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Pediatr Neurol 2019 Feb 20. Epub 2019 Feb 20.

Pediatric Department, University Hospital Of Heraklion, Medical School, University of Crete, Heraklion, Crete, Greece. Electronic address:

Background: Tolosa-Hunt syndrome (THS) is a rare condition in children characterized by painful ophthalmoplegia caused by inflammation of unknown etiology in the cavernous sinus, superior orbital fissure, or orbital apex. Our main purpose was to report two pediatric cases of THS, a typical one and another extremely rare one preceded by facial palsy.

Methods: Both cases were diagnosed with THS based on the 2013 International Classification of Headache Disorders (ICHD-3 beta) criteria. Read More

BMC Ophthalmol 2019 Feb 11;19(1):48. Epub 2019 Feb 11.

Department of Neurology, Shandong Provincial Hospital affiliated to Shandong University, No 324, Jingwu Road, Huaiyin Zone, Jinan, Shandong Province, 250012, People's Republic of China.

Background: Painful ophthalmoplegia can be caused by various etiologies, and broad differential diagnosis is needed. Carotid-cavernous fistula (CCF) is a rare cause of painful ophthalmoplegia, and early diagnosis is quite difficult.

Case Presentation: Here, we present a case of paroxysmal painful ophthalmoplegia caused by CCF. Read More

Neurol Sci 2019 Mar 26;40(3):543-551. Epub 2018 Dec 26.

Neurology Department, Hospital de Santo António, Centro Hospitalar do Porto, Porto, Portugal.

Background: Hypertrophic pachymeningitis (HP) is characterized by cranial and/or spinal thickening of the dura mater with or without associated inflammation. Neuroimaging studies reveal dura mater thickening and focal or diffuse contrast enhancement. It is described in association with trauma, infections, tumors, autoimmune/inflammatory diseases, and cerebrospinal fluid hypotension syndrome, with some cases remaining idiopathic. Read More

Turk J Emerg Med 2018 Sep 31;18(3):134-136. Epub 2017 Oct 31.

Mersin University, Faculty of Medicine, Department of Neurology, Mersin, Turkey.

Headache and double vision symptoms can be seen especially neurological and optical problems. We present Tolosa-Hunt syndrome in a 34-year-old female patient who presented to the emergency department (ED) with the complaints of unilateral severe headache, double vision and ptosis, presented asymmetric contrast enhancement in the right superior cavernous sinus on MR. This pain was reduced with steroid therapy. Read More

Pract Neurol 2018 10 30;18(5):350-351. Epub 2018 May 30.

Department of Neurology, The Canberra Hospital, Canberra, Australia.

Neuroophthalmology 2018 Jun 6;42(3):159-163. Epub 2017 Sep 6.

Neurosurgical Service, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, Massachusetts, USA.

The Tolosa-Hunt syndrome is a rare clinical condition characterized by painful opthalmoparesis associated with idiopathic granulomatous inflammation of the orbital apex and cavernous sinus. Historically, this condition was thought to result from arteritic changes in the internal carotid artery and cavernous sinus. Modern digital angiographic techniques were unavailable when THS was initially described, and few reports exist on its high-resolution angiographic findings. Read More

Eur J Intern Med 2018 11;57:e6-e7

Diagnosezentrum Lahr, Turmstrasse 21, 77933 Lahr, Germany.

Intern Med 2018 Sep 27;57(18):2735-2738. Epub 2018 Apr 27.

Department of Neurology and Neuroscience, Nagoya City University Graduate School of Medical Sciences, Japan.

We herein report a patient with Miller Fisher syndrome mimicking Tolosa-Hunt syndrome. A 47-year-old man presented with right orbital pain and diplopia. On a neurological examination, he had right oculomotor nerve palsy and diminished deep tendon reflexes. Read More

J Clin Neurosci 2018 Jul 21;53:239-240. Epub 2018 Apr 21.

Mayo Clinic, 200 1st Street SW, Rochester, MN 55905, USA. Electronic address:

Tolosa Hunt syndrome (THS) is characterized by painful ophthalmoplegia secondary to idiopathic granulomatous inflammation of the cavernous sinus. The characteristic finding on MRI is an enhancing T1 isointense and T2 hypo- or hyperintense cavernous sinus mass lesion, which may result in focal narrowing of the ipsilateral internal carotid artery. Although the incidence is quite rare, it is a common diagnostic consideration in cases that present with multiple cranial neuropathies. Read More

Pract Neurol 2018 Oct 12;18(5):373-377. Epub 2018 Apr 12.

