| ophthalmoplegic migraine is an uncommon disorder, usually starting in older childhood. Its physiopathology remains obscure and diagnosis is reliant on clinical grounds and exclusion of other disorders. We report four cases of childhood ophthalmoplegic migraine, one of them starting in infancy. association with other types of migraine is common. Two of the three patients studied by magnetic resonance imaging (MRI) showed enhancement and enlargement of the cisternal portion of the oculomotor nerve, which spontaneously resolved after 2 and 4 years, respectively. Persistence of clinical recurrences was associated with long-lasting presence of the MRI finding, and possibly with mild sequelae. These radiological abnormalities suggest a common physiopathological mechanism with other inflammatory diseases, except for a benign evolution which, added to its specific anatomic site, seems to be the only neuroradiological marker, besides normality, in ophthalmoplegic migraine. The very long potential duration of MRI changes and the scarcity of clinical episodes make feasible its incident discovery once the migraine attack has become a remote memory. ( info) |
| Choroidal involvement is a well-recognized manifestation of metastatic disease, particularly from breast and lung primaries. However, breast cancer can involve other ocular structures. The two patients presented illustrate diffuse orbital involvement of the extraocular muscles, simulating tolosa-hunt syndrome. This association has not been reported previously. Both patients responded well to local radiotherapy. ( info) |
| The tolosa-hunt syndrome consists of a painful ophthalmoplegia related to a granulomatous inflammatory process in the cavernous sinus, which may be documented by cerebral magnetic resonance imaging with gadolinium enhancement. Two cases of tolosa-hunt syndrome preceded by facial palsy observed in 1998 at the Department of neurosurgery of the Second University of Naples are presented here. Both patients developed tolosa-hunt syndrome following an ipsilateral facial palsy that resolved in about 15 days with medical treatment. Cerebral magnetic resonance imaging with gadolinium enhancement showed, in both cases, inflammatory tissue in the cavernous sinus. The patients underwent corticosteroid therapy (prednisolone, 80 mg per day, intravenously) with pain regression. In the first case, the patient experienced recurrence of the syndrome that was definitively resolved with further corticosteroid treatment. The rare reports of facial palsy in patients with tolosa-hunt syndrome suggest the inclusion of this disease in the so-called multiple cranial nerve palsy syndrome. It is probable that tolosa-hunt syndrome has an inflammatory pathogenesis. ( info) |
4/27. Really painful double vision. A 67-year-old woman with a history of chronic headache and recent removal of two squamous cell lesions from her forehead presented with left facial pain and diplopia. A diagnosis of tolosa-hunt syndrome was made based on clinical presentation and imaging studies. When the patient did not respond to steroids, further studies were done, including biopsy, which revealed perineural spread of squamous cell carcinoma. ( info) |
5/27. Superior orbital fissure syndrome in a latent type 2 diabetic patient. Although isolated cranial nerve palsies are common in diabetic patients, multiple, simultaneous cranial neuropathies are rare. We describe the second case of a complete superior orbital fissure syndrome including the optic nerve in a middle-aged Papuan man with newly diagnosed diabetes mellitus. The differential diagnosis included septic cavernous sinus thrombosis and Tolosa Hunt syndrome, and management was initially directed at excluding these serious, treatable conditions. ( info) |
6/27. Squamous cell carcinoma with perineural invasion presenting as a Tolosa-Hunt-like syndrome: a potential pitfall in diagnosis. PURPOSE: To describe a case of perineural invasion resulting from squamous cell carcinoma of forehead. methods: Case report. RESULTS: Perineural invasion resulting from squamous cell carcinoma of the periocular skin can present as a Tolosa-Hunt-like syndrome with lack of radiologic findings on magnetic resonance imaging (MRI) in its early stages. CONCLUSION: A high level of suspicion for perineural invasion is required when assessing multiple cranial nerve palsies in patients with a history of cutaneous malignancy, despite negative sequential MRI. Perineural invasion must be ruled out by a biopsy of the involved nerves, whenever possible, before empiric therapy with systemic steroids is contemplated. ( info) |
