Abstract
Ten patients (6F, 4M) with recurrent Tolosa-Hunt syndrome are reported. Besides ocular motor symptoms, one patient had trigeminal nerve involvement, one had ipsilateral ocular sympathicoplegia with miosis and ptosis, and one tinnitus during an episode of Tolosa-Hunt syndrome, ipsilateral to the pain side. One patient had Bell's palsy, one had a possible Raeder's syndrome, and one had a period of tinnitus between the Tolosa-Hunt syndrome episodes. Three of the 10 patients reported periods of periocular pain without ophthalmoplegia between the Tolosa-Hunt episodes, the pain located ipsilateral to the ophthalmoplegic side in the Tolosa-Hunt episodes. Systemic symptoms associated with Tolosa-Hunt syndrome, e.g., back pain, chronic fatigue, arthralgia, gut problems among others, occurred with the same frequency in these 10 patients as in an earlier report. Seventy per cent of the patients had signs of inflammation in serum during a period of Tolosa-Hunt syndrome. Orbital phlebograms showed pathologic signs in four of the five patients investigated during a Tolosa-Hunt period. One phlebogram was normal in a sixth patient when performed during a period of unilateral periocular pain without ophthalmoplegia. Magnetic resonance imaging of the head (with contrast) was only performed in three patients during the Tolosa-Hunt period: one showed signs of inflammation in the middle fossa and two were normal. In one of the patients with normal magnetic resonance imaging, the orbital phlebogram was pathologic. Steroid treatment promptly relieved the pain in all patients.
Tolosa-Hunt syndrome is a rare disorder. Contrary to painful ophthalmoplegia, Tolosa-Hunt syndrome is recurrent and usually ends spontaneously after a period of weeks or months. The International Headache Society (IHS) criteria for Tolosa-Hunt syndrome are set out in Table 1 (1). Most reports deal with only a few patients and sometimes with patients who have had only one episode, which makes them less relevant (2). The results of an earlier study of 20 consecutive “recurrent Tolosa-Hunt patients” (3) are compared with the findings in the present series of 10 consecutive Tolosa-Hunt patients (3) with, at least, two episodes of painful ophthalmoplegia.
Tolosa-Hunt syndrome according to IHS taxonomy (1).
Patients and methods
Ten patients (6F, 4M) each with at least two episodes of Tolosa-Hunt syndrome were studied with regard to the occurrence of painful ophthalmoplegia in their parents and with their earlier medical history. The following information was obtained: signs of inflammation in serum (serum electrophoresis and tests for rheumatoid and anti-nuclear factors), findings in neuroradiological investigations of the patients (including computed tomography of the head), findings with magnetic resonance imaging of the brain with contrast and orbital phlebography, during a period of Tolosa-Hunt syndrome, prior to treatment. Evaluation of pathological signs in the orbital phlebograms was the same as in an earlier study (3). The magnetic resonance imaging with contrast was performed with gadopentate dimeglumine.
Results
Demographics and heredity
Of the 10 consecutive patients with recurrent Tolosa-Hunt syndrome, one reported that his father had suffered from a period of ophthalmoplegia with periocular pain, which had ameliorated spontaneously. Further information about the father was not possible to obtain.
Unilateral, periorbital pain between episodes of Tolosa-Hunt syndrome
Three patients reported episodic or chronic, unilateral periocular pain without ophthalmoplegia between Tolosa-Hunt episodes. The pain was ipsilateral to the symptoms.
Cranial nerves involved
During an episode of Tolosa-Hunt syndrome one patient also had involvement of the two upper trigeminal branches; one had ocular sympaticoplegia with miosis and ptosis and one had tinnitus, all ipsilateral to the syndrome side.
Between Tolosa-Hunt syndrome episodes, one patient had Bell's palsy and one had a possible Raeder's syndrome, i.e., ipsilateral periocular pain, miosis, ptosis and involvement of the two upper trigeminal branches; one had a period of vertigo with tinnitus.
