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Show J. Clin. Neuro-ophthalmol. 3: 263-266, 1983. Recurrent Multiple Cranial Nerve Palsies (Tolosa-Hunt Plus ?) TUlAY KANSU, M.D. ONDER US, M.D. CUNSELI: SARPEl, M.D. NIlCUN ARA<,::, M.D. Abstract Twelve patients with recurrent cranial nerve palsies in whom no focal cause was found were seen during a period of 4 years. The literature is reviewed, and the relationship and similarity to Tolosa-Hunt syndrome is discussed. Early recognition is important since the response to steroids, although not specific, is rapid in most patients, and the prognosis for complete recovery is relatively good. Introduction When a cranial nerve is out of action, the physician may be driven into an extensive search for a diagnosis in the"endless caves and tunnels of the skull and pathways of the brain stem." A benign form of multiple cranial nerve involvement is a distinctive syndrome without any underlying disease. In the literature, little attention has been paid to those patients with multiple cranial neuropathies with a recurrent course. I - 5 This present series comprises 12 patients with the syndrome seen in the Hacettepe University Hospitals during a period of 4 years. Patients and Methods From 1979 to 1982, 12 patients with this syndrome were examined. In spite of our investigations, no clear etiology could be found. All patients received general medical, neurological and nasopharyngeal examinations. The following three cases histories illustrate the features of this syndrome Details of the other nine patients with recurrent cranial neurohies which relapsed and remitted are shown in Table 1. Blood cell count, serum glucose or glucose tolerance test, erythrocyte sedimentation rate (ESR), lumbar puncture (LP), skull x-rays, arteriography, and From the Department of Neurology. Hacettepe University Hospitals (TK,OU,NA), Ankara; and C;:ukurova Medical School (GS), Adana, Turkey. December 1983 computerized tomography (CT) were performed according to the clinical picture, as indicated on the table. Case Reports Case 1 A 59-year-old man developed headache, diplopia, and mild ptosis of the left eyelid in 1954. He recovered completely in 2 months and was symptom- free for 13 years, when he developed a left peripheral-type facial weakness. Two months later, he had a left-sided ptosis which led to carotid angiography, which was found to be normal. Recovery was complete in 3 months. In 1978, he had diplopia and ptosis of the right eye which recovered in 15 days. The diplopia and ptosis recurred in 1981, affecting the right eye, and examination revealed mild ptosis and limitation of movement of the right eye on aduction, elevation, and depression. The pupils were equal. The remainder of the neurological examination was normal. Investigation, including erythrocyte sedimentation rate, lumbar puncture, Tensilon test, and repeat carotid angiography, was within normal limits. A follow-up examination 6 months later revealed normal findings. Case 3 A 59-year-old woman developed a right seventh nerve palsy in 1975, for which she underwent facial nerve decompression. She remained well thereafter until December 1980, when she was admitted to the hospital with a 2-day history of left-sided headache, diplopia, and ptosis. Examination revealed a left third nerve palsy and a residual rightsided facial weakness. The pupils were equal. A diagnosis of an ischemic cranial neuropathy was considered. A glucose tolerance test was normal. She recovered completely in 2 months. In June 1981, she was seen again with the complaint of diplopia, and she was found to have a left sixth nerve palsy on examination. Erythrocyte sedimentation rate was 50 mm/hour (Westergren). She 263 Recurrent Cranial Nerve Palsies TABLE l. Clinical and Laboratory Findings Cases laboratory Crania! Nerves Therapy Follow-Up ESR No. Sex Affected mm/ CSF Arteriography CAT Age hour 59M L 3 (1954). L 7 (1967). L 3 Complete recovery 3 Prot. - 44 CAG-Normal (1967), R 3 (1978), R 3 Cell = 0 (1981) VAG-Normal Normal 2 46M R 6, 7, 9, 10, 11, 12, L 5, 6, Prednisolone Mild (R) 7, 11 and 50 Prot. = 34 (1981) 12 palsy Cell = 0 3 59 F R 7 (1979). L 7 (1981, Feb.) Prednisolone Mild (R) 7 palsy 50 Prot. = 17 L 6 (1981, june), R 9, 10 Cell = 0 (1981, Sept.) 4 48 F R 3 (1981, June). L 6 (1981, Complete recovery CAG-Normal Oct.) 5 34 M L 7 (1981). L 6 (1981) Complete recovery 5 6 18 F R 7 (1981, May), R 6 (1981, Prednisolone Mild(R) 7 palsy 8 july) 7 48M L 9, 10, 11, 12 (1980, jan.) Prednisolone Complete recovery Prot. = 38 VAG-Normal Normal Cell = 0 8 43 F L 6 (1981, Aug.). L 7 (1982, Complete recovery 8 Feb.) 9 28 M L 3 (1976). L 3 (1980), R 6 Complete recovery CAG-Normal (1982, March) VAG-Normal 10 50 F R 7 (1975), R 5, 6, (1981) Complete recovery Normal 11 60 F L 7 (1971). R 7 (?). L 3 (1981, Mild (R) ptosis 6 Normal Apr.) 12 50 F L 7 (1962). R 6, 2 (1972). R Prednisolone Mild (L) 7 palsy and 20 CAG-Normal 6 (1981, July), L 3 (1982, decreased visual Sept.) acuity Notes: CAT: Computerized axial tomography; CAG: Carotid arteriography; VAG: Vertebral arteriography. responded well to oral administration of prednisolone, 60 mg daily for 1 month. In September 1981, she was readmitted with a chief complaint of difficulty in swallowing. She was found to have palatal weakness and a decreased gag reflex on the right. She improved without treatment, and when she was seen again 6 months later, she was asymptomatic. Neurological examination was normal except for a residual right facial weakness. Case 7 A 48-year-old man was admitted to the hospital in January 1980, with a 2-week history of difficulty with speech and swallowing. Neurological examination revealed paresis of the ninth, tenth, 11th and 12th nerves on the left. Skull x-rays, tomography of the foramen jugulare, vertebral angiography, lumbar puncture, computed tomography, and nasopharyngeal biopsy were all normal. Oral prednisolone was begun, 60 mg a day, and moderate recovery occurred in 1 month. Six months later, recovery was almost complete except for a mild 12th nerve paresis. Results Patients ranged in age from 18 to 60. There was no sex preponderance (five men and seven 264 women). The duration of the cranial nerve dysfunction and follow-up ranged from 1 to 27 years. There was no history of diabetes mellitus, tuberculosis, sarcoidosis, syphilis, or any other disease in the past histories. The erythrocyte sedimentation rate was elevated in two cases. Skull x-rays, lumbar puncture, arteriography, and computed tomography were normal in cases in which these tests were performed. Seven patients had unilateral headache as the initial symptom. A single cranial nerve palsy occurred in nine patients, and three patients experienced multiple cranial nerve palsies following the initial symptom. Involvement of the third, sixth, and seventh cranial nerves were found more often, and lower cranial nel>'es were affected in three cases. Discussion Multiple cranial nerve dysfunction can occur with a primarily intracranial process, invasive tumors of the nasopharynx, metastatic neoplasm, or granulomatous meningitis. Although there are other possible etiological factors6- 9 which may cause the snydrome, in the majority of the cases the etiology remains unknown. In 1968, Ratnavale1 reported 40 cases from Ceylon with recurrent multiple cranial nerve palsies in whom no etiology was Journal of Clinical Neuro-ophthalmology found, and the patients responded to steroid treatment. Three years later, Steele and Vasuvae reported 14 more patients from Thailand with similar clinical features. Takahashi3 in 1974, reported seven Japanese patients and called it "migrating disseminated multiple cranial neuropathy." In 1978, Hokkanen et al. 4 added 12 more patients to the literature from Finland. There have been a few other reports from Europe and America,5.fi. 10. II possibly indicating a low incidence of the syndrome there. Our patients and the other reported cases have common clinical features. The symptoms are self-limited and steroid therapy is effective in most cases. Prodromal headache precedes the onset of multiple or single cranial nerve palsies. Recurrence after months or years is not uncommon, and different cranial nerves may be affected. Wider neurological involvement does not occur. The erythrocyte sedimentation rate is elevated in some cases; however, other laboratory examinations are usually within normal limits, and there is no specific test to aid in diagnosis. When only the oculomotor nerves are involved (associated with pain), it may be difficult to distinguish from ophthalmoplegic migraine (as in our case 9) or painful ophthalmoplegia, known as T0losa- Hunt syndrome. This is another distinctive entity of a combination of multiple cranial neuropathies without identifiable disease. First reported by Tolosa13 in an autopsied case and later described by Hunt et al. 12 after analysis of six patients, their criteria included retro-orbital pain, neurological deficits involving any combination of the second, third, fourth, and sixth cranial nerves, and symptoms lasting days or weeks, often with spontaneous remission with a tendency for attacks to recur at intervals of months or years. Since a few reported cases have had histological evidence of "nonspecific" granulomatous involvement of the cavernous sinus or superior orbital fissure 1.3. 