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Arch Rheumatol 2020 Mar 25;35(1):128-131. Epub 2019 Jun 25.

Department of Internal Medicine, Wonkwang University Hospital, Iksan, South Korea.

Behçet's disease (BD) is a rare, chronic, auto-inflammatory disorder of unknown origin. Mucocutaneous lesions and pan-uveitis constitute the hallmark of BD. Additionally, vessels of all sizes and types are affected. Read More

Clin Imaging 2020 Jun 18;68:71-89. Epub 2020 Jun 18.

NYU Langone Health, Department of Radiology, New York, NY, United States of America. Electronic address:

The cavernous sinus is a complex structure susceptible to a wide variety of vascular, neoplastic and inflammatory pathologies. Vascular pathologies include ICA aneurysms, carotid-cavernous fistulas, cavernous sinus thrombosis, and cavernous hemangioma. Neoplasms that involve the cavernous sinus include pituitary adenoma, meningioma, schwannoma, lymphoma, perineural tumor spread, metastases, and direct tumor invasion. Read More

Indian J Ophthalmol 2020 Jun;68(6):1212-1214

Dr. Rajendra Prasad Centre for Ophthalmic Sciences, New Delhi, India.

Optic perineuritis is an inflammatory disorder involving the optic nerve sheath. It is currently considered as a part of idiopathic orbital inflammatory disease which also includes dacryoadenitis, orbital myositis, superior orbital fissure, and cavernous sinus syndrome (Tolosa hunt syndrome). As it is idiopathic, it is considered a diagnosis of exclusion. Read More

J Clin Med Res 2020 Apr 30;12(4):266-268. Epub 2020 Mar 30.

Department of Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ 07753, USA.

Tolosa-Hunt syndrome is a rare condition involving the orbital and retro-orbital space. The typical symptoms are orbital pain, swelling, headache, palsies of the cranial nerves, and sensory loss in the distribution of the trigeminal nerve. Tolosa-Hunt syndrome relapses and remits with episodes separated by months to years. Read More

Medicine (Baltimore) 2020 Apr;99(17):e19882

Department of Neurology.

Rationale: Tolosa-Hunt syndrome (THS) is rare condition characterized by painful ophthalmoplegia that usually responds well to corticosteroid. About a half of THS patients experience recurrence within intervals of months to years from initial presentation. Recurrence is more common in younger patients, and can be ipsilateral, contralateral, or bilateral. Read More

Case Rep Neurol Med 2020 30;2020:6737018. Epub 2020 Mar 30.

Department of Neurology, Toyohashi Municipal Hospital, Toyohashi, Japan.

Objective: Painful ophthalmoplegia includes nonspecific magnetic resonance imaging (MRI) manifestations and various clinical features including orbital pain and cranial nerve palsies. Treatment for painful ophthalmoplegia remains controversial. The aim of this report was to describe detailed clinical features, MRI findings, treatments, and prognosis of patients with painful ophthalmoplegia. Read More

Can J Ophthalmol 2020 Mar 31. Epub 2020 Mar 31.

University of Toronto, Toronto, Ont.. Electronic address:

Ann Indian Acad Neurol 2020 Mar-Apr;23(2):201-205. Epub 2020 Feb 25.

Department of Neurological Sciences, Neurology Unit, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.

Background: Tolosa-Hunt Syndrome (THS) is one of the causes of cavernous sinus syndrome causing painful ophthalmoplegia. Literature on long-term outcome of this rare condition is scarce.

Aims And Objectives: The aim is to study the recurrence and role of steroid-sparing agents in THS. Read More

Endocr Pract 2020 Mar 11. Epub 2020 Mar 11.

From: Division of Endocrinology, UCLA David Geffen School of Medicine, Los Angeles, California.

J Clin Neurosci 2020 Feb 24;72:458-460. Epub 2019 Dec 24.

Wexham Park Hospital, Frimley Health NHS Foundation Trust, Internal Medicine, Berkshire, UK; University of Cyprus Medical School, Cardiology, Nicosia, Cyprus. Electronic address:

Tolosa-Hunt syndrome is a rare disorder characterized by severe peri-orbital headache and ophthalmoplegia resulting from pseudotumour in the cavernous sinus compressing structures within it, namely cranial nerves III, IV, and VI and the superior divisions of cranial nerve V. We report the case of a 47 year old female who presented with painless left unilateral ptosis and complete external ophthalmoplegia. Magnetic Resonance Imaging (MRI) identified an enhancing heterogeneous mass filling the left cavernous sinus, following the course of the oculomotor nerve. Read More

Eye Brain 2019 12;11:63-72. Epub 2019 Dec 12.

Department of Ophthalmology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, UPMC Eye Center, Pittsburgh, PA 15224, USA.

Orbital apex syndrome is characterized by vision loss from optic neuropathy and ophthalmoplegia due to the involvement of ocular motor nerves in the anatomical region of the orbital apex. Patients could present with signs and symptoms deriving from the involvement of structures within the orbital apex, the superior orbital fissure or the cavernous sinus. The primary focus of the ophthalmologist should be to locate the lesion and then identify its etiology. Read More

Neurohospitalist 2020 Jan 6;10(1):48-50. Epub 2019 Mar 6.

Department of Neurosciences, New York Presbyterian Brooklyn Methodist Hospital, Brooklyn, NY, USA.

The occurrence of Tolosa-Hunt syndrome (THS) in the setting of discoid lupus erythematosus (DLE) has not been previously reported. We report a case of a 55-year-old Chinese man with established cutaneous lupus who presented with 1 week of worsening blurry vision and ptosis of the left eye with severe headache 2 weeks prior to presentation. His cranial nerve examination was significant for left afferent pupillary defect, red desaturation, ptosis, and oculomotor nerve palsy. Read More

Eur J Rheumatol 2019 Dec 3:1-3. Epub 2019 Dec 3.

