Cause Of Painful Ophthalmoplegia Research Articles

Disappearing diplopia - An unusual presentation of Tolosa-Hunt syndrome: A case report

Tolosa-Hunt syndrome (THS) is a rare benign treatable cause of painful ophthalmoplegia with frequent VI, III, IV cranial nerve (CN) involvement. Optic and trigeminal nerve involvement is rare but described. The disease is characterized by severe preceding or concomitant headache and remarkable response to steroids. Although labelled benign residual neurology is not uncommon in THS.We present a case of THS in a middle-aged Sri Lankan male who presented initially with vertical diplopia and normal contrast enhanced CT scan, treated as for idiopathic IV nerve palsy. A month henceforth he developed severe episodic headache with autonomic symptoms, right sided ptosis and progressive visual impairment with improved diplopia, normal fundoscopy, inflammatory and infective markers and autoimmune profile. MRI brain showed enhancing lesion of cavernous sinus extending to orbital apex consistent with THS. He showed dramatic improvement with steroids with complete resolution of neurology at the end of 4 weeks.THS should be considered in a patient presenting with disappearing painful diplopia. Headache may mimic primary headache syndromes and may not precede ophthalmoplegia. Imaging may be initially normal. MRI is mandatory for diagnosis as histology is not always feasible. Steroid responsiveness can be diagnostic if more sinister differentials are ruled out.

Neuro-opthalmological aspect of Tolosa-Hunt Syndrome: A Case Report

Tolosa-Hunt Syndrome is an idiopathic granulomatous inflammatory disease of the cavernous sinus, superior orbital fissure, or orbit which is manifested by ocular pain and ophthalmoplegia. Granulomatous inflammation of the dural wall of the cavernous sinus and/or superior orbital fissure is the basis of the pathological process. The disease is clinically manifested by orbital pain, diplopia, exophthalmos, and/or oculomotor abnormalities. The syndrome is also called painful ophthalmoplegia with a key symptom being neuropathic periorbital or retro-orbital pain, and paresthesias along the first branch of the trigeminal nerve. We present a case of this syndrome. The patient was administered hormonal therapy, Medrol at a daily dose of 1 mg/kg body weight/day intravenously for five days, with subsequent transition within a month to oral Medrol. The pain in the eye completely relieved and restoration of normal ocular motility was observed in the presence of hormonal therapy. Tolosa-Hunt syndrome is a clinical diagnosis of exclusion (i.e., it is diagnosed after excluding other causes of painful ophthalmoplegia).

Single internal carotid cavernous sinus aneurysm presented as bilateral painful ophthalmoplegia: a case report

BackgroundIntracranial aneurysms are the most common vascular cause of painful ophthalmoplegia. Symptoms include retro-orbital pain, diplopia, ophthalmoplegia, trigeminal neuropathy, or a combination of these. Most single aneurysms cause ipsilateral, painful ophthalmoplegia. Here, we report the first, to our knowledge, case of bilateral painful ophthalmoplegia possibly caused by an aneurysm of the cavernous segment of the left internal carotid artery.Case presentationA 62-year-old male patient presented with headache and bilateral ptosis. Laboratory tests revealed hypopituitary function. Computerized tomography angiography showed a large aneurysm in the cavernous sinus segment of the left internal carotid artery. Aneurysm embolization was performed in the Nerve Interventional Department. Four months after surgery, the patient's symptoms returned to normal.ConclusionsThis case suggests that patients with bilateral painful ophthalmoplegia should be screened for aneurysms using computed tomography angiography or magnetic resonance angiography immediately.

A case report on recurrent alternating Tolosa-Hunt syndrome due to bacterial sphenoid sinusitis: rediscussing the diagnostic terminology and classification

BackgroundTolosa-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.Case presentationA 34-year-old woman presented with left painful ophthalmoplegia. Magnetic resonance imaging (MRI) revealed abnormal signals in the left cavernous sinus area, and these abnormal signals were suspected to be THS. After steroid treatment, the patient obtained pain relief and had complete recovery of her ophthalmoplegia. However, right painful ophthalmoplegia appeared during the follow-up period. MRI showed obvious inflammatory signals in the right cavernous sinus and right sphenoid sinus. Then nasal sinus puncture and aspiration culture were performed, and the results showed a coagulase-negative staphylococcus infection. After antibiotic treatment with vancomycin, the painful ophthalmoplegia completely resolved, and the neurological examination and MRI returned to normal.ConclusionSome other causes of painful ophthalmoplegia also fulfill the diagnostic criteria for THS in the International Classification of Headache Disorders third edition (ICHD-3) and respond well to steroid therapy. Early diagnosis of THS may be harmful to patients, and clinicians should exercise great caution when dealing with similar cases without a biopsy. Using “cavernous sinus syndrome” instead of “Tolosa-Hunt syndrome” as a diagnostic category may provide a better clinical thinking for etiological diagnosis.

