxillofacial pain, nasal tenderness, and a white, blood-flecked nasal discharge developed. These symptoms were accompanied by discomfort of the gum on the upper right side but no fever. Roentgenograms of the sinuses showed thickened membranes of both maxillary antra and the ethmoid sinuses. The erythrocyte sedimentation rate was 40 mm in 1 hour. Infected, carious teeth were removed, and the patient received ampicillin (250 mg four times a day). The nasal tenderness and discharge subsided, but the pain, which had been constant, moved to the right retrobulbar region. The patient had no ocular pain or diplopia. Results of an otorhinolaryngologic examination were normal. Because inflammatory pseudotumor was considered a likely cause of the pain, a 7-day course of prednisone was given, but the pain persisted. A trial of indomethacin was also ineffective. The retrobulbar pain continued, and extension of the pain to the jaw and diplopia developed on May 12, 1984. Six days later, when the patient returned for reexamination, the right eye was displaced inferiorly and the right upper lid was swollen. The pupils were equal and reacting; an incomplete right external ophthalmoplegia was noted, and only slight downward and lateral movement remained. Vision was 20/50, 14/89 in the right eye and 20/30, 14/21 in the left eye. Tension was 16 mm Hg in each eye. The fundi were normal. A computed tomographic scan disclosed a dense, soft-tissue mass at the right orbital apex that extended into the superior and inferior orbital fissures. Elevation of the inferior rectus muscle suggested that the bulk of the mass was extraconal. No bone destruction was found. The adjacent ethmoid sinuses showed some membrane thickening. On May 30, the left eye of the patient was normal, whereas he perceived only hand movements with the right eye. The right pupil was larger than the left and had an afferent defect. External ophthalmoplegia was virtually complete, and only minimal voluntary medial deviation of the eye remained. On June 4, the patient could not perceive light with the right eye. In the left eye, visual acuity was 20/30, 14/21 and the visual field was normal. Ptosis (2.5 mm) and complete ophthalmoplegia were noted on the right. The fundi were normal. At right medial orbitotomy on June 18, dissection along the lamina papyracea showed diffuse thickening of the periosteum but no definite mass. Biopsy material showed a necrotizing vasculitis consistent with pseudotumor. Therapy with cyclophosphamide (100 mg/day) was instituted, but the severe retrobulbar and facial pain continued. Two days after orbitotomy, light perception returned to the right eye, but the complete ophthalmoplegia persisted. By July 6, the patient could appreciate hand movements and complained of diplopia in addition to the severe retrobulbar pain. The ptosis was complete. The right pupil had an afferent defect, and the external ophthalmoplegia remained. The patient became confused and had hallucinations. A second computed tomographic scan with axial and coronal views of the orbit revealed no abnormality. Tomograms of the ethmoid sinuses showed persistent, slightly thickened membranes. A lumbar puncture was performed; the cerebrospinal fluid values were as follows: leukocytes, 220/mm3Adams AH Warner AM Painful ophthalmoplegia: report of a case with cerebral involvement and psychiatric complications.Bull Los Angeles Neurol Soc. 1975; 40: 49-55PubMed Google Scholar (180 polymorphonuclear leukocytes, 31 monocytes, and 9 lymphocytes); erythrocytes, 8/mm3Adams AH Warner AM Painful ophthalmoplegia: report of a case with cerebral involvement and psychiatric complications.Bull Los Angeles Neurol Soc. 1975; 40: 49-55PubMed Google