The efficacy of radiation monotherapy for Tolosa-Hunt syndrome

Abstract

Sirs, Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia due to an idiopathic chronic granulomatous inflammation of the cavernous sinus [9]. THS usually improves with corticosteroids, however, in cases showing an insufficient effect of corticosteroid therapy or side effects, alternative therapies such as immunosuppressive agents [3] or focal radiation therapy have been reported [2, 5–7]. We describe a patient with THS who was successfully treated with radiation monotherapy. A 36-year-old man with chronic hepatitis B and impaired glucose tolerance since age 30 developed acute left orbital pain and double vision at the end of July 2006. Neurologic examination on 3 August 2006 demonstrated only mild restriction of abduction of the left eye. Magnetic resonance imaging (MRI) of the brain showed no abnormality including the cavernous sinus region at that time. His illness progressed rapidly and he was admitted to our hospital on 14 August 2006. On admission, body temperature and blood pressure were normal. Neurologic examination demonstrated almost complete ophthalmoplegia in all directions and blepharoptosis without pupil abnormality on the left. Spontaneous left ophthalmic pain was also reported. There were no ophthalmic symptoms on the right and there were no symptoms of lower cranial nerves on both sides. Urinalysis and routine hematological examination were normal. Blood chemistry examination demonstrated mild elevation of aspartate aminotransferase (60 IU/L; normal range 11–34 IU/L), alanine aminotransferase (151 IU/L; normal range 6–39 IU/L) and glycohemoglobin A1c (6.1%; normal range 4.3–5.8%). Surface antigen and core antibody of hepatitis B virus were positive. The following immunological and inflammatory investigations were normal or negative; antinuclear antibody, cytoplasmic and perinuclear antineutrophil cytoplasmic antibody, angiotensin converting enzyme level and b-D-glucan. Chest radiography and Gallium scintigraphy of the whole body showed no abnormality suggestive of systemic inflammatory disease. Cerebrospinal fluid examinations were normal including cytology and culture for bacteria and mycobacteria. Brain MRI showed abnormal soft tissue enhanced with gadolinium within the left cavernous sinus (Fig. 1). Based on a diagnosis of THS, we first considered corticosteroid therapy for THS with simultaneous antiviral therapy for hepatitis; however, the patient refused corticosteroid therapy because of the possible exacerbation of hepatitis. One week after admission, the left pupil was dilated and light response was diminished, indicating progression of THS. Thereafter, the patient received a total of 30 Gy in 15 fractions focused on the left cavernous sinus by three-dimensional conformal radiotherapy (3D-CRT). Left blepharoptosis and ophthalmoplegia gradually improved within 2 weeks after the initiation of radiation therapy; blepharoptosis had almost completely disappeared by the middle of October 2006. Ophthalmoplegia also Y. Furukawa W. Yamaguchi K. Ito T. Hamada H. Miyaji Department of Neurology, Fukui Prefectural Hospital, Fukui, Japan