MRI versus CT scan of brain in evaluation of suspected cavernous sinus syndromes - Prospective study of 48 patients in a tertiary centre in India

Background: Cavernous sinus syndrome is a common cause of painful ophthalmoplegia. These patients have diverse etiologies, presentations and outcomes and often pose a diagnostic dilemma as they are not easily amenable for biopsy hence there is immense dependence on neuroimaging for diagnosis.However till date there is no study which has compared the diagnostic utility of CT scan versus MRI scan of brain in suspected CSS.

Painful ophthalmoplegia as an initial presentation of sarcoidosis

There are numerous causes of painful ophthalmoplegia, including microvascular infarcts, aneurysmal compression and Tolosa‐Hunt syndrome. Tolosa‐Hunt syndrome was first described after a series of p...

Tolosa Hunt Syndrome: Increased Severity on Second Attack

Tolosa Hunt syndrome (THS) is a rare cause of painful ophthalmoplegia with an estimated annual incidence of 1 case per million per year. It is caused by a nonspecific inflammatory process, of unknown etiology, involving the cavernous sinus, the superior orbital fissure and/or the orbital apex. It is classically described as an episodic orbital pain associated with paralysis of one or more of the 3rd, 4th and 6th cranial nerves, resolving spontaneously or with the commencement of steroids. I report a female patient who had 2 attacks of THS separated by a period of 10 months. She presented with retro-orbital headache, exophthalmos, and ophthalmoplegia of left eye, on both attacks, and was treated with high dose prednisolone. On first attack she improved within 6 weeks of treatment. On second attack, the patient had a higher Erythrocyte sedimentation rate (ESR) and developed 2 relapses on corticosteroids tapering, requiring a prolonged course of treatment, for 27 weeks, which suggests that the disease activity may get more severe on further attacks of the disease.

Painful ophthalmoplegia due to Tolosa-Hunt syndrome: a case report

Tolosa-Hunt syndrome (THS) is a rare clinical entity, characterized by sudden onset of painful ophthalmoplegia and prompt response to steroid therapy. Generally, it involves the third, fourth, and sixth cranial nerves due to presence of non-specific inflammation at the level of cavernous sinus or superior orbital fissure. In some cases, first or second division of trigeminal nerve may also be involved. Here we present a case report on THS, a rare cause of painful ophthalmoplegia. The patient presented with sudden onset, unilateral headache, drooping of left upper eyelid followed by diplopia. On examination, the patient had complete ophthalmoplegia, along with ophthalalmic division of trigeminal nerve was also involved. Magnetic resonance imaging (MRI) brain showed expansion of left cavernous sinus suggesting THS. Other differentials of THS were ruled out on the basis of careful history, examination, and investigations.

The importance of social history in the work-up of painful ophthalmoplegia

Painful ophthalmoplegia is not one uncommon clinic phenomenon in both ophthalmological and neurological clinics, which can be caused by various pathological conditions and easily be misdiagnosed by some clinical practitioners. Extensive clinical, laboratory and imaging assessment is required to exclude or confirm the diagnosis. Herein, we report an unusual case of painful ophthalmoplegia caused by orbital wall fracture in one young lady. Our case indicates that orbit wall fracture can be a rare cause of painful ophthalmoplegia, which may enrich our etiological diagnosis and avoid some unnecessary mistakes in certain cases of ophthalmoplegia. • Painful ophthalmoplegia is a challenging clinic phenomenon. • Painful ophthalmoplegia can be caused by various pathological conditions. • Orbit wall fracture is a rare cause of painful ophthalmoplegia.