Scholar; glucose, 64 mg/dl; protein, 54 mg/dl; and culture, negative. A blood culture was also negative. Generalized seizures occurred, and the patient become comatose. A third computed tomographic scan showed a large frontal subarachnoid hemorrhage. Death occurred 4 days after the loss of consciousness. The brain weighed 1,515 g. Extensive subarachnoid hemorrhage involved the right frontal and basal areas. The dura at the base of the cavernous sinus and the roof of the right orbit showed a hemorrhagic discoloration (Fig. 1). The eyes, optic nerve, and orbital contents were removed by a retro-orbital approach. The fatty tissue of the right orbit was discolored and hemorrhagic. The dorsum sellae, together with the pituitary and cavernous sinus, was removed en bloc (Fig. 2).Fig. 2Specimen obtained from en bloc dissection of dorsum sellae together with pituitary and cavernous sinus. Note distended cavernous sinus on right and intramural thrombus within carotid artery.View Large Image Figure ViewerDownload (PPT) The wall of the intracranial portion of the carotid artery was thickened by an infiltration of polymorphonuclear leukocytes, lymphocytes, plasma cells, and fibrin. No epithelioid cells or giant cells were noted. At the site of rupture, necrosis of the arterial wall was pronounced. Special stains for bacteria and fungi were negative. The intracavernous portion of the right carotid artery showed a similar necrotizing inflammation of the walls; a mural thrombus was also present (Fig. 3). In the right cavernous sinus, partial obstruction, inflammation of the wall, and mural thrombus were evident. The right sphenopalatine sinus also showed thrombophlebitis. A postmortem angiogram disclosed rupture of the intracranial portion of the right internal carotid artery (Fig. 4). In the brain, acute infarction was present in the distribution of the right middle cerebral artery.Fig. 4Postmortem angiogram, showing rupture of intracranial portion of right internal carotid artery.View Large Image Figure ViewerDownload (PPT) There was no evidence of vasculitis elsewhere in the body and no evidence of infection apart from a terminal bronchopneumonia. Examination of the paranasal sinuses revealed that they were free of pus. The complex of symptoms and signs consisting of cranial or orbital pain or both, with or without facial pain, accompanied by complete or incomplete ophthalmoplegia and impairment of sensation in the upper division (or divisions) of the trigeminal nerve is known clinically by several terms, including the superior orbital fissure syndrome,7Hallpike JF Superior orbital fissure syndrome: some clinical and radiological observations.J Neurol Neurosurg Psychiatry. 1973; 36: 486-490Crossref PubMed Scopus (32) Google Scholar, 20Rochon-Duvigneaud Quelques cas de paralysie de tous les nerfs orbitaires (ophtalmoplégie totale avec amaurose et anesthésie dans le domaine de l'ophtalmique), d'origine syphilitique.Arch Ophtalmol (Paris). 1896; 16: 746-760Google Scholar the orbital apex syndrome,27Holt H de Rötth A Orbital apex and sphenoid fissure syndrome.Arch Ophthalmol. 1940; 24: 731-741Crossref Google Scholar the cavernous sinus syndrome,28Foix C Syndrome de la paroi externe du sinus caverneux. (Ophtalmoplégie unilatérale à marche rapidement progressive. Algie du territoire de l'ophtalmique.) Amélioration considérable par le traitement radiothérapique.Rev Neurol (Paris). 1922; 38: 827-832Google Scholar the parasellar syndrome,29Thomas JE Yoss RE The parasellar syndrome: problems in determining etiology.Mayo Clin Proc. 1970; 45: 617-623PubMed Google ScholarCollier's syndrome,30Collier J Discussion on ocular palsies.Proc R Soc Med. 1921; 14: 10-11Google Scholar, 31Cheah JS Ransome GA Collier's syndrome (orbital periostitis).Med J Aust. 1970; 1: 277-278PubMed Google Scholar and Tolosa-Hunt syndrome.2Smith JL Taxdal DSR Painful ophthalmoplegia: the Tolosa-Hunt syndrome.Am J Ophthalmol. 