Department of Neurology, Concord Repatriation General Hospital, Concord, New South Wales, Australia.

A 63-year-old man presented with a 2-month history of progressive right-sided exophthalmos, painful ophthalmoplegia and fevers. As more features developed, he was diagnosed with giant cell arteritis, then Tolosa-Hunt syndrome, and transiently responded to corticosteroids. A bland cerebrospinal fluid and highly metabolically active brain (F)-fluoro-D-glucose-positron emission tomography suggested lymphoma. Read More

J Clin Neurol 2018 Jan;14(1):126-127

Department of Neurology, American University Medical Center, Beirut, Lebanon.

Neurohospitalist 2018 Apr 13;8(2):104-105. Epub 2017 Jun 13.

Department of Neurology, Detroit Receiving Hospital, Detroit, MI, USA.

Headache 2018 05 8;58(5):758-760. Epub 2018 Mar 8.

Department of Neurology, Akershus University Hospital, Lørenskog, Norway.

Acta Clin Croat 2017 Jun;56(2):331-337

Clinical Department of Neurology, Sestre milosrdnice University Hospital Center, Zagreb, Croatia.

This case report presents a patient diagnosed with Tolosa-Hunt syndrome (THS) after an extensive neuro-diagnostic and neuroimaging evaluation. Diagnostic work-up included thorough physical and neurological examination, complete laboratory serum assessments, neuroendocrine and immunohistochemistry analysis, cerebrospinal fluid analysis, neurophysiology assessment, ophthalmologist examination and neuroimaging. Th e most important diagnostic tool in deriving the diagnosis of THS was neuroimaging evaluation that included baseline and follow-up cranial contrast enhanced magnetic resonance imaging (MRI). Read More

Zhonghua Yi Xue Za Zhi 2018 Jan;98(3):202-207

Department of Neurology, Beijing Tongren Hospital, Capital Medical University, Beijing 100730, China.

To classify different causes of cavernous sinus lesion in patients with ophthalmoplegia and analyze their clinical and imaging features. We confirmed the etiological diagnosis of 137 hospitalized ophthalmoplegia patients with cavernous sinus lesion retrospectively from January 2005 to January 2014 in the Department of Neurology of Beijing Tongren Hospital.The diagnosis was made according to clinical feature, laboratory test, imaging studies and pathology. Read More

Cephalalgia 2018 09 23;38(10):1696-1700. Epub 2017 Nov 23.

1 Department of Neurosurgery, Icahn School of Medicine at Mount Sinai, New York, NY, USA.

Introduction The term Tolosa-Hunt Syndrome was first used more than half a century ago to describe painful ophthalmoplegia accompanied by cranial nerve palsies. In the decades since, its diagnostic criteria have evolved considerably. The beta version of the 3rd Edition of the International Classification of Headache Disorders narrows these criteria to require the demonstration of granulomatous inflammation on MRI or biopsy. Read More

Noro Psikiyatr Ars 2017 Sep 1;54(3):251-254. Epub 2016 Sep 1.

Department of Radiology, Ondokuz Mayıs University School of Medicine, Samsun, Turkey.

Introduction: The etiology of Tolosa-Hunt Syndrome (THS) is still unknown. The initial standard magnetic resonance imaging (MRI) may not be sufficient for diagnosis, so dynamic contrast-enhanced MRI may be necessary to demonstrate the presence of lesions.

Methods: Seven patients diagnosed with THS according to the International Headache Society criteria (beta version) were included into the study. Read More

Endocrine 2017 Dec 14;58(3):582-586. Epub 2017 Oct 14.

Department of Endocrinology and Internal Medicine, Medical University of Gdańsk, Gdańsk, Poland.

The authors report a rare case of bilateral Tolosa-Hunt syndrome, which occurred in a 80-year-old female and remitted spontaneously. Inflammatory lesions were found not only in typical locations, i.e. Read More

Intern Med 2017 Oct 15;56(20):2769-2772. Epub 2017 Sep 15.

Komoro Kosei General Hospital, Japan.

Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare condition that manifests as headache and ophthalmoplegia. It typically occurs in children. Although migraine or neuropathy have been suggested as etiologies, the precise etiology remains unclear. Read More

Front Neurol 2017 18;8:409. Epub 2017 Aug 18.

Department of Neurology, University of Florida, Gainesville, FL, United States.