Validation of ICHD-3 beta diagnostic criteria for 13.7 Tolosa-Hunt syndrome: Analysis of 77 cases of painful ophthalmoplegia

Three editions of International Classification of Headache Disorders (ICHD) diagnostic criteria for Tolosa-Hunt syndrome (THS) have been published in 1988, 2004 and 2013, in ICHD-3 beta, there have been considerable changes [corrected]. The validity of these new diagnostic criteria remains to be established. We retrospectively identified 77 patients with non-traumatic painful ophthalmoplegia (PO) admitted between 2003 and 2013. We reviewed patients' age at onset and gender, time courses between onset of pain and development of cranial nerve palsy, the cranial nerves involved, imaging findings, therapeutic efficacy of steroid treatment and recurrence of attacks. THS was the most frequent type of PO (46/77). In THS patients, the third cranial nerve was most commonly involved (76.3%). The median time interval between pain and cranial nerve palsy was two days, although in five patients (10.9%) the interval ranged from 16 to 30 days. Definitely abnormal MRI findings were found in 24 patients (52.2%). It is essential to rule out other causes of PO in diagnosing THS, with MRI playing a crucial role in differential diagnosis. It may be helpful to understand and master the entity of THS for researchers and clinicians to adjust the gradation and ranking of the diagnostic criteria.

Confirmation of Tolosa‐Hunt Syndrome via Endoscopic Endonasal Biopsy of the Orbital Apex

Objectives: 1) Review the criteria for diagnosing Tolosa-Hunt Syndrome (THS), a rare cause of painful ophthalmoplegia due to idiopathic granulomatous inflammation of the orbital apex or cavernous sinus. 2) Review the literature of THS and other surgical techniques used previously in the setting of THS. Methods: We present a literature review of THS, focusing on the surgical approaches to and findings in the cavernous sinus and orbital apex. We report a recent case of unilateral vision loss and painful ophthalmoplegia where endonasal endoscopic biopsy of the orbital apex was performed after initial evaluation failed to yield a diagnosis. We present the pre-operative imaging, intra-operative observations, and pathologic results of this patient with THS. Results: In our review of THS, there are no other documented reports of endonasal endoscopic biopsy of the orbital apex. There are few reports of variable neurosurgical approaches used previously in THS. In our case, endoscopic biopsy yielded pathologic evidence of granulomatous inflammation without evidence of infectious or neoplastic disease. The patient received intravenous steroids, and symptoms improved within 72 hours, confirming THS. Conclusions: THS is an extremely rare cause of painful ophthalmoplegia. Traditionally, THS is a diagnosis of exclusion without tissue confirmation, as biopsies are difficult to perform. This may delay diagnosis of a neoplasm. We present a single case of THS where endonasal endoscopic biopsy of the orbital apex confirmed granulomatous inflammation, suggesting that endoscopic techniques may be used as a safe, minimally invasive approach to the orbital apex in the setting of painful ophthalmoplegia.

Utility of Thallium-201 Scintigraphy in Tolosa-Hunt Syndrome

Tolosa-Hunt syndrome (THS) is a rare disorder, especially in the pediatric population, characterized by unilateral painful ophthalmoplegia with a relapsing-remitting course. Because the diagnosis of THS is based on the exclusion of other causes of painful ophthalmoplegia, attention should be paid to possible alternative diagnoses. Thallium-201 chloride ((201)Tl) scintigraphy has been used to evaluate tissue histology in clinical oncology with a marker, the retention index (RI). A higher value indicates histological malignancy. Although its utility in pediatric THS has not been discussed, we suggest that (201)Tl scintigraphy may be informative as a marker in the diagnosis. We present an 11-year-old boy with THS who was evaluated with (201)Tl scintigraphy before treatment with corticosteroids, when he had headache, photophobia, and diplopia. The RI of (201)Tl indicated that the lesion would be benign. Although his clinical symptoms did not fulfill the THS criteria completely, his eye symptoms disappeared 2 weeks after corticosteroid treatment, which was not within the 72 h as in the diagnostic criteria of THS. He has been symptom-free for more than 2 years with only an initial 4-week corticosteroid therapy. This report not only shows the potential of (201)Tl scintigraphy to contribute to the correct diagnosis of pediatric THS but also suggests the possibility that the diagnosis of THS could be supported uniquely even in a pediatric THS-suspicious patient who did not fulfill the current THS criteria completely. In conclusion, we suggest that (201)Tl scintigraphy may be useful for making the diagnosis of THS, especially in pediatric patients.