1966; 61: 1466-1472Abstract Full Text PDF PubMed Scopus (114) Google Scholar, 13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar, 32Hunt WE Meagher JN LeFever HE Zeman W Painful ophthalmoplegia: its relation to indolent inflammation of the cavernous sinus.Neurology. 1961; 11: 56-62Crossref PubMed Google Scholar, 33Hunt WE Tolosa-Hunt syndrome: one cause of painful ophthalmoplegia.J Neurosurg. 1976; 44: 544-549Crossref PubMed Scopus (89) Google Scholar In the literature, confusion has arisen because similar signs and symptoms may result from lesions that involve the cavernous sinus or the superior orbital apex because the same cranial nerves pass through each of these sites. The superior orbital fissure is merely 2 cm distal to the cavernous sinus; thus, the same pathologic process may involve both sites, as demonstrated in our case and by other investigators.1Kline LB The Tolosa-Hunt syndrome.Surv Ophthalmol. 1982; 27: 79-95Abstract Full Text PDF PubMed Scopus (96) Google Scholar, 4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google Scholar, 7Hallpike JF Superior orbital fissure syndrome: some clinical and radiological observations.J Neurol Neurosurg Psychiatry. 1973; 36: 486-490Crossref PubMed Scopus (32) Google Scholar, 16Donin JF Borit A Orbital myositis: its relationship to the Tolosa-Hunt syndrome.in: Smith JL Neuro-ophthalmology Update. Masson Publishing, New York1977: 99-103Google Scholar, 17Levy IS Wright JE Lloyd GAS Orbital and retro-orbital pseudo-tumours.Mod Probl Ophthalmol. 1975; 14: 364-367PubMed Google Scholar, 18Rosenbaum DH Davis MJ Song IS The syndrome of painful ophthalmoplegia: a case with intraorbital mass and hypervascularity.Arch Neurol. 1979; 36: 41-43Crossref PubMed Scopus (14) Google Scholar Orbital pseudotumor and inflammation around the cavernous sinus seem to be part of a similar disease process. The character of the lesion responsible for the symptoms also varies; these lesions include primary and metastatic tumors,26Spector RH Fiandaca MS The “sinister” Tolosa-Hunt syndrome.Neurology. 1986; 36: 198-203Crossref PubMed Google Scholar, 29Thomas JE Yoss RE The parasellar syndrome: problems in determining etiology.Mayo Clin Proc. 1970; 45: 617-623PubMed Google Scholar, 34Fowler TJ Earl CJ McAllister VL McDonald WI Tolosa-Hunt syndrome: the dangers of an eponym.Br J Ophthalmol. 1975; 59: 149-154Crossref PubMed Scopus (31) Google Scholar, 35Godtfredsen E Lederman M Diagnostic and prognostic roles of ophthalmoneurologic signs and symptoms in malignant nasopharyngeal tumors.Am J Ophthalmol. 1965; 59: 1063-1069PubMed Scopus (30) Google Scholar, 36Messmer EP Font RL McCrary III, JA Murphy D Epithelioid angiosarcoma of the orbit presenting as Tolosa-Hunt syndrome: a clinicopathologic case report with review of the literature.Ophthalmology. 1983; 90: 1414-1421Abstract PubMed Scopus (25) Google Scholar, 37Unsöld R Safran AB Safran E Hoyt WF Metastatic infiltration of nerves in the cavernous sinus.Arch Neurol. 1980; 37: 59-61Crossref PubMed Scopus (31) Google Scholar trauma,38Hirschfeld L Epanchement de sang dans le sinus caverneux du cōté gauche diagnostiqué pendant la vie.C R Soc Biol (Paris). 1858; 10: 138-140Google Scholar diabetes mellitus,1Kline LB The Tolosa-Hunt syndrome.Surv Ophthalmol. 1982; 27: 79-95Abstract Full Text PDF PubMed Scopus (96) Google Scholar aneurysm,34Fowler TJ Earl CJ McAllister VL McDonald WI Tolosa-Hunt syndrome: the dangers of an eponym.Br J Ophthalmol. 