1/841. Absent pituitary gland and hypoplasia of the cerebellar vermis associated with partial ophthalmoplegia and postaxial polydactyly: a variant of orofaciodigital syndrome VI or a new syndrome?We report two sibs with features overlapping those of orofaciodigital syndrome type VI (Varadi syndrome). Both presented at birth with oculomotor abnormalities, dysmorphic facial features, and dysgenesis of the cerebellar vermis. There were minimal oral manifestations (high arched palate) in both of them and one had postaxial polydactyly of both hands and one foot. In addition, there was evidence of aplasia of the pituitary gland on MRI scan in both of them with evidence of hypopituitarism. Both responded well to hormone replacement therapy with improvement in their linear growth and mental ability. These cases may represent a new autosomal recessive midline defect syndrome with features overlapping OFDS VI. Alternatively the features in these children could represent variability within OFDS VI.- - - - - - - - - - ranking = 1keywords = ophthalmoplegia (Clic here for more details about this article) |
2/841. Internuclear ophthalmoplegia following minor head injury: a case report.Internuclear ophthalmoplegia (INO) is a common sign of multiple sclerosis in young patients and of vascular diseases in older people. Traumatic bilateral internuclear ophthalmoplegia following severe head injuries may occur. We present the unusual case of a young patient suffered from bilateral INO as an isolated finding after a minor head injury, without other signs of brain stem or cortical injury. The ophthalmoplegia has persisted for 22 months.- - - - - - - - - - ranking = 1.75keywords = ophthalmoplegia (Clic here for more details about this article) |
3/841. Tonic ocular tilt reaction simulating a superior oblique palsy: diagnostic confusion with the 3-step test.BACKGROUND: The tonic ocular tilt reaction (OTR) consists of vertical divergence of the eyes (skew deviation), bilateral conjugate ocular torsion, and paradoxical head tilt. The head and superior pole of both eyes are rotated toward the hypotropic eye. OBJECTIVE: To describe ocular motility and torsion findings in 5 patients with OTRs that mimicked superior oblique palsies (SOPs). RESULTS: In 5 patients, results of the 3-step test suggested an SOP (bilateral in 1 patient); however, no patient had the expected excyclotorsion of the hypertropic eye. Two patients had conjugate ocular torsion (intorsion of the hypertropic eye and extorsion of the hypotropic eye), and 2 patients had only intorsion of the hypertropic eye. All had other neurologic features consistent with more widespread brainstem disease. CONCLUSIONS: Vertical ocular deviations that 3-step to an SOP are not always caused by fourth nerve weakness. When a patient with an apparent fourth nerve palsy has ocular torsion that is inconsistent with an SOP, OTR should be suspected, especially if vestibular system or posterior fossa dysfunction coexists. The rules for the 3-step test for an SOP may be fulfilled by damaging the otolithic projections corresponding to projections of the contralateral anterior semicircular canal. Because results of the Bielschowsky head tilt test may be positive in patients with the OTR, the feature distinguishing OTR from an SOP is the direction of torsion. We advocate use of a fourth step-evaluation of ocular torsion-in addition to the standard 3 steps.- - - - - - - - - - ranking = 0.00078752535417137keywords = ocular (Clic here for more details about this article) |
4/841. Congenital myasthenia gravis: clinical and HLA studies in two brothers.Two brothers with congenital myasthenia gravis are described. In both, ptosis and ophthalmoplegia responded poorly to oral anticholinesterase therapy and to thymectomy. The brothers had two different HLA haplotypes and neither had the HLA-A1-B8-DW3 haplotypes which are commonly associated with myathenia gravis in adult-onset cases.- - - - - - - - - - ranking = 0.25keywords = ophthalmoplegia (Clic here for more details about this article) |
5/841. Cerebral B-cell lymphoma following treatment for tolosa-hunt syndrome.We herein report a unique case of cerebral lymphoma which occurred after lymphocytic neuritis of cranial nerves causing tolosa-hunt syndrome and demonstrate the histological difference between these two diseases. A 70-year-old woman developed a sensory disturbance in the first and third divisions of the left trigeminal nerve and a left ocular movement disturbance five years before death. Although she was clinically diagnosed to have a schwannoma in the left cavernous sinus, a histologic examination verified a diffuse infiltration of T lymphocytes in the left trigeminal ganglion. Corticosteroid therapy was effective. Thereafter she demonstrated a disturbance of consciousness and dysphasia four years after surgery. A T1-weighted magnetic resonance image (MRI) disclosed high intensity lesions in both the basal ganglia and corpus callosum. She also showed progressive spastic paralysis. At autopsy a diagnosis of primary intracranial B-cell lymphoma was made. Although there was no invasion of the lymphoma cells into the left trigeminal nerves, a mild inflammatory infiltration of T cells still remained.- - - - - - - - - - ranking = 7.1593214015579E-5keywords = ocular (Clic here for more details about this article) |
