A Rare Case of Tolosa-Hunt Syndrome Imaged With FDG PET/CT and MRI

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I

NTERESTING

I

MAGE

A Rare Case of Tolosa-Hunt Syndrome Imaged With FDG PET/CT

and MRI

Yu-Chin Wu, MD,† Te-Chun Hsieh, MD,‡ Chia-Hung Kao, MD,§ Yen-Liang Liu, MD,¶*

Kuo-Yang Yen, RT,‡ and Shung-Shung Sun, MD‡

Abstract: We report on the imaging findings of Tolosa-Hunt syndrome in a 59-year-old patient. Clinical findings included periorbital pain, ptosis, dis-ordered eye movements, and blurred vision. Treatment with intravenous administration of steroid resolved all symptoms. Currently, magnetic reso-nance imaging plays a key role in the diagnosis of Tolosa-Hunter syndrome for locating the inflammatory tissue and follow-up. This case of Tolosa-Hunter syndrome with representative (FDG PET/CT) images may imply that FDG PET/CT is a useful tool in detecting and monitoring of this disease. Key Words: Tolosa-Hunt syndrome, FDG PET/CT, MRI

(Clin Nucl Med 2011;36: 574 –575)

REFERENCES

1. Sugie K, Morikawa M, Taoka T, et al. Serial neuroimaging in Tolosa-Hunt syndrome with acute bilateral complete ophthalmoplegia. J Neuroimaging. 2011;21:79 – 82.

2. Colnaghi S, Versino M, Marchioni E, et al. ICHD-II diagnostic criteria for Tolosa-Hunt syndrome in idiopathic inflammatory syndromes of the orbit and/or the cavernous sinus. Cephalalgia. 2008;28:577–584.

3. Schuknecht B, Sturm V, Huisman TA, et al. Tolosa-Hunt syndrome: MR

imaging features in 15 patients with 20 episodes of painful ophthalmoplegia.

Eur J Radiol. 2009;69:445– 453.

4. Zanus C, Furlan C, Costa P, et al. The Tolosa-Hunt syndrome in children: a case report. Cephalalgia. 2009;29:1232–1237.

5. Smith JL, Taxdal DS. Painful ophthalmoplegia. The Tolosa-Hunt syndrome.

Am J Ophthalmol. 1966;61:1466 –1472.

6. Abalo-Lojo JM, Gonzalez F, Pereiro-Zabala I. Metastatic B-cell lymphoma of the cecum masquerading as Tolosa-Hunt syndrome. Can J Ophthalmol. 2007;42:323–325.

7. O’Connor G, Hutchinson M. Tolosa-Hunt syndrome responsive to infliximab therapy. J Neurol. 2009;256:660 – 661.

8. Smith JR, Rosenbaum JT. A role for methotrexate in the management of non-infectious orbital inflammatory disease. Br J Ophthalmol. 2001;85: 1220 –1224.

9. Hatton MP, Rubin PA, Foster CS. Successful treatment of idiopathic orbital inflam-mation with mycophenolate mofetil. Am J Ophthalmol. 2005;140:916–918. 10. Furukawa Y, Yamaguchi W, Ito K, et al. The efficacy of radiation

mono-therapy for Tolosa-Hunt syndrome. J Neurol. 257:288 –290.

11. Jain R, Sawhney S, Koul RL, et al. Tolosa-Hunt syndrome: MRI appearances.

J Med Imaging Radiat Oncol. 2008;52:447– 451.

12. Colnaghi S, Versino M, Marchioni E, et al. A prospective multicentre study to evaluate the consistency of the IHS diagnostic criteria, the usefulness of brain MRI for the diagnosis, follow-up and treatment management, and the outcome after high dosage 6-methylprednisolone therapy, in subjects with Tolosa-Hunt syndrome. J Headache Pain. 2010;11:285.

13. Oyen WJ, Mansi L. FDG-PET in infectious and inflammatory disease. Eur

J Nucl Med Mol Imaging. 2003;30:1568 –1570.

14. Kresnik E, Mikosch P, Gallowitsch HJ, et al. F-18 fluorodeoxyglucose positron emission tomography in the diagnosis of inflammatory bowel dis-ease. Clin Nucl Med. 2001;26:867.

15. Blockmans D, Maes A, Stroobants S, et al. FDG positron emission tomo-graphic scintigraphy can reveal Castleman’s disease as a cause of inflamma-tion. Clin Nucl Med. 2001;26:975–976.

16. Bakheet SM, Powe J, Kandil A, et al. F-18 FDG uptake in breast infection and inflammation. Clin Nucl Med. 2000;25:100 –103.

Received for publication July 26, 2010; revision accepted September 23, 2010. From the *Department of Nuclear Medicine and PET Center, China Medical

Uni-versity and Hospital, and †China Medical UniUni-versity, Taichung, Taiwan; ‡De-partment of Biomedical Imaging and Radiological Science, China Medical University and Hospital, Taichung, Taiwan; §School of Medicine, China Medical University and Hospital, Taichung, Taiwan; and ¶Department of Neurology, China Medical University and Hospital, Taichung, Taiwan.