1975; 59: 149-154Crossref PubMed Scopus (31) Google Scholar, 39Meadows SP Intracavernous aneurysms of the internal carotid artery: their clinical features and natural history.Arch Ophthalmol. 1959; 62: 566-574Crossref Scopus (49) Google Scholar, 40Trobe JD Glaser JS Post JD Meningiomas and aneurysms of the cavernous sinus: neuro-ophthalmologic features.Arch Ophthalmol. 1978; 96: 457-467Crossref PubMed Scopus (80) Google Scholar and infective19Finlay CE Two cases of syphilitic lesions situated at the sphenoidal fissure: sphenoidal syndrome.South Med J. 1930; 23: 51-54Crossref Scopus (3) Google Scholar, 20Rochon-Duvigneaud Quelques cas de paralysie de tous les nerfs orbitaires (ophtalmoplégie totale avec amaurose et anesthésie dans le domaine de l'ophtalmique), d'origine syphilitique.Arch Ophtalmol (Paris). 1896; 16: 746-760Google Scholar, 21Trantas A Ophtalmoplégie totale et autres complications oculaires dans les polysinusites.Arch Ophtalmol (Paris). 1893; 13: 358-362Google Scholar, 22Kjoer I A case of orbital apex syndrome in collateral pansinusitis.Acta Ophthalmol (Copenh). 1945; 23: 357-366Crossref Scopus (23) Google Scholar, 23Kretzschmar S Jacot P Des symptōmes précoces et d'une étiologie souvent méconnue du syndrome de la fente sphénoidale (à propos de quatre cas personnels).Schweiz Med Wochenschr. 1939; 69: 1103-1107Google Scholar and noninfective inflammation.13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar, 17Levy IS Wright JE Lloyd GAS Orbital and retro-orbital pseudo-tumours.Mod Probl Ophthalmol. 1975; 14: 364-367PubMed Google Scholar, 25Lakke JPWF Superior orbital fissure syndrome: report of a case caused by local pachymeningitis.Arch Neurol. 1962; 7: 289-300Crossref PubMed Scopus (79) Google Scholar In only a few instances (Table 1) has material from the cavernous sinus been obtained by biopsy or autopsy to show the character of the inflammatory infiltrate.4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google Scholar, 9Schatz NJ Farmer P Tolosa-Hunt syndrome: the pathology of painful ophthalmoplegia.in: Smith JL Neuro-ophthalmology: Symposium of the University of Miami and the Bascom Palmer Eye Institute. Vol 6. CV Mosby Company, St. Louis1972: 102-112Google Scholar, 13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar, 17Levy IS Wright JE Lloyd GAS Orbital and retro-orbital pseudo-tumours.Mod Probl Ophthalmol. 1975; 14: 364-367PubMed Google Scholar, 24Berg E Gay AJ Tolosa-Hunt syndrome.Eye Ear Nose Throat Digest. 1967; 29: 51-52Google Scholar, 25Lakke JPWF Superior orbital fissure syndrome: report of a case caused by local pachymeningitis.Arch Neurol. 1962; 7: 289-300Crossref PubMed Scopus (79) Google Scholar, 26Spector RH Fiandaca MS The “sinister” Tolosa-Hunt syndrome.Neurology. 1986; 36: 198-203Crossref PubMed Google ScholarTable 1Reported Cases With Histologic Confirmation of Inflammatory Tissue From the Cavernous SinusYearAuthorAge (yr) and sex of patientPathologic finding1954Tolosa13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar47 MIntracavernous periarteritic granulation tissue*Autopsy examination.1962Lakke25Lakke JPWF Superior orbital fissure syndrome: report of a case caused by local pachymeningitis.Arch Neurol. 1962; 7: 289-300Crossref PubMed Scopus (79) Google Scholar47 MAcute inflammatory granulation tissue lining cavernous sinus and superior orbital fissure1967Berg and Gay24Berg E Gay AJ Tolosa-Hunt syndrome.Eye Ear Nose Throat Digest. 1967; 29: 51-52Google Scholar? ?Noncaseating granulomas in left sphenoid bone and sinus1972Schatz and Farmer9Schatz NJ Farmer P Tolosa-Hunt syndrome: the pathology of painful ophthalmoplegia.in: Smith JL Neuro-ophthalmology: Symposium of the University of Miami and the Bascom Palmer Eye Institute. Vol 6. CV Mosby Company, St. Louis1972: 102-112Google Scholar38 FGranulomatous tissue in lateral wall of cavernous sinus61 F“Granulomatous tissue” extending from cavernous sinus to gasserian ganglion1975Levy et al17Levy IS Wright JE Lloyd GAS Orbital and retro-orbital pseudo-tumours.Mod Probl Ophthalmol. 