[Tolosa-Hunt Syndrome; clinical and brain MRI features and treatment]. / Tolosa-Hunt Sendromu; klinigi, kranyal MR özellikleri ve tedavisi.

Tolosa-Hunt Syndrome (THS) is an idiopathic condition characterized by periorbital and hemicranial pain, ipsilateral oculomotor and sixth cranial nerve involvement, good response to steroids, granulomatous inflammation of the cavernous sinus or the top of the orbita. A 25 year- old- male patient presented with severe burning burn pain near the left eye with double vision. The left eye exhibited semiptosis, limited outward gaze, and difficulty in the downward outward gaze. No history of trauma or drug use was reported. Cranial Magnetic Resonance Imaging (MRI) showed thickening cavernous sinus walls to the left, while contrasted MRI sections suggested inflammation as indicated by intense staining. Cerebral arterial and venous angiography were normal. Parenteral treatment with 1mg/kg methylprednisolon commenced. The pain passed on the third day of treatment. Ophthalmoplegia began improving in the third week. The clinical symptoms were completely over after the third month of the clinic. The pain did not recur after corticotheraphy started. The cranial MRI of the patient was reviewed in a comparative perspective in the first and third months of the clinic. Inflammation was observed to get better. THS should always be considered in painful ophthalmoplegia cases. Cranial MRI towards the cavernous sinus, in particular, would suffice for diagnosis, provided that the vertex of orbita is not affected.

Bilateral facial nerve involvement in a patient with Tolosa-Hunt syndrome.

Tolosa-Hunt syndrome is characterized by unilateral retro-orbital headaches and cranial nerve palsies, usually involving cranial nerves III-VI. It is rare for other cranial nerves to be involved, although this has previously been reported. We report a 19-year-old woman presenting with typical features of Tolosa-Hunt syndrome but ultimately developing bilateral facial nerve palsies and enhancement of both facial nerves on magnetic resonance imaging. The patient presented with unilateral retro-orbital headaches and palsies of cranial nerves III-VI. She was diagnosed with Tolosa-Hunt syndrome but was non-compliant with her corticosteroid treatment due to side effects. She returned with progressive left followed by right facial nerve palsy. Her corresponding follow-up magnetic resonance imaging scans showed sequential enhancement of the left and right facial nerves. She ultimately had clinical improvement with IV methylprednisolone. To our knowledge, Tolosa-Hunt syndrome associated with bilateral facial nerve palsy and corroborative facial nerve enhancement on magnetic resonance imaging has not previously been described. Moreover, our patient's clinical course is instructive, as it demonstrates that this atypical presentation of Tolosa-Hunt syndrome can indeed respond to corticosteroid treatment and should not be mistaken for other entities such as Bell's palsy.

The aetiologies of unilateral oculomotor nerve palsy: a clinical analysis on 121 patients.

Background and purpose: 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. Methods: 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. Results: The main causes identified in the 121 patients with unilateral oculomotor nerve palsy were intracranial aneurysm (29.8%), diabetic peripheral neuropathy (26.5%), painful ophthalmoplegia (9.9%), and other causes (33.9%). The results from the six month follow up showed that in all the patients, 53.7% were fully recovered, 38.0% improved, and 8.3% had no significant change in symptoms. The results also indicated that the patients with diabetic peripheral neuropathy had the best outcome with 71.9% full recovery rate, which was significantly higher than that in the patients with intracranial aneurysm (36.1%, p < .05), and idiopathic causes (44.5%, p < .05). Conclusions: Our data indicates that intracranial aneurysm is the leading cause of unilateral oculomotor nerve palsy, and that diabetic peripheral neuropathy has better outcome. Understanding the common causes and clinical features of unilateral oculomotor nerve paralysis is helpful for its early diagnosis and treatment.

Bilateral Tolosa-Hunt syndrome mimicking pituitary adenoma.

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. superior orbital fissures and cavernous sinuses, but also in the pituitary; these imitated gland's macroadenoma in imaging studies.

Steroid-responsive painful ophthalmoplegia: Tolosa-Hunt syndrome, Eales disease, or both?

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. Case report We report the case of a 32-year-old woman with a subacute left ophthalmoplegia and evidence of a gadolinium-enhanced lesion suggesting an inflammatory granuloma that resolved within 48 hours after treatment with steroids. A diagnosis of THS was considered at this time. On a follow-up ophthalmological examination, a diagnosis of Eales disease with involvement of the left eye was made. The patient was treated successfully. Conclusion Eales disease could be a cause of painful ophthalmoplegia and may mimic THS. Long-term follow-up of patients diagnosed with THS may be necessary to exclude other diagnoses.

Cryopyrin-associated periodic fever syndrome manifesting as Tolosa-Hunt syndrome.

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. Progression of the disease over the following years, accompanied by increasing signs of inflammation on cerebral magnetic resonance imaging and cerebrospinal fluid pleocytosis, led to further diagnostic tests. Genetic analyses revealed a heterozygote low-penetrance mutation (Q703K) of the cryopyrin/NLRP3 gene compatible with a cryopyrin-associated periodic fever syndrome. DISCUSSION: This case report demonstrates that THS can be a central nervous system manifestation of cryopyrin-associated periodic fever syndrome, which therefore represents a differential diagnosis of THS, even in elderly patients.

Painful ophthalmoplegia: a case report and literature review.

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. A 45-year-old female reported episodes of reversible left eye pain and diplopia. Examination showed periorbital oedema and left palpebral semiptosis, paresis of the partial left third nerve palsy with normal pupillary reactions, fourth and sixth left cranial nerves, and hypoesthesia over the first and second division of the left trigeminal nerve. Blood analysis, postcontrast cranial and orbital magnetic resonance (MR) imaging, cranial MR angiography and CSF analysis demonstrated no abnormalities. The clinical diagnosis satisfies the criteria for THS. After steroid therapy her symptoms and clinical signs dramatically reverses. Painful ophthalmoplegia with inflammatory conditions such as THS is highly responsive to corticosteroids but should be diagnoses of exclusion. The THS diagnosis should be used rarely and with great caution.

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.

A rare case of Tolosa-Hunt syndrome imaged with FDG PET/CT and MRI.

We report on the imaging findings of Tolosa-Hunt syndrome in a 59-year-old patient. Clinical findings included periorbital pain, ptosis, disordered eye movements, and blurred vision. Treatment with intravenous administration of steroid resolved all symptoms. Currently, magnetic resonance 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.

Tolosa-Hunt syndrome with reversible dissection aneurysm.

A 49-year-old female presented with recurrent intolerable right retro-orbital pain, ptosis and diplopia for 4 months. Neurological evaluation revealed right oculomotor nerve and abducent nerve injuries. Neuroimaging found enlarged right cavernous sinus, right intracavernous carotid dissection aneurysm and a stenosis proximal to it. Tolosa-Hunt syndrome (THS) was suggested and treated with steroid. The clinical symptoms were alleviated after the treatment. After 3 months of follow-up cerebral angiography showed the lesions of the right intracavernous carotid stenosis and the dissection almost disappeared. Therefore, we proposed that the dissection and the stenosis are directly induced by the inflammation of THS.