tolosa hunt syndrome

Crystal-storing histiocytosis (CSH) is a rare disease associated with the accumulation of histiocytes containing crystalline matter within their cytoplasm. Herein, we present the case of a female patient who was diagnosed with Tolosa-Hunt syndrome at 45 years of age and idiopathic retroperitoneal fibrosis when she was 48 years. She developed portal hypertension (PH), but did not present with cirrhosis; as such, the cause of PH was not identified. Her PH gradually worsened when she was 54 years, and at the age of 60 years, she died from an acute subdural hematoma...

Tolosa Hunt syndrome is a rare disorder characterized by idiopathic chronic granulomatous lesion involving cavernous sinus. The presenting symptoms are severe frontal headache, periorbital pain, ptosis, and diplopia. Usually, patients with headache and ptosis primarily visit neurologists or ophthalmologists. Only when imaging reveals any intracranial lesion involving paranasal sinuses, these patients get referred to otorhinolaryngologists. We would like to describe here the challenges we faced as otorhinolaryngologist, in diagnosis and management of a case of painful ophthalmoplegia as Tolosa Hunt Syndrome...

Tolosa-Hunt syndrome (THS) is a painful ophthalmoplegia due to non-specific granulomatous inflammation in the cavernous sinus region. It is diagnosed by the International Classification of Headache Disorders (ICHD)-3 criteria. We report the case of a young lady who presented with a right-sided headache for 2 weeks, followed by right-sided diplopia for 4 days. Clinical examination revealed right trochlear nerve palsy. Magnetic resonance imaging (MRI) of her brain showed abnormal thickening and postcontrast enhancement of the right orbital apex and superior orbital fissure, suggesting THS...

BACKGROUND: Tolosa-Hunt syndrome (THS) is characterized by painful ophthalmoplegia caused by idiopathic granulomatous inflammation involving the cavernous sinus region. Patients respond well to steroid therapy. THS is included in the differential diagnosis of cavernous sinus syndrome, so it is important to fully exclude other lesions in this area before treatment, otherwise steroid treatment may lead to fatal outcomes. Here we describe a patient who initially presented with symptoms that simulated THS symptoms and developed recurrent alternating painful ophthalmoplegia during follow-up, and the patient was finally diagnosed with cavernous sinusitis caused by bacterial sphenoid sinusitis...

Tolosa-Hunt syndrome (THS) remains a challenging diagnosis for many neurologists. Often believed to be a rare presentation, the classical presentation is known to involve cranial nerves and tissues surrounding the cavernous sinus. Traditionally, a diagnosis of THS is considered when all secondary conditions have been ruled out. Yet, newer findings have elaborated a complex pathogenetic process with some overlap from the IgG4 spectrum of disorders, with which it shares many phenotypic similarities. In this narrative review, we present an updated picture of the condition focusing on the latest developments in the pathogenesis, diagnosis, and clinical management of these two conditions and use illustrative examples to highlight the salient features of this rare presentation...

BACKGROUND: Hypertrophic pachymeningitis (HPM) is a unique disorder characterized by thickening and fibrosis of the dura mater. Clinically it presents with headache, cranial nerve palsies, and other focal neurological deficits. Two forms exist, one is primary, where all other causes have been excluded and the other is secondary where an identifiable cause exists. It is important to recognize these secondary causes as treatment depends on the etiology. OBJECTIVE: To elucidate the various characteristics of HPM...

Tolosa-Hunt syndrome is a rare cause of painful ophthalmoplegia, most commonly presenting with retro-orbital pain and eye motor nerve palsy, most often affecting a single eye. The condition is characterised by an idiopathic process causing granulomatous inflammation to the cavernous sinus affecting, one or multiple cranial nerves that pass therein. The mechanism underlying the inflammation is not well understood, but patients have been observed to respond to steroids during a flare. We present this as a unique case where a multidisciplinary team diagnosed Tolosa-Hunt syndrome despite non-specific MRI findings in the cavernous sinus 1 day following an initially normal MRI...

No abstract text is available yet for this article.

Tolosa-Hunt syndrome (THS) is an idiopathic condition included in the differential diagnosis of painful ophthalmoplegia. Although this was once a common diagnosis, the increasing availability of tests reveals an alternative etiology in many cases. Exclusion of treatable disorders is important, because the prognosis may otherwise be poor. We here describe a patient who presented with painful ophthalmoplegia with an infiltrating lesion in the cavernous sinus. Initially suspected of THS, he had a fatal evolution, and postmortem evaluation revealed cervicocephalic actinomycosis...

The presence of neurological symptoms within the clinical range of COVID-19 disease infection has increased. This paper presents the situation of a 45-year-old man having the medical antecedent diabetes mellitus, who presented to the emergency department with fever, headache, and respiratory symptoms, nine days following vaccination with the Ad26.COV2-S COVID-19 vaccine. The patient tested positive for SARS-CoV-2 based on nasal polymerase chain reaction (RT-PCR). Two weeks after the presentation, he developed Tolosa-Hunt Syndrome, an autoimmune phenomenon, with painful left ophthalmoplegia...