Reprints: Shung-Shung Sun, MD, and Te-Chun Hsieh, MD, Department of Nuclear Medicine and PET Center, China Medical University Hospital, No. 2, Yuh-Der Road, Taichung 404, Taiwan. E-mail: [email protected]. and [email protected].

Clinical Nuclear Medicine • Volume 36, Number 7, July 2011

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FIGURE 1. A 59-year-old woman presented with periorbital pain and dizziness for more than 10 days. Despite symptomatic

treatment with acetaminophen and prochlorperazine, symptoms persisted and aggravated in the following week. Additionally,

she presented with ptosis, disordered eye movements, and blurred vision (right third and bilateral sixth cranial nerve palsy).

However, computed tomography of the brain did not reveal any lesions. Laboratory testing including complete blood count, serum

chemistry profile, serum tumor markers, serum rheumatologic testing, and cerebrospinal fluid analysis was unremarkable

except for an elevated erythrocyte sedimentation rate of 41 mm/h (normal range, 0 –20 mm/h). Subsequently, magnetic resonance

imaging (A) showed bilateral cavernous sinus/sella enhancement with extension to right orbital fissure on contrasted T1-weighted

images.

1

_{F-18 fluorodeoxyglucose positron emission tomography and computed tomography (FDG PET/CT; B) demonstrated a}

hypermetabolic lesion in the corresponding area. Thus, the condition was considered to be an inflammatory process and not to be

primary/metastatic brain tumor, carotid-cavernous fistula, sinus thrombosis, multiple sclerosis, or central nervous system

infection. Thus, the presumptive diagnosis was Tolosa-Hunt syndrome. For that, she was treated with intravenous hydrocortisone

(300 mg/d for 5 days) followed by a tapering oral dose with prednisolone 20 mg/d. One week later, she was well and discharged.

Four weeks after discharge, she had similar symptoms again. Also, treatment with intravenous administration of high-dose steroid

resolved all symptoms. She remained stable for more than 5 months after recurrence. Tolosa-Hunter syndrome is a rare disorder

caused by granulomatous inflammatory processes with a typical relapsing-remitting course, characterized by painful

ophthalmoplegia, oculomotor paresis, and a dramatic response to corticosteroids.

2– 4

_{The use of corticosteroids has been suggested}

as a diagnostic test as well as a therapeutic procedure.

5,6

_{Additionally, there have been a number of other therapies used in treating this}

condition including methotrexate, mycophenolate mofetil, infliximab, and radiotherapy.

7–10

_{Currently, magnetic resonance}

imaging plays a key role in diagnosis of Tolosa-Hunter syndrome for locating the inflammatory tissue and follow-up.

11,12

_{It is}

well recognized that FDG PET/CT is helpful to detect inflammatory tissue.

13–16

A Rare Case of Tolosa-Hunt Syndrome Imaged With FDG PET/CT and MRI

I

NTERESTING

I

MAGE

A Rare Case of Tolosa-Hunt Syndrome Imaged With FDG PET/CT

and MRI

Yu-Chin Wu, MD,† Te-Chun Hsieh, MD,‡ Chia-Hung Kao, MD,§ Yen-Liang Liu, MD,¶*

Kuo-Yang Yen, RT,‡ and Shung-Shung Sun, MD‡

REFERENCES

Clinical Nuclear Medicine • Volume 36, Number 7, July 2011

FIGURE 1. A 59-year-old woman presented with periorbital pain and dizziness for more than 10 days. Despite symptomatic

treatment with acetaminophen and prochlorperazine, symptoms persisted and aggravated in the following week. Additionally,

she presented with ptosis, disordered eye movements, and blurred vision (right third and bilateral sixth cranial nerve palsy).

However, computed tomography of the brain did not reveal any lesions. Laboratory testing including complete blood count, serum

chemistry profile, serum tumor markers, serum rheumatologic testing, and cerebrospinal fluid analysis was unremarkable

except for an elevated erythrocyte sedimentation rate of 41 mm/h (normal range, 0 –20 mm/h). Subsequently, magnetic resonance

imaging (A) showed bilateral cavernous sinus/sella enhancement with extension to right orbital fissure on contrasted T1-weighted

images.

_{F-18 fluorodeoxyglucose positron emission tomography and computed tomography (FDG PET/CT; B) demonstrated a}

hypermetabolic lesion in the corresponding area. Thus, the condition was considered to be an inflammatory process and not to be

primary/metastatic brain tumor, carotid-cavernous fistula, sinus thrombosis, multiple sclerosis, or central nervous system

infection. Thus, the presumptive diagnosis was Tolosa-Hunt syndrome. For that, she was treated with intravenous hydrocortisone

(300 mg/d for 5 days) followed by a tapering oral dose with prednisolone 20 mg/d. One week later, she was well and discharged.