Merkel cell carcinoma (MCC) is an uncommon but highly malignant neuroendocrine tumor of the skin. MCC can metastasize, but involvement of the central nervous system is rare. Here, we report a case of rapidly progressing metastatic MCC to the clivus and bilateral cavernous sinus in an immunocompromised patient. Read More

Clin Exp Rheumatol 2017 May-Jun;35 Suppl 104(2):15-16. Epub 2017 May 29.

Paediatric Rheumatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy.

BMJ Case Rep 2017 May 27;2017. Epub 2017 May 27.

Department of Medicine, Midnapore Medical College & Hospital, Midnapore, West Bengal, India.

J Assoc Physicians India 2017 Feb;65(2):82-84

Additional Chief Heath Director, B.R Singh Hospital and Centre for Medical Education and Research, Kolkata, West Bengal.

Tolosa- Hunt syndrome is a rare steroid responsive disorder caused by granulation tissue involving the cavernous sinus or superior orbital fissure presenting as painful ophthalmoplegia and facial pain. In this report, we describe coexistence of Tolosa-Hunt syndrome with ocular myasthenia which may point towards an autoimmune etiological basis behind the cavernous sinus granulation tissue formation and also offered therapeutic challenge to ameliorate the symptoms. Read More

Cephalalgia 2017 Jun 11;37(7):705-713. Epub 2017 Apr 11.

1 Department of Neurology, Lindenbrunn Hospital, Coppenbrügge, Germany.

Premise This review summarises the pain syndromes that overlap between headache and facial pain and overlap between pain and cranial nerve lesion. Problem These syndromes share two features in common. First, they show both cranial nerve impairment (e. Read More

Rinsho Shinkeigaku 2017 04 30;57(4):163-167. Epub 2017 Mar 30.

Department of Neurological Science, Nippon Medical School Hospital.

A 66-year-old man presented with headache and ophthalmalgia. Diplopia developed, and he was hospitalized. The left eye had abducent paralysis and proptosis. Read More

J Ophthalmic Vis Res 2017 Jan-Mar;12(1):106-109

Department of OMF Surgery, GDC Jammu, Jammu and Kashmir, India.

Purpose: To highlight the role of constructive interference steady state (CISS) magnetic resonance imaging (MRI) in the diagnosis of Tolosa-Hunt Syndrome (THS).

Case Report: We describe a case of THS in a 55-year-old woman presenting with left painful opthalmoplegia that was diagnosed by CISS MRI. Patient responded to steroid treatment and the lesion resolved. Read More

Int J Gynaecol Obstet 2017 06 31;137(3):340-341. Epub 2017 Mar 31.

Department of Obstetrics and Gynecology, McGill University, Montreal, QC, Canada.

Clin Neurol Neurosurg 2017 Apr 22;155:63-69. Epub 2017 Feb 22.

Department of Neurology, Postgraduate Institute of Medical Education and Research (PGIMER), Chandigarh, India. Electronic address:

Objectives: To study the clinical and etiological profile of patients with cavernous sinus syndrome (CSS) and identify factors which could determine the etiology and influence the outcome of these patients.

Patients And Methods: This prospective observational study included 73 consecutive patients satisfying the criteria of CSS (i.e. Read More

J Investig Med High Impact Case Rep 2017 Jan-Mar;5(1):2324709616689478. Epub 2017 Jan 1.

UIC/Advocate Christ Medical Center, Oak Lawn, IL, USA.

Tolosa-Hunt syndrome is a rare disease with a limited number of cases reported in the literature. It typically presents with orbital pain associated with palsy of the third, fourth, or sixth cranial nerve. We present an interesting case of Tolosa-Hunt syndrome in a young male who responded well to high-dose steroids and in a few days had significant improvement in his retro-orbital pain and ocular movements. Read More

Surg Neurol Int 2016 11;7(Suppl 30):S779-S784. Epub 2016 Nov 11.

Department of Neurosurgery, St. Marianna University School of Medicine, Kawasaki, Japan.

Background: We report a case of steroid-resistant Tolosa-Hunt syndrome (THS) with recurrent bilateral painful ophthalmoplegia, accompanied with sphenoid sinusitis, pituitary abscess, and an aneurysm arising from the cavernous portion of the internal carotid artery.

Case Description: A 53-year-old woman suffered severe left painful ophthalmoplegia. A magnetic resonance image (MRI) revealed thickness of the left cavernous sinus (CS). Read More

Radiol Clin North Am 2017 Jan;55(1):83-101

Division of Neuroradiology, Department of Radiology, Keck School of Medicine, University of Southern California, 1200 North State Street, Room 3740A, Los Angeles, CA 90033, USA.