Tolosa-Hunt syndrome (THS) is an extremely rare disorder characterized by painful unilateral ophthalmoplegia triggered by idiopathic inflammation of the cavernous sinus affecting the third, fourth, and/or sixth cranial nerve. Corticosteroid therapy effectively improves THS symptoms; thus, early clinical suspicion and diagnosis are essential. We report the case of a 37-year-old patient who presented with left-sided eye pain and double vision for four days. Physical examination was significant for oculomotor, trochlear and abducent nerves palsies on the left eye with slow light reflex...

We are presenting a rare case of an acute complete external ophthalmoplegia with positive polymerase chain reaction (PCR) for SARS-CoV-2. Our case is the first case that depicts development of Tolosa-Hunt Syndrome (THS) following infection with COVID-19, with a challenging diagnosis and spontaneous improvement. A 65-year-old diabetic female presented with a complete external ophthalmopegia in the left eye and a severe left-sided headache. The PCR result for SARS-CoV-2 was positive. Brain and orbital computed tomography scan and magnetic resonance imaging were both unremarkable...

BACKGROUND: A worldwide mass vaccination campaign against the coronavirus disease 2019 (COVID-19) pandemic is currently underway. Although the safety data of the clinical trials did not report specific concerns regarding neuro-ophthalmological adverse events, they involved a limited number of individuals and were conducted over a relatively short time. The aim of the current review is to summarize the available postmarketing data regarding the occurrence of neuro-ophthalmological and other ocular complications of the COVID-19 vaccines...

BACKGROUND: The diagnostic criteria for Tolosa-Hunt syndrome (THS) were updated in 2013 in the 3rd Edition of the International Classification of Headache Disorders. It is now possible to diagnose THS based on the presence of granulomatous inflammation demonstrated on magnetic resonance imaging (MRI) without confirmation by biopsy. No previous study has reported the use of arterial spin labeling (ASL) perfusion MRI for diagnosing THS. Here, we report a case of THS in which ASL was used in the initial identification and to monitor therapeutic response following steroid therapy...

Tolosa-Hunt syndrome (THS) is a rare neuro-immunological disorder characterized by severe periorbital headaches and ophthalmoplegia. In some patients, THS may occur in parallel with other autoimmune disorders. The underlying etiology of THS remains to be definitively established. However, inflammation of the cavernous sinus or orbital apex represents a hallmark feature; magnetic resonance imaging, therefore, plays a key role in establishing a diagnosis. We describe a patient who presented with concomitant THS and granulomatosis with polyangiitis...

PURPOSE: to describe a rare case of orbital apex syndrome caused by aspergillosis with acute presentation. CASE DESCRIPTION: retrospective case report of a 70-year-old man who developed unilateral ophthalmoplegia overnight. He was initially given the diagnosis of suspect Tolosa-Hunt syndrome, but biopsy of the involved tissue showed aspergillosis. CONCLUSION: orbital apex syndrome caused by fungal disease is a life-threatening condition that should be promptly diagnosed and treated...

Painful ophthalmoplegia syndrome is characterised by the presence of peri-orbital or peri-cranial pain that is accompanied by paralysis of the oculomotor nerves. The differential diagnosis is broad, and requires a rigorous study. Three clinical cases of patients with painful ophthalmoplegia syndrome are described, in which multiple extension studies were required to obtain an aetiological diagnosis. Painful ophthalmoplegia syndrome is a complex disorder that has multiple causes, including infections, inflammatory, and tumour processes...

An 11-year-old boy presented with 2 weeks of intermittent headache, right orbital pain, and constant diplopia. Brain MRI showed dural thickening and enhancement of the right lateral cavernous sinus, right orbital apex, and tentorium. Initial cerebral spinal fluid analysis showed only mild pleocytosis, and serum diagnostics were unrevealing. The working diagnosis was Tolosa-Hunt syndrome. His pain and sixth nerve palsy resolved with corticosteroids. Five months after initial presentation, he developed new numbness of the right cheek, complete right ophthalmoplegia, and weakness and numbness of his right hand and leg, all of which were responsive to steroids...

Recurrent painful ophthalmoplegic neuropathy (RPON), previously called ophthalmoplegic migraine, is a rare condition characterised by recurrent episodes of headache and ophthalmoplegia. We report a case of 11-year-old girl with recurrent painful ophthalmoplegia due to isolated right oculomotor nerve involvement. MR brain imaging showed enhancing lesion of cisternal segment of right oculomotor nerve. A possibility of Tolosa Hunt syndrome was considered and she was treated with glucocorticoids, followed by azathioprine due to recurrence...

Here we discuss a presentation of Tolosa-Hunt syndrome (THS) in a 44-year-old female with new right-eye ptosis, ophthalmoplegia and headache. Four days prior, she had almost identical ptosis and ophthalmoplegia in her left eye, which resolved. Cavernous sinus inflammation and symptom improvement with glucocorticoid treatment indicated THS with bilateral eye involvement, a presentation which may be undervalued by the current THS classification.