1/132. The criteria of the International headache Society for tolosa-hunt syndrome need to be revised.In 1988 the International headache Society defined the diagnostic criteria of tolosa-hunt syndrome (THS) to include episode(s) of unilateral orbital pain for an average of 8 weeks if untreated, with associated paresis of one or more of the third, fourth, and sixth cranial nerves. Cranial nerve paresis may coincide with the onset of pain or follow it within a period of up to 2 weeks, and the pain must be relieved within 72 h after the initiation of corticosteroid therapy. Other causative lesions must be excluded by neuroimaging. On the basis of the history and neuroradiological findings of six patients we show the pitfalls in diagnosing THS with these criteria. We propose a revision of the criteria: Other causative lesions must be excluded by neuroimaging, especially of the region of the cavernous sinus and the orbita, and by blood and CSF examinations. Since imaging techniques have dramatically improved, it is now possible to visualize the inflammatory tissue in THS. Positive magnetic resonance imaging or computed tomography findings compatible with inflammatory tissue neither exclude nor confirm THS and remain suspect until a malignant tumor or inflammation other than THS is excluded. Clinical and radiological follow-up examinations must be performed for at least 2 years, even in patients with negative findings on magnetic resonance imaging at onset.- - - - - - - - - - ranking = 1keywords = episode (Clic here for more details about this article) |
2/132. Recurrent miller fisher syndrome: clinical and laboratory features and hla antigens.In rare cases, miller fisher syndrome (MFS) has been known to recur. However, clinical features of recurrent MFS have not been well analyzed, and the precipitating factors relating to recurrence remain unknown. From 1981 to 1996, we examined four patients with recurrent MFS among 28 Japanese MFS patients. In the four patients, the recurrent episodes occurred after long asymptomatic intervals, ranging from 2.5 to 12.5 years. The clinical and laboratory features of recurrent episodes were similar either to those of the initial episodes or to those of the 24 non-recurrent patients. Of the two patients tested for serum IgG anti-GQ1b antibody, both were positive. Serological HLA typing showed that all recurrent patients were both HLA-Cw3 and -DR2 positive. However, out of 13 non-recurrent patients examined, six had HLA-Cw3, and four had HLA-DR2. The frequency of HLA-DR2 among the recurrent patients was significantly higher than among healthy controls (corrected P = 0.038), and was also higher than among the non-recurrent patients but not significantly. These findings suggest that recurrent MFS is clinically the same as typical MFS and that HLA-DR2 is possibly associated with recurrence.- - - - - - - - - - ranking = 3keywords = episode (Clic here for more details about this article) |
3/132. Mitochondrial maculopathy: geographic atrophy of the macula in the MELAS associated A to G 3243 mitochondrial dna point mutation.PURPOSE: To report ocular findings in the mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (melas syndrome) in a family with the A to G 3243 mitochondrial (mt) dna point mutation. methods: case reports. Ocular findings are described from four family members with the MELAS associated A to G 3243 mt dna point mutation. RESULTS: Findings included ophthalmoplegia, neurosensory deafness, reduction of photopic and scotopic electroretinogram b-wave amplitudes, and myopathy, as well as macular retinal pigment epithelial atrophy. No family members had nyctalopia, attenuation of retinal blood vessels, or retinal bone spicule pigmentation. CONCLUSION: The finding of slowly progressive macular retinal pigment epithelial atrophy expands the reported phenotypic diversity of patients with A3243G mt dna mutations.- - - - - - - - - - ranking = 563.11518656595keywords = myopathy, lactic acidosis, acidosis, episode (Clic here for more details about this article) |
4/132. Resolution of MRI abnormalities of the oculomotor nerve in childhood ophthalmoplegic migraine.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.- - - - - - - - - - ranking = 1keywords = episode (Clic here for more details about this article) |
5/132. Recurrent steroid-responsive trismus and painful ophthalmoplegia.A 63-year-old woman experienced two episodes of trismus and painful ophthalmoplegia at an interval of six years. She suffered left visual loss, and enhanced CT scan and MR imaging revealed heterogeneous enlargement of the left extraocular muscles extending to the orbital apex. In addition, the left pterygopalatine fossa was filled with a mass isointense with muscle without evidence of surrounding tissue invasion; 67Ga scintigraphy showed high uptake in this lesion. Steroid administration dramatically resolved the trismus, and the mass in the orbit and extraorbit vanished completely. orbital pseudotumor is characterized by self-limited, relapsing, steroid-responsive painful ophthalmoplegia, and this case could be a variant of this entity with inflammation extending into the extraorbital area.- - - - - - - - - - ranking = 1keywords = episode (Clic here for more details about this article) |