1975; 14: 364-367PubMed Google Scholar40 FChronic inflammatory tissue covering cavernous sinus26 F“Chronic inflammatory granuloma of the dura” (biopsy). Fibrotic chronic inflammatory tissue occluding cavernous sinus bilaterally*Autopsy examination.1978Aron-Rosa et al4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google ScholarTwo patients†Age and sex not specified.“Granulomatous inflammation” of dura on border of orbital fissure1986Spector and Fiandaca26Spector RH Fiandaca MS The “sinister” Tolosa-Hunt syndrome.Neurology. 1986; 36: 198-203Crossref PubMed Google Scholar37 MChronic inflammatory granulation tissue and “erythema of the third nerve just before it entered the cavernous sinus”* Autopsy examination.† Age and sex not specified. Open table in a new tab By current definition, the term “granulomatous inflammation” is reserved for the microscopic description of an inflammatory infiltrate that contains epithelioid cells. The epithelioid cells are modified macrophages, and giant cells result from their fusion. Granulomatous infiltration is seen in such entities as sarcoidosis, tuberculosis, and Wegener's granulomatosis. In contrast, granulation tissue consists of proliferating vessels and fibroblasts with acute and chronic inflammatory cells admixed. In 1954, Tolosa13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar described a patient with the aforementioned symptoms who had a periarteritic inflammatory lesion of the carotid siphon at autopsy. Tolosa used the term “granulomatous” to describe the gross appearance of the abnormality. It is apparent from Tolosa's microscopic description and the subsequent substantiation and illustration of the case by Hunt and associates32Hunt WE Meagher JN LeFever HE Zeman W Painful ophthalmoplegia: its relation to indolent inflammation of the cavernous sinus.Neurology. 1961; 11: 56-62Crossref PubMed Google Scholar that the histologic appearance was that of granulation tissue. Epithelioid cells were not mentioned, nor were they evident in the illustration. Many authors have subsequently misquoted Tolosa and have stated that he described a granulomatous inflammation. Others claim to have seen a granulomatous inflammation, but the verification is incomplete or lacking.4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google Scholar, 9Schatz NJ Farmer P Tolosa-Hunt syndrome: the pathology of painful ophthalmoplegia.in: Smith JL Neuro-ophthalmology: Symposium of the University of Miami and the Bascom Palmer Eye Institute. Vol 6. CV Mosby Company, St. Louis1972: 102-112Google Scholar Eight years after Tolosa's report was published, Lakke25Lakke JPWF Superior orbital fissure syndrome: report of a case caused by local pachymeningitis.Arch Neurol. 1962; 7: 289-300Crossref PubMed Scopus (79) Google Scholar described a patient who was the same age and sex as Tolosa's patient and had identical signs. In this patient, craniotomy showed that the lateral wall of the cavernous sinus was covered by a thin layer of grayish red tissue; the histologic finding was inflammatory tissue that contained polynuclear cells. Dura from the region of the superior orbital fissure was partly necrotic and lined by granulation tissue. Schatz and Farmer,9Schatz NJ Farmer P Tolosa-Hunt syndrome: the pathology of painful ophthalmoplegia.in: Smith JL Neuro-ophthalmology: Symposium of the University of Miami and the Bascom Palmer Eye Institute. Vol 6. CV Mosby