The sella and parasellar region, found between the anterior and central skull base, represents the central aspect of the skull base. Given the location of the pituitary gland in this location, small lesions in this location may have major physiologic effects on the human body. This article reviews the anatomy, development, and pathologic processes that may involve this region. Read More

BMJ Case Rep 2016 Nov 21;2016. Epub 2016 Nov 21.

Üsküdar University, Istanbul, Turkey.

Am J Ophthalmol Case Rep 2017 Apr 15;5:41-43. Epub 2016 Nov 15.

Vittala International Institute of Ophthalmology, Bangalore, India.

Purpose: We report an interesting case of atypical Tolosa Hunt syndrome with bilateral Sclerokeratitis and optic atrophy in a patient with Takayasu's arteritis.

Observations: A 31-year- old lady presented with severe retroorbital pain in the right eye and right sided headache of 2 weeks duration. She had a history of 6th and 7th nerve palsies which improved with oral corticosteroids. Read More

Med Clin (Barc) 2016 Nov 25;147(9):420. Epub 2016 Aug 25.

Servicio de Urgencias, Hospital Universitario Central de Asturias, Oviedo, Asturias, España. Electronic address:

Clin Exp Optom 2017 May 15;100(3):291-293. Epub 2016 Sep 15.

College of Optometry, Nova Southeastern University, Fort Lauderdale, Florida, USA.

Rev Neurol 2016 Sep;63(6):284-5

HUMS. Hospital Universitario Miguel Servet, 50009 Zaragoza, Espana.

Pediatr Neurol 2016 09 25;62:18-26. Epub 2016 Jun 25.

Division of Community and General Pediatrics, Department of Pediatrics, University of Texas Health Science Center at Houston, Houston, Texas.

Background: Tolosa-Hunt syndrome is a painful ophthalmoplegia caused by an inflammatory process of unknown etiology in the region of the cavernous sinus, orbital apex, or superior orbital fissure. This disease is rare in the pediatric population. The objective of this study was to provide a clinical framework for the evaluation and treatment of children with this disorder. Read More

J Neurosurg 2017 May 24;126(5):1479-1483. Epub 2016 Jun 24.

Department of Neurosurgery, Clinical Neurosciences Center, and.

Epstein-Barr virus (EBV)-associated smooth muscle tumors (SMTs) have recently been associated with primary and secondary immunodeficiencies. They are broadly divided into 3 subgroups: HIV-related, posttransplant, and congenital immunodeficiency. Subsequent to organ transplantation and acquired immunosuppression, a few cases of EBV-associated SMTs have been described in the liver, respiratory tract, and gastrointestinal system. Read More

Surv Ophthalmol 2017 May - Jun;62(3):378-382. Epub 2016 May 10.

Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas, USA.

A 40-year-old Somali woman presented features of a right-sided cavernous sinus syndrome which was confirmed with neuroimaging. Although initial investigations were equivocal for an infectious etiology, subsequent investigations led to a diagnosis of tuberculosis as the cause for right-sided cavernous sinus syndrome. This case illustrates that, although the incidence of tuberculosis cavernous sinus syndrome is reportedly low, patients originating from tuberculosis endemic regions warrant scrupulous investigations in order not to miss the diagnosis and effect appropriate treatment. Read More

J Clin Diagn Res 2016 Mar 1;10(3):TD16-7. Epub 2016 Mar 1.

Head of Department, Institute of Diagnostic and Interventional Radiology/Neuroradiology, Westküstenklinikum Heide, Academic Teaching Hospital of the Universities of Kiel , Lübeck and Hamburg, Germany .

We present the case of a man of 47 years with vertical and horizontal paresis of view combined with periorbital pain that developed initially on the right side but extended after 3-4 days to the left. Gadolinum uptaking tissue in the cavernous sinus was shown by MRI of the orbital region in the T1 spin echo sequence with fat saturation (SEfs) with a slice thickness of 2 mm. As no other abnormalities were found and the pain resolved within 72 hours of treatment with cortison a bilateral Tolosa-Hunt Syndrome (THS) was assumed. Read More

Case Rep Med 2016 20;2016:9763621. Epub 2016 Mar 20.

Section of Hospital Medicine, Overlook Medical Center, Summit, NJ 07901, USA.