6/132. Recurrent isolated ptosis in presumed ophthalmoplegic migraine of childhood.PURPOSE: To report a rare case of isolated, recurrent unilateral eyelid ptosis as the sole manifestation of ophthalmoplegic migraine in a healthy young girl. DESIGN: Single observational case report with review of the literature. TESTING: Serologic evaluation, electromyographic, nerve conduction and Tensilon testing, magnetic resonance and computerized tomographic imaging, and magnetic resonance angiography were performed. MAIN OUTCOME MEASURES: Four-year descriptive history of recurrent eyelid ptosis. RESULTS: Between 1994 and 1998, seven discrete episodes of right upper eyelid ptosis without ophthalmoplegia or pupil abnormality occurred. On each occasion, spontaneous resolution occurred over a period of 6 to 10 weeks. Extensive clinical and laboratory evaluation, including testing for myasthenia gravis and magnetic resonance cerebral angiography, was unremarkable. CONCLUSIONS: Recurrent isolated ptosis is a rare manifestation of presumed ophthalmoplegic migraine in childhood. As far as we are aware, this has not been previously reported.- - - - - - - - - - ranking = 1keywords = episode (Clic here for more details about this article) |
7/132. Painful ophthalmoplegia with reversible carotid stenosis in a child.Painful ophthalmoplegia in childhood has different causes. One is tolosa-hunt syndrome, in which a first episode may be difficult to diagnose because of its clinical similarity to ophthalmoplegic migraine. A 10-year-old male with painful ophthalmoplegia and a cavernous sinus inflammation associated with an intracavernous carotid stenosis demonstrated by magnetic resonance imaging and angiography is reported. These findings resolved in follow-up imaging. This report suggests that in the presence of painful ophthalmoplegia, magnetic resonance imaging detection of cavernous sinus inflammation can facilitate the diagnosis of tolosa-hunt syndrome when other causes are excluded.- - - - - - - - - - ranking = 1keywords = episode (Clic here for more details about this article) |
8/132. Recurrent idiopathic familial facial nerve palsy and ophthalmoplegia.INTRODUCTION: Idiopathic facial nerve palsy is a common neurologic condition but episodes of ophthalmoplegia in these patients are uncommon. MATERIALS AND methods: Retrospective case series. RESULTS: Three patients with facial nerve palsy with unusual features, including ophthalmoplegia, are described. CONCLUSION: Recurrent episodes, familial tendency, and associated ophthalmoplegia are uncommon in patients with idiopathic facial nerve palsy.- - - - - - - - - - ranking = 2keywords = episode (Clic here for more details about this article) |
9/132. Painful ophthalmoplegia: report of a case with cerebral involvement and psychiatric complications.The diagnosis of tolosa-hunt syndrome should be suspected in the presence of recurrent "painful ophthalmoplegia." The most useful tests are the rapid (within 48 hours) response to steroids and positive findings on orbital venography. It should be emphasized that Tolosa-Hunt's syndrome may not be a "pure syndrome." Perhaps it is only an occasional presentation of another rather poorly understood syndrome, that of "recurrent cranial neuropathies." The present patient had at least three episodes of painful ophthalmoplegia prior to this hospitalization. During the last hospitalization, he presented with painful ophthalmoplegia, showed a rapid response to steroids, had narrowing of the carotid artery on arteriogram and an abnormal orbital venogram. However, during his hospitalization he developed involvement of cranial nerves II, III, V, VI and VII, papilledema, pyramidal tract signs and severe psychiatric disturbances, all of which remitted. This, coupled with the abnormal pneumoencephalogram and electroencephalogram and organicity on psychological testing, suggests cerebral involvement in our case.- - - - - - - - - - ranking = 1keywords = episode (Clic here for more details about this article) |
10/132. Progressive external ophthalmoplegia associated with retinal pigment epitheliopathy.The diagnosis of ocular myopathy associated with a primary retinal pigment epitheliopathy in a 20-year-old man was based on the integrity of the retinal functions, despite progressive worsening of muscular activity in the systems affected by the disease, and on the results of retinal fluorescein angiography. Although the changes in the pigment epithelium were not prominent ophthalmoscopically, they were clearly visible angiograpically. Atypical forms of retinal pigment dystrophy occurring during the course of ocular myopathy appear to be clinical expressions of a unique genetic defect confined to the pigment epithelial layer. We assume that the gene is capable of inducing a pleiotropic effect.- - - - - - - - - - ranking = 535.05068614965keywords = myopathy (Clic here for more details about this article) |
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