6/841. Vestibulo-ocular reflex pathways in internuclear ophthalmoplegia.We measured the vestibulo-ocular reflex (VOR) during head impulses in a patient with right-sided internuclear ophthalmoplegia. Head impulses are rapid, passive, high-acceleration, low-amplitude head rotations in the direction of a particular semicircular canal (SCC). Adduction of the right eye was abnormally slow during right lateral SCC head impulses. The VOR during left posterior SCC impulses was severely deficient in both eyes, but the VOR during left anterior SCC impulses was only slightly deficient. We suggest that the vertical vestibulo-ocular pathways in humans are connected in SCC-plane coordinates, not the traditional roll and pitch coordinates, and that anterior SCC signals do not travel exclusively in the medial longitudinal fasciculus.- - - - - - - - - - ranking = 1.2504295592841keywords = ophthalmoplegia, ocular (Clic here for more details about this article) |
7/841. Bilateral ophthalmoplegia and exophthalmos complicating central hemodialysis catheter placement.We describe a 58-year-old woman who presented with bilateral ophthalmoplegia, exophthalmos, and headache and was found to have retrograde internal jugular vein flow secondary to a high-grade obstruction of the ipsilateral brachiocephalic vein from a previous hemodialysis catheter placement. The patient had also a high-flow dialysis graft in the ipsilateral arm. The cranial and extracranial venous system congestion resolved, and the signs disappeared soon after a balloon angioplasty and stent placement at the level of the obstruction.- - - - - - - - - - ranking = 1.25keywords = ophthalmoplegia (Clic here for more details about this article) |
8/841. ophthalmoplegic migraine and periodic migrainous neuralgia, migraine variants with ocular manifestations.The spectrum of migraine has been outlined with particular attention to two entities: ophthalmoplegic migraine and Periodic Migrainous neuralgia. Although quite different in many respects from classical migraine, the relationship of a periodic localized vascular phenomenon giving rise to headache and transient neurologic signs, classify PMN and OPGM as migraine variants. Supportive of this concept, the literature has been reviewed in both entities, and some observations are made on the validity of earlier reports. It is the author's opinion that Raeder's syndrome should be reserved for patients with a lesion localizing in the paratrigeminal area. This does not exclude migraine as an etiologic agent but also recognizes tumors, infections and fractures as being more common.- - - - - - - - - - ranking = 0.00028637285606232keywords = ocular (Clic here for more details about this article) |
9/841. Isolated inferior rectus paresis secondary to a mesencephalic cavernous angioma.PURPOSE: To report a case of recurrent binocular, vertical diplopia presenting as an isolated, unilateral inferior rectus paresis occurring in a patient with a mesencephalic tegmental cavernous angioma. METHOD: Case report. A 30-year-old man with recurrent vertical diplopia underwent neuro-ophthalmic examination, laboratory examination, pharmacologic testing, and magnetic resonance imaging and angiography. RESULTS: magnetic resonance imaging disclosed a lesion in the mesencephalon consistent with a cavernous angioma. magnetic resonance angiography was negative. CONCLUSIONS: Isolated inferior rectus paresis is a rare phenomenon. This unique case involved a patient with recurrent inferior rectus paresis secondary to a mesencephalic cavernous angioma. The disparity between the extent of the lesion and the neuro-ophthalmologic consequences is remarkable.- - - - - - - - - - ranking = 7.1593214015579E-5keywords = ocular (Clic here for more details about this article) |
10/841. meningioma presenting as tolosa-hunt syndrome.A 23-year-old woman was admitted with headache, nausea, vomiting and blurred vision on the left side. Neurological examination showed ptosis with a complete internal and external ophthalmoplegia and a red fullness around the left orbita. Computed tomographic scanning of the brain revealed no abnormalities. As she improved on high doses of steroids a diagnosis of tolosa-hunt syndrome (THS) seemed to be indicated. However, magnetic resonance imaging (MRI) showed a lesion with intermediate signal intensity in the left cavernous sinus. craniotomy was performed when symptoms of THS recurred. Histopathological examination revealed a meningioma with a papillary aspect and some mitoses. This case illustrates that: (1) THS is still a diagnosis by exclusion; (2) MRI and histopathological examination are important if there is any doubt about the diagnosis; and (3) also when there is no doubt, improvement after steroid therapy may be a diagnostic pitfall. Therefore, not only MRI but also orbital phlebography and angiography should seriously be considered.- - - - - - - - - - ranking = 0.25keywords = ophthalmoplegia (Clic here for more details about this article) |
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