Department of Pediatrics, University of Alabama, Birmingham, Alabama, USA.

Objective: Tolosa-Hunt syndrome (THS) is a rare disease characterized by painful unliteral ophthalmoplegia and headache. THS is caused by granulomatous inflammation of the cavernous sinus, and its diagnosis is typically made by elimination and exclusion. The characteristic pain can typically be managed with steroid therapy, but relapses are common. Read More

Headache 2020 Jan 4;60(1):259-264. Epub 2019 Nov 4.

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

Introduction: Tolosa-Hunt syndrome (THS), a condition characterized by painful ophthalmoplegia and accompanied by cranial nerve palsies, remains as a diagnosis of exclusion. Nevertheless, the 3rd Edition of the International Classification of Headache Disorders (ICHD) has refined its diagnostic criteria to require the demonstration of granulomatous inflammation on magnetic resonance imaging or biopsy. We sought to assess the effectiveness of the new criteria in arriving at accurate diagnoses. Read More

J Neurosci Rural Pract 2019 Jul 9;10(3):504-505. Epub 2019 Oct 9.

Department of Ophthalmology, Kasturba Medical College, Mangalore; Manipal University of Higher Education (MAHE), Manipal, Karnataka, India.

Rev Neurol 2019 Sep;69(6):249-254

Hospital Universitario 12 de Octubre, Madrid, Espana.

Introduction: The cavernous sinus is a structure in the base of the skull that houses several nerve and vascular structures. Its compromise leads to cavernous sinus syndrome, which is a combination of oculomotor disorders and others affecting the first two trigeminal branches, often accompanied by pain or proptosis. Infiltration due to Burkitt's lymphoma is a rare cause of this syndrome. Read More

Reumatol Clin 2019 Sep 2. Epub 2019 Sep 2.

Servicio de Medicina Interna, Hospital Universitario Central de Asturias, Oviedo, Asturias, España.

J Laryngol Otol 2019 Jun 4;133(6):501-507. Epub 2019 Jul 4.

Department of Otolaryngology,Charing Cross Hospital, Imperial College Healthcare NHS Trust,London,UK.

Objective: Prompted by a recurring skull base multidisciplinary team debate on the necessity of securing a definitive tissue diagnosis before initiating treatment for lesions of the orbital apex, a review of anterior skull base procedures over an 11-year period was undertaken.

Methods: Data collected prospectively on cases from 2006 to 2017 were analysed. Presenting symptoms, imaging and histology findings, outcomes, complications, and impact on treatment were evaluated. Read More

Somatosens Mot Res 2019 06 30;36(2):102-108. Epub 2019 Apr 30.

a Department of Neurology , The Affiliated Hospital of Xuzhou Medical University , Xuzhou , China.

To strengthen the understanding, increase the early diagnostic rate, and improve the outcome of unilateral oculomotor nerve palsy through the analysis of the 121 patients suffering from this disease in our hospital. A retrospective study was performed on the 121 patients with unilateral oculomotor nerve palsy diagnosed at the Affiliated Hospital of Xuzhou Medical University from October 2014 to October 2015. The clinical data, such as gender, age, aetiology, clinical features, laboratory tests, and six months follow up reports were analyzed. Read More

Eur J Ophthalmol 2020 Mar 25;30(2):NP41-NP46. Epub 2019 Apr 25.

Ruiz Department of Ophthalmology and Visual Science, McGovern Medical School at The University of Texas Health Science Center at Houston (UTHealth), Houston, TX, USA.

Purpose: To describe a rare case of Burkitt lymphoma metastatic to the cavernous sinus that was initially diagnosed as Tolosa-Hunt syndrome.

Case Description: A 31-year-old confirmed and treatment-compliant HIV-positive Hispanic male acutely developed diplopia, ptosis, numbness in the V1 distribution, and complete external ophthalmoplegia in the right eye over 1 week. Imaging showed a mass-like lesion within the right cavernous sinus without other intracranial abnormalities. Read More

J Investig Med High Impact Case Rep 2019 Jan-Dec;7:2324709619838309

1 Duke University School of Medicine, Durham, NC, USA.

Tolosa-Hunt syndrome is characterized by a painful ophthalmoplegia secondary to a granulomatous inflammation in or adjacent to the cavernous sinus. Magnetic resonance imaging will show enhancement of the cavernous sinus and/or the orbital apex. Although this syndrome is extremely rare in children, it should be a diagnostic consideration in patients presenting with painful ophthalmoplegia with variable involvement of cranial nerves II to VI. Read More

Pediatr Neurol 2019 10 20;99:60-63. 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

Med J Aust 2018 09;209(7):293-294

Mater Health Services, Brisbane, QLD.

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

JA Clin Rep 2018 Jul 31;4(1):58. Epub 2018 Jul 31.

Department of Ophthalmology, Hachinohe Heiwa Hospital, 4-6 Minatotakadai 2-chome, Hachinohe, 031-8545, Japan.

Background: Corticosteroid therapy is useful for the resolution of pain and paresis in Tolosa-Hunt syndrome; however, there is no definitive protocol for appropriate dosing, route of administration, or duration of therapy. Steroid psychosis is an adverse reaction to corticosteroid therapy; in severe cases, it can develop into psychiatric disorders such as delirium, depression, and mania. In this case study, we report a patient with Tolosa-Hunt syndrome who developed delirium while receiving corticosteroid 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