14 the etiology of the Tolosa-Hunt syndrome is poorly understood. Since Hunt's original formulation, several case reports have described involvement of the second and third division of the trigeminal nerve, and there are a total of seven reported cases of Tolosa-Hunt syndrome associated with facial nerve palsy.15.16 The authors suggested that Tolosa- Hunt syndrome is related to syndromes of more generalized cranial neuropathy, and its clinical features do not necessarily indicate a single pathological entity. I? Although granulomatous inflammation, 12. 1:\ vasomotor disturbances,5 focal or selective hypersensitivity, 2 and toxic circulating substances l " have been suggested as the probable cause for recurrent cranial neuropathies, none of the authors cited were able to draw definite conclusions regarding the etiology of these conditions. The major conclusion drawn from the previous reports and our experience is that there is some December 1983 Kansu, Us, Sarpel, Ara\ overlap between a syndrome that has been called recurrent multiple cranial neuropathies and painful ophthalmoplegia, known as Tolosa-Hunt syndrome. We also postulate that the condition parallels other transient cranial mononeuropathies, namely, those affecting the seventh and perhaps the fifth and sixth nerves, in which recovery frequently occurs. References 1. Ratnavale, C.5.: Cranial polyneuritis-A distinct clinical entity. Proc. Aust. Assoc. Neurol. 5: 527-529, 1968. 2. Steele, J.C, and Vasuvat, A.: Recurrent multiple cranial nerve palsies: A distinctive syndrome of cranial polyneuropathy. f. Neurol. Neurosurg. Psychiatry 33: 828-832, 1970. 3. Takahashi, A.: Migrating disseminated multiple cranial neuropathy. Clin. Neurol. (Jpn.) 14: 838-843, 1974. 4. Hokkanen, E., Haltia, T., and Myllyla, V.V.: Recurrent multiple neuropathies. fur. Neurol. 17: 32-37, 1978. 5. Symonds, C: Recurrent multiple cranial nerve palsies. f. Neurol. Neurosurg. Psychiatry 21: 95-100, 1958. 6. Aupy, M., Orgogozo, I.M., Loiseau, P., Aparicio, M., and Vital, CL.: Atteinte multiple des nerfs craniens revelant une periaterite noueuse. Revue. Neurol. (Paris) 136: 59-65, 1980. 7. Kreindler, A., and Macovei-Patrichi, M.: Recurrent cranial nerve palsies of dysglobulinemia origin. f. Neurol. Sci. 6: 117-123, 1968. 8. Munsat, T.L., and Barnes, I.E.: Relation of multiple cranial nerve dysfunction to the Cuillane-Barre syndrome. f. Neurol. Neurosurg. Psychiatry 28: 115120, 1965. 9. Siegel, E., and Heidrich, R.: Uber kranielle polyneuritis sowie multiple kranielle neuritis and mononeuritis. Z. Aerztl. Fortbild. (Jena) 59: 633-638, 1965. 10. Castaigne, P., Brunet, P., Pierrot-Deseilligny, C, Rousseaux, P., and Capron. L.: Paralysies multiples et recidivantes des nerfs craniens. Ann. Intern. Med. 130: 479-480, 1979. 11. Contamin, F., Mignot, B., Ollat, H., and Singer, B.: Paralysies multiples et recidivantes de nerfs craniens. A propos du proces-verbal. Ann. Intern. Med. 130: 479-480, 1979. 12. Hunt, W.E.J., Meagher, J.N., Lefever, H.E., and Zeman, W.: Painful opthalmoplegia. Its relation to indolent inflammation of the cavernous sinus. Neurology 11: 56-62,1961. 13. Tolosa, E.: Periarteritis lesion of carotid siphon with clinical features of a carotid infraclinoid aneurysm. f. Neurol. Neurosurg. Psychiatry 17: 300-302, 1954. 14. Schatz, N.)., and Farmer, P.: Tolosa-Hunt syndrome: The pathology of painful ophthalmoplegia. In Neuro-optha/mologv Symposium of the University of Miami and the Bascom Palmer Eye Institute Vol. 6, J. L. Smith, Ed. C V. Mosby Co., St. Louis, 1972. pp. 102-] 12. 15. Swerdlow, B.: Tolosa-Hunt syndrome. A case with associated facial nerve palsy. Ann. Neurol. 8: 542543, 1980. 265 Recurrent Cranial Nerve Palsies 16. Vallat, J.M., Vallat, M., Julien, L Duman, M., and Dany, A.: Painful ophthalmoplegia (Tolosa-Hunt) accompanied by peripheral facial paralysis. Ann. Neurol. 8: 645, 1980. 17. Dornan, T.L., Espir, M.L.E., Gale, LA.M., Tattersall, R.B., and Worthington, B.5.: Remittent painful ophthalmoplegia: The Tolosa-Hunt syndrome? A report of seven cases and review of the literature. f. 266 Neurol. Neurosurg. Psychiatry 42: 270-275, 1979. 18. Scott, M.: Cranial nerve syndromes. Relation to exogenous and endogenous substances. f. Am. Med. Assoc. 203: 893, 1968. Write for reprints to: Tiilay Kansu, M.D., Department of Neurology, Hacettepe University Hospitals, Ankara, Turkey. 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