Four weeks after discharge, she had similar symptoms again. Also, treatment with intravenous administration of high-dose steroid

resolved all symptoms. She remained stable for more than 5 months after recurrence. Tolosa-Hunter syndrome is a rare disorder

caused by granulomatous inflammatory processes with a typical relapsing-remitting course, characterized by painful

ophthalmoplegia, oculomotor paresis, and a dramatic response to corticosteroids.

_{The use of corticosteroids has been suggested}

as a diagnostic test as well as a therapeutic procedure.

_{Additionally, there have been a number of other therapies used in treating this}

condition including methotrexate, mycophenolate mofetil, infliximab, and radiotherapy.

_{Currently, magnetic resonance}

imaging plays a key role in diagnosis of Tolosa-Hunter syndrome for locating the inflammatory tissue and follow-up.

_{It is}

well recognized that FDG PET/CT is helpful to detect inflammatory tissue.

_{This case of Tolosa-Hunter syndrome with unique}

representative FDG PET/CT images may imply that FDG PET/CT is a useful tool in detecting and monitoring of this disease.

Clinical Nuclear Medicine • Volume 36, Number 7, July 2011

Imaging Findings of Tolosa-Hunt Syndrome

A Rare Case of Tolosa-Hunt Syndrome Imaged With FDG PET/CT and MRI

I

NTERESTING

I

MAGE

A Rare Case of Tolosa-Hunt Syndrome Imaged With FDG PET/CT

and MRI

Yu-Chin Wu, MD,*† Te-Chun Hsieh, MD,*‡ Chia-Hung Kao, MD,*§ Yen-Liang Liu, MD,¶

Kuo-Yang Yen, RT,*‡ and Shung-Shung Sun, MD*‡

REFERENCES

Clinical Nuclear Medicine • Volume 36, Number 7, July 2011

FIGURE 1. A 59-year-old woman presented with periorbital pain and dizziness for more than 10 days. Despite symptomatic

treatment with acetaminophen and prochlorperazine, symptoms persisted and aggravated in the following week. Additionally,

she presented with ptosis, disordered eye movements, and blurred vision (right third and bilateral sixth cranial nerve palsy).

However, computed tomography of the brain did not reveal any lesions. Laboratory testing including complete blood count, serum

chemistry profile, serum tumor markers, serum rheumatologic testing, and cerebrospinal fluid analysis was unremarkable

except for an elevated erythrocyte sedimentation rate of 41 mm/h (normal range, 0 –20 mm/h). Subsequently, magnetic resonance

imaging (A) showed bilateral cavernous sinus/sella enhancement with extension to right orbital fissure on contrasted T1-weighted

images.

F-18 fluorodeoxyglucose positron emission tomography and computed tomography (FDG PET/CT; B) demonstrated a

hypermetabolic lesion in the corresponding area. Thus, the condition was considered to be an inflammatory process and not to be

primary/metastatic brain tumor, carotid-cavernous fistula, sinus thrombosis, multiple sclerosis, or central nervous system

infection. Thus, the presumptive diagnosis was Tolosa-Hunt syndrome. For that, she was treated with intravenous hydrocortisone

(300 mg/d for 5 days) followed by a tapering oral dose with prednisolone 20 mg/d. One week later, she was well and discharged.

Four weeks after discharge, she had similar symptoms again. Also, treatment with intravenous administration of high-dose steroid

resolved all symptoms. She remained stable for more than 5 months after recurrence. Tolosa-Hunter syndrome is a rare disorder

caused by granulomatous inflammatory processes with a typical relapsing-remitting course, characterized by painful

ophthalmoplegia, oculomotor paresis, and a dramatic response to corticosteroids.

The use of corticosteroids has been suggested

as a diagnostic test as well as a therapeutic procedure.

Additionally, there have been a number of other therapies used in treating this

condition including methotrexate, mycophenolate mofetil, infliximab, and radiotherapy.

Currently, magnetic resonance

imaging plays a key role in diagnosis of Tolosa-Hunter syndrome for locating the inflammatory tissue and follow-up.

It is

well recognized that FDG PET/CT is helpful to detect inflammatory tissue.

This case of Tolosa-Hunter syndrome with unique

representative FDG PET/CT images may imply that FDG PET/CT is a useful tool in detecting and monitoring of this disease.

Clinical Nuclear Medicine • Volume 36, Number 7, July 2011

Imaging Findings of Tolosa-Hunt Syndrome

Yu-Chin Wu, MD,† Te-Chun Hsieh, MD,‡ Chia-Hung Kao, MD,§ Yen-Liang Liu, MD,¶*

Kuo-Yang Yen, RT,‡ and Shung-Shung Sun, MD‡

_{F-18 fluorodeoxyglucose positron emission tomography and computed tomography (FDG PET/CT; B) demonstrated a}

_{The use of corticosteroids has been suggested}

_{Additionally, there have been a number of other therapies used in treating this}

_{Currently, magnetic resonance}

_{It is}

_{This case of Tolosa-Hunter syndrome with unique}