Other symptoms
Eight of the 10 patients (4F, 4M) had suffered from back pain, 6 from chronic fatigue (5F, 1M), 6 from non-rheumatoid arthralgia (4F, 2M), 6 from gut problems (4F, 2M), 4 from cold feet (3F, 1M), 3 from vertigo (1F, 2M), and 3 reported spontaneously occurring ecchymoses (all women) (Table 2).
Frequency of associated symptoms in 10 patients with recurrent Tolosa-Hunt syndrome in the present study. For comparison, the findings of an earlier study of 20 patients with recurrent Tolosa-Hunt syndrome are given in parentheses.
Signs of inflammation in serum and cerebrospinal fluid
Five of the 7 patients (71%) showed signs of inflammation in electrophoresis of serum when investigated during an episode of Tolosa-Hunt syndrome. Tests for cells, proteins, and electrophoresis of cerebrospinal fluid (n = 4) were all normal except for an increase of protein in one patient (1.0 g/L).
Neuroradiologic investigations during symptomatic periods
Orbital phlebograms were performed in 5 patients during an episode of non-treated Tolosa-Hunt syndrome; 4 showed signs considered pathological. A sixth patient had normal orbital phlebography during a long-lasting episode of unilateral, periocular pain without ophthalmoplegia.
Computed tomography scans of the head of 8 of the 10 patients were all normal except for signs of cerebral atrophy in 2 of the 3 patients older than 60 years. Magnetic resonance imaging with contrast was performed in three patients during an episode of Tolosa-Hunt syndrome prior to steroid treatment. Only one showed pathologic changes in the cavernous sinus. In this patient, orbital phlebography was also pathologic. The phlebogram was pathologic in only one of the other two patients with normal magnetic resonance imaging investigated with orbital phlebography.
Treatment of Tolosa-Hunt syndrome
All 10 patients responded promptly with a decrease in pain on prednisolone treatment in doses of 0.5–1.0 mg/kg body weight. Amelioration of the cranial neuropathies varied in time, but there were no permanent sequelae in this group of 10 patients.
Discussion
We have earlier reported the findings in 20 consecutive patients with recurrent Tolosa-Hunt syndrome (3) and the findings in the 10 patients in the present study confirm the earlier results. However, while in the earlier study there were 6 women and 14 men, this present study comprised 6 women and 4 men.
The report of one patient that his father had suffered from, at least, one episode of painful ophthalmoplegia may possibly support the notion suggested in the previous study that Tolosa-Hunt syndrome is a hereditary disorder. The findings of episodic or chronic, unilateral periocular pain between the periods of Tolosa-Hunt syndrome agrees with the findings of the earlier study. The folding of one episode of Tolosa-Hunt syndrome associated with ipsilateral miosis and ptosis in these 10 patients shows that sympathetic nerve involvement is not always absent in Tolosa-Hunt syndrome, although it may still be considered rare. The findings of involvement of other cranial nerves between episodes of Tolosa-Hunt syndrome, as in the previous study, indicate that Tolosa-Hunt syndrome may possibly be considered a manifestation of what has been termed recurrent multiple cranial neuropathy.
In the earlier study (3) backache, cold feet, arthralgia, gut problems, vertigo, chronic fatigue, and spontaneous ecchymoses were all reported to occur fairly frequently in patients with Tolosa-Hunt syndrome. The occurrence of these symptoms was similar in the present study (Table 2). Along with the signs of inflammation in serum, these findings support the notion that Tolosa-Hunt syndrome is only one manifestation of a systemic disease of presumably inflammatory character.
Orbital phlebography has been reported to be pathologic in Tolosa-Hunt syndrome (4, 5). In later years, magnetic resonance imaging with contrast has been found to be useful for diagnosing Tolosa-Hunt syndrome (6). However, in the two patients investigated with both techniques in the present study only orbital phlebography showed signs of pathology in both patients. In an earlier study of three patients with Tolosa-Hunt syndrome, the results were the opposite, three had pathologic changes in the magnetic resonance images but only one in the orbital phlebogram (7). Further studies of the advantages of these techniques for diagnosing Tolosa-Hunt syndrome are warranted. For the time being, both techniques can be used, together when needed, for proper diagnosis.
The treatment of Tolosa-Hunt syndrome is still primarily steroids.