Paediatric Tolosa-Hunt syndrome: the need for treatment guidelines and renewed criteria

SIR–Tolosa-Hunt syndrome (THS) is a rare disorder, especially in the paediatric population, characterized by unilateral painful ophthalmoplegia of, typically, a relapsing-remitting course and a substantial response to corticosteroids. The symptoms of THS are not specific and clinical criteria themselves do not guarantee a firm diagnosis. Neuroimaging or biopsy confirming granulomatous inflammation is essential to ad iagnosis of THS. Although diagnostic criteria were established by the International Headache Society in 1988, then revised in 2004 (Table I), 1 THS remains a diagnosis of exclusion of other causes of painful ophthalmoplegia, (e.g. neoplasms, aneurysms, cranial neuropathy [diabetes mellitus], opthalmoplegic migraine, intracranial vasculitis, pseudotumor of the orbit, basal meningitis, or cavernous sinus thrombosis). 2

The Tolosa-Hunt Syndrome in Children: A Case Report

Tolosa-Hunt syndrome (THS) is characterized by unilateral painful ophthalmoplegia with oculomotor paresis, associated with an idiopathic granulomatous inflammation involving the cavernous sinus, with a typical relapsing-remitting course. We report a case of an 8-year-old girl who was admitted because of an ophthalmoplegia with exotropia and ptosis of the left eyelid, accompanied by diplopia and left sovraorbital pain. The clinical data, neuroradiological findings and response to steroid treatment suggested THS, as defined by the 2004 International Classification of Headache Disorders (ICHD)-II criteria. THS must be considered a possible cause of painful ophthalmoplegia in childhood, as well as in adults, and confirmed with a focused neuroradiological investigation. The few paediatric cases described in the literature that meet the 2004 ICHD-II criteria are not sufficient to identify possible differences between the paediatric and the adult forms. Every new paediatric case should therefore be reported in order to gather and compare further information.

Les ophtalmoplégies douloureuses de l'enfant : place du syndrome de Tolosa-Hunt et de la migraine ophtalmoplégique

In children with painful ophthalmoplegia, the diagnosis of Tolosa-Hunt syndrome or of ophthalmoplegic migraine should only be considered when tumoral, infectious, inflammatory or vascular causes have been excluded by appropriate investigations. Both entities are classified as “neuralgia” by the International Headache Society, and seem to share a similar pathogenic mechanism. Both diseases have many clinical similarities with slight differences concerning pain characteristics or ocular associated symptoms. High resolution CT scan or contrast enhanced MRI can be necessary to exclude other causes of painful ophthalmoplegia. They can sometimes objectify an inflammatory process of the cavernous sinus in Tolosa–Hunt syndrome or a reversible enhancement and thickening of the cisternal segment of the oculomotor nerve during an ophthalmoplegic migraine. Pain and ophthalmoplegia quickly resolve with corticosteroids. Such treatment may decrease the risk of recurrence. It is important to follow-up these patients for a 2 years period and to repeat the etiologic assessment. We report here 2 cases of children with painful ophtalmoplegia.

Painful ophthalmoplegia: an unresolved clinical problem

Painful ophthalmoplegia (PO) is an important presenting problem to ophthalmologists and neurologists. The etiological differential diagnosis is extensive, including different syndromes and causes (vascular, neoplastic, infectivous, inflammatory). Current neuroimaging techniques allow visualisation of the area of the suspected pathology. Some rare causes of PO, such as Tolosa Hunt syndrome with negative neuroimaging findings or ophthalmoplegic migraine remain till now of uncertain classification. Correct approach to the patient requires correlation to clinical data and careful monitoring, to avoid diagnostic mistakes, as the "history" of Tolosa-Hunt syndrome has underlined.

Tolosa-Hunt syndrome: one cause of painful ophthalmoplegia.

The author reports 12 cases of Tolosa-Hunt syndrome, a benign steroid-resistant cryptogenic granuloma that presents as painful ophthalmoplegia. This syndrome is differentiated from other causes of painful ophthalmoplegia including tumors, aneurysms, collagen disease, specific infections, mucoceles, and benign granulomas of unknown etiology. These other conditions should be excluded by appropriate tests. Surgical exploration is not necessary if there is a prompt remission on steroid therapy.