Company, St. Louis1972: 102-112Google Scholar in a report of four patients with the Tolosa-Hunt syndrome, claimed to confirm the pathologic findings of Tolosa in three of the patients. Two patients underwent craniotomy. In the first of these patients, the true granulomatous nature of the infiltrate involving the lateral wall of the cavernous sinus was illustrated. Epithelioid cells, the sine qua non of a granulomatous inflammation, were neither described nor illustrated in the second patient. The third patient had a histologically confirmed granuloma of the parotid gland, but no cranial biopsy material was obtained. In a study of seven patients, Levy and associates17Levy IS Wright JE Lloyd GAS Orbital and retro-orbital pseudo-tumours.Mod Probl Ophthalmol. 1975; 14: 364-367PubMed Google Scholar concluded that a chronic inflammatory condition of unknown cause could affect the superior orbital fissure and the cavernous sinus. In their series of patients, two underwent craniotomy and had histologic evidence of chronic inflammation (five had orbital biopsy specimens with evidence of pseudotumor). Aron-Rosa and associates4Aron-Rosa D Doyon D Salamon G Michotey P Tolosa-Hunt syndrome.Ann Ophthalmol. 1978; 10: 1161-1168PubMed Google Scholar obtained biopsy material at the time of surgical exploration in 2 of 10 patients with Tolosa-Hunt syndrome. In both patients, the dura of the lateral aspect of the cavernous sinus was thickened by “granulomatous inflammation.” The authors did not specify whether this was a gross or a microscopic description. In a recent report by Spector and Fiandaca,26Spector RH Fiandaca MS The “sinister” Tolosa-Hunt syndrome.Neurology. 1986; 36: 198-203Crossref PubMed Google Scholar a patient with Tolosa-Hunt syndrome underwent exploratory craniotomy. They described the biopsy material from the region of the cavernous sinus as granulation tissue. Exploratory craniotomy revealed normal findings in 1 of 22 patients described by Mathew and Chandy,8Mathew NT Chandy J Painful ophthalmoplegia.J Neurol Sci. 1970; 11: 243-256Abstract Full Text PDF PubMed Scopus (49) Google Scholar 1 patient described by Hallpike,7Hallpike JF Superior orbital fissure syndrome: some clinical and radiological observations.J Neurol Neurosurg Psychiatry. 1973; 36: 486-490Crossref PubMed Scopus (32) Google Scholar and 1 patient (case 2) described by Hunt and associates.32Hunt WE Meagher JN LeFever HE Zeman W Painful ophthalmoplegia: its relation to indolent inflammation of the cavernous sinus.Neurology. 1961; 11: 56-62Crossref PubMed Google Scholar Interestingly, in the patient with painful bilateral ophthalmoplegia described by Donin and Borit,16Donin JF Borit A Orbital myositis: its relationship to the Tolosa-Hunt syndrome.in: Smith JL Neuro-ophthalmology Update. Masson Publishing, New York1977: 99-103Google Scholar autopsy showed that the cavernous sinus was normal but that the orbital muscle had subacute inflammation with a few multinucleated giant cells. To our knowledge, our case report is the second autopsy description of painful ophthalmoplegia and a chronic nongranulomatous inflammation that involved the carotid siphon and the cavernous sinus. The character of the inflammatory reaction was similar to that in the first report by Tolosa13Tolosa E Periarteritic lesions of the carotid siphon with the clinical features of a carotid infraclinoidal aneurysm.J Neurol Neurosurg Psychiatry. 1954; 17: 300-302Crossref PubMed Scopus (246) Google Scholar and in three biopsy reports by other investigators.17Levy IS Wright JE Lloyd GAS Orbital and retro-orbital pseudo-tumours.Mod Probl Ophthalmol. 1975; 14: 364-367PubMed Google Scholar, 25Lakke JPWF Superior orbital fissure syndrome: report of a case caused by local pachymeningitis.Arch Neurol. 