7/27. Cerebral and oculorhinal manifestations of a limited form of Wegener's granulomatosis with c-ANCA-associated vasculitis. The authors report on cerebral and oculorhinal manifestations in a patient with a cytoplasmic pattern of antineutrophil cytoplasmic autoantibody (c-ANCA)-associated vasculitis. Recurrent tolosa-hunt syndrome, cavernous sinus syndrome, Raeder's paratrigeminal neuralgia, and seizures were the major clinical manifestations. Brain MRI showed localized enhancing lesions initially in the cavernous sinus and later in the convexity pachymeninges. The lesions disappeared following 9 months of oral prednisolone (15 mg/day) and cyclophosphamide (100 mg/day) therapy. The presence of c-ANCA, demonstration of vasculitis, and depositions of immunoglobulin g (IgG) and fibrinogen in the vessel walls of pachymeninges of the patient confirmed an immune-mediated cause of the vasculitis. Cranial pathology without renal and pulmonary involvement suggests a variant of Wegener's granulomatosis, which is called the "limited" form of Wegener's granulomatosis. MRI, Raeder's paratrigeminal neuralgia, localized pachymeningitis. ( info) |
8/27. Tolosa Hunt Syndrome--intractable pain treatment with acupuncture? PURPOSE: The Tolosa Hunt Syndrome (THS) is a painful granular inflammation of the cerebral vessels followed by pain and disorders of the extrabulbar muscles. The therapy consists of corticosteroids and analgetics. There was a 70 year old woman who suffered from painful paresis of the abducent and oculomotor nerves following an infection with borrelia burgdorferi--but without ocular symptoms. The treatment with corticosteroids reduced the palsy but she complained of excessively painful attacks in the region of the first branch of the trigeminal nerve. Opiold analgetic therapy did not bring about any relief. acupuncture is an irritative method with a physical effect on the nervous system: its pain-reducing effect is caused by the activation of transmitters like endorphins in thalamus and brain stem. Knowing this effect, the THS patient, after informed consent, was treated with acupuncture. To measure the extent of pain, a visual analog scale (0: no pain - 10: maximum pain) was used. acupuncture was performed according to the empirical rules of the Traditional Chinese medicine (TCM), during a period of 10 weeks and 12 weeks. There was a significant pain relief after acupuncture from VAS 10 to VAS 5. The effect vanished during the next four months. After a second series of 12 sessions pain reduction was reported from VAS 10 to 4. One year after the last Tolosa Hunt Syndrome - intractable pain pain strength ranged between VAS 4 - 6. Therefore acupuncture seems to be a good additional method for reduction of intractable pain. ( info) |
9/27. Epidermoid cyst of the cavernous sinus: clinical features, pathogenesis and treatment. Epidermoid cyst may rarely arise within the lateral wall of the cavernous sinus (CS) and produce clinical symptoms mimicking the tolosa-hunt syndrome. Pathogenically, it is suggested that some of the subpopulation of cells arising from the neural crest which give rise to skin, may remain entrapped in the meninges around the nerves in the lateral wall in an uncompleted stage of maturation and may develop epidermoid cysts. A case is presented of a 68-year-old man with a 4-week history previously diagnosed as tolosa-hunt syndrome. MRI is the investigation of choice. Surgical resection can result in excellent recovery of preoperative deficits of cranial nerves (CN) III through VI. ( info) |
| Mycotic aneurysms have been associated with many clinical conditions. A tender pulsatile abdominal mass in association with fever, chills, and unrelenting back pain is suggestive of a leaking mycotic aneurysm. However, the extracranial manifestations of tolosa-hunt syndrome (THS) may mimic several of these symptoms. We report the case of a woman who was successfully treated with high-dose steroids for THS. Two months later, she was admitted to another hospital with rigors and unremitting back and abdominal pain. CT-guided aspiration of an L5-S1 paravertebral mass was done. The aspirate and blood cultures grew staphylococcus aureus. Intravenous antibiotics and analgesics were administered with good relief. A month after discharge from that hospital, she was admitted to our hospital with classic signs and symptoms of a leaking mycotic aneurysm. She was treated surgically and has remained asymptomatic for 21 months. tolosa-hunt syndrome associated with mycotic aortic aneurysms has not been previously reported. ( info) |