We report a case of a 50-year-old female with diabetes mellitus who presented with progressive second, third, fifth, sixth, and eighth cranial nerve palsy. Diagnostic investigation revealed hyperglycemic state, and brain imaging showed a right cavernous sinus enhancement suggestive of and consistent with Tolosa-Hunt syndrome. The patient was started on steroids with tight glycemic control for eight weeks; subsequently, the cranial nerve palsies resolved as well as documented resolution of the right cavernous sinus enhancement. Read More

Pediatr Neurosurg 2016 13;51(4):214-7. Epub 2016 Apr 13.

Vanderbilt University School of Medicine, Nashville, Tenn., USA.

Tolosa-Hunt syndrome is an idiopathic inflammatory process of the cavernous sinus or orbit manifesting as painful ophthalmoplegia. In this report, we detail the case of a 6-year-old boy who presented with several weeks of unilateral headache and diplopia. He was found to have an infiltrative process involving the bilateral cavernous sinuses and pituitary gland on MRI. Read More

Neurologia 2018 Jan - Feb;33(1):68-70. Epub 2016 Mar 11.

Servicio de Neurología, Unidad de Cefaleas, Hospital Clínico Universitario San Carlos, Madrid, España. Electronic address:

Agri 2015 ;27(4):219-23

Antalya Yasam Hospital, Antalya, Turkey.

Painful ophthalmoplegia consists of periorbital or hemicranial pain with ipsilateral ocular motor nerve palsies. There are many etiologies of painful ophthalmoplegia. Tolosa-Hunt syndrome (THS) is an uncommon disease caused by non-specific inflammation of the cavernous sinus, superior orbital fissure and the apex of the orbit. Read More

Cephalalgia 2016 Dec 3;36(14):1392-1396. Epub 2016 Feb 3.

Department of Neurology, Ludwig-Maximilians University, Munich, Germany.

Background: Tolosa-Hunt syndrome (THS) is characterized by unilateral orbital pain, ipsilateral oculomotor paresis and a prompt response to treatment with corticosteroids. Several reports have demonstrated that the clinical features of THS are not specific to one causal aetiology and can lead to misdiagnosis.

Case Report: We report the case of a patient diagnosed with THS after an episode of unilateral orbital pain and diplopia with demonstration of granulomatous inflammation of both cavernous sinus on cerebral magnetic resonance imaging and an immediate response to treatment with corticosteroids. Read More

Cephalalgia 2017 Feb 11;37(2):191-194. Epub 2016 Jul 11.

1 Neurology Department, Hospital Egas Moniz - Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal.

Introduction Tolosa-Hunt syndrome (THS) is one of the most common 'benign' causes of painful ophthalmoplegia. Diagnosis is based on clinical and imaging findings and the exclusion of other causes because there is no specific biomarker for the syndrome. Eales disease, an idiopathic inflammatory venous disease that primarily affects the eye, can also affect the central (as stroke or myelitis) and peripheral nervous system. Read More

Cephalalgia 2016 Dec 28;36(14):1397-1398. Epub 2016 Jan 28.

Department of Neurology, Mitsui Memorial Hospital, Japan.

Am J Obstet Gynecol 2016 07 7;215(1):103.e1-103.e14. Epub 2016 Jan 7.

Oregon Health and Science University, Portland, OR.

Background: Although preterm birth <37 weeks' gestation is the leading cause of neonatal morbidity and mortality in the United States, the majority of data regarding preterm neonatal outcomes come from older studies, and many reports have been limited to only very preterm neonates. Delineation of neonatal outcomes by delivery gestational age is needed to further clarify the continuum of mortality and morbidity frequencies among preterm neonates.

Objective: We sought to describe the contemporary frequencies of neonatal death, neonatal morbidities, and neonatal length of stay across the spectrum of preterm gestational ages. Read More

Acta Neurochir (Wien) 2016 Jan 27;158(1):143-5. Epub 2015 Nov 27.

Department of Neurosurgery, Research Institute of Clinical Medicine, Institute for Medical Science, Chonbuk National University Medical School and Hospital, Jeonju, Korea.

Tolosa-Hunt syndrome is a rare cause of painful ophthalmoplegia due to idiopathic chronic granulomatous inflammation in the cavernous sinus. Usually clinical manifestations are well controlled by corticosteroid therapy, but steroid dependency or resistance is common. We report a case of marked improvement of Tolosa-Hunt syndrome without symptom relapse after Gamma Knife radiosurgery in a patient with steroid intolerance. Read More