1962; 7: 289-300Crossref PubMed Scopus (79) Google Scholar, 26Spector RH Fiandaca MS The “sinister” Tolosa-Hunt syndrome.Neurology. 1986; 36: 198-203Crossref PubMed Google Scholar In addition, the orbital involvement in our patient confirmed the overlap that exists between the Tolosa-Hunt syndrome and orbital pseudotumor. Our case was also unique in that the noninfectious necrotic arteritis involved the intracranial portion of the internal carotid artery and subsequently led to rupture of the vessel and a fatal outcome. It is of note that, despite the inflammatory nature of the pathologic findings, the patient had no response to corticosteroid therapy. One might even speculate that corticosteroid therapy promoted rupture of the carotid vessel. In contrast, painful ophthalmoplegia caused by tumor or aneurysm may respond to corticosteroid therapy.29Thomas JE Yoss RE The parasellar syndrome: problems in determining etiology.Mayo Clin Proc. 1970; 45: 617-623PubMed Google Scholar, 32Hunt WE Meagher JN LeFever HE Zeman W Painful ophthalmoplegia: its relation to indolent inflammation of the cavernous sinus.Neurology. 1961; 11: 56-62Crossref PubMed Google Scholar, 33Hunt WE Tolosa-Hunt syndrome: one cause of painful ophthalmoplegia.J Neurosurg. 1976; 44: 544-549Crossref PubMed Scopus (89) Google Scholar, 34Fowler TJ Earl CJ McAllister VL McDonald WI Tolosa-Hunt syndrome: the dangers of an eponym.Br J Ophthalmol. 1975; 59: 149-154Crossref PubMed Scopus (31) Google Scholar For those in favor of eponyms, we suggest that Tolosa-Hunt syndrome be reserved for patients with painful ophthalmoplegia proved by histologic diagnosis to be inflammatory. We concur with others that the use of various terms, including that of Tolosa-Hunt syndrome, to describe the clinical entity of painful ophthalmoplegia has little merit, in light of the varied underlying pathologic lesion involved. Regardless of the underlying cause, the essential signs and symptoms can be aptly described by the term “painful ophthalmoplegia.” The cause of the painful ophthalmoplegia in our patient remains an enigma. The swollen lid suggested that the ophthalmoplegia was due to orbital disease. Sparing of the pupil in the presence of an increasing ophthalmoplegia also suggested an orbital pathologic condition. Possibly, the initial inflammation of the ethmoid and sphenoid sinuses subsequently involved the orbit and internal carotid artery by direct extension through the thin wall of the adjacent bony sinus or through the venous system. Associated infection of the paranasal sinuses and ears has been noted and emphasized by other authors.9Schatz NJ Farmer P Tolosa-Hunt syndrome: the pathology of painful ophthalmoplegia.in: Smith JL Neuro-ophthalmology: Symposium of the University of Miami and the Bascom Palmer Eye Institute. Vol 6. CV Mosby Company, St. Louis1972: 102-112Google Scholar, 21Trantas A Ophtalmoplégie totale et autres complications oculaires dans les polysinusites.Arch Ophtalmol (Paris). 1893; 13: 358-362Google Scholar, 22Kjoer I A case of orbital apex syndrome in collateral pansinusitis.Acta Ophthalmol (Copenh). 1945; 23: 357-366Crossref Scopus (23) Google Scholar, 23Kretzschmar S Jacot P Des symptōmes précoces et d'une étiologie souvent méconnue du syndrome de la fente sphénoidale (à propos de quatre cas personnels).Schweiz Med Wochenschr. 1939; 69: 1103-1107Google Scholar, 28Foix C Syndrome de la paroi externe du sinus caverneux. (Ophtalmoplégie unilatérale à marche rapidement progressive. Algie du territoire de l'ophtalmique.) Amélioration considérable par le traitement radiothérapique.Rev Neurol (Paris). 1922; 38: 827-832Google Scholar
Referência(s)