Search results for "Oculomotor Muscles"
showing 10 items of 15 documents
Eye Movement Involvement in Parry-Romberg Syndrome: A Clinicopathologic Case Report
2008
We report the case of a 38-year-old woman who developed a progressive bilateral disease in which the eye motility disorder-diplopia-is the outstanding feature over a period of 12 years. The muscle biopsy of the medial rectus muscle did not show any trace of striated muscle. To the best of our knowledge, this is the first pathological report in an affected extraocular muscle of a patient with Parry-Romberg syndrome (PRS). Previous rare reports of diplopia in PRS have been attributed to enophthalmos, progressive atrophy of the orbit, ocular motor nerve dysfunction, or mechanical restrictions.
Eyes open and eyes closed as rest conditions: impact on brain activation patterns
2003
The patterns of associated brain activations during eyes-open and eyes-closed states in complete darkness considerably differ in fMRI. An "interoceptive" state with the eyes closed is characterized by visual cortex activation, while an "exteroceptive" state with the eyes open is characterized by ocular motor system activity. The impact of the chosen rest condition (eyes open or eyes closed in complete darkness) on the pattern of brain activations during visual stimulation was evaluated in 14 healthy volunteers. During fixation or dim light room illumination, the activation of the visual cortex was larger with the eyes-open rest condition than with the eyes-closed rest condition; however, ac…
Oculopharyngeal muscular dystrophy in a northern German family linked to chromosome 14q, and presenting carnitine deficiency
1997
We report the evaluation of oculopharyngeal muscular dystrophy (OPMD) in a large northern German family, which can be traced back six generations and is unrelated to French-Canadian families. The symptoms in this family start at about 50 years of age and include dysphagia, bilateral ptosis, and in some cases a slowly progressive atrophy and weakness of other extraocular, facial or limb girdle muscles. The muscle biopsies showed the pathognomonic ultrastructural finding of characteristic intranuclear filaments. Linkage analysis confirmed that this family is also linked to chromosome 14q markers. Haplotype analysis revealed that a unique haplotype segregates with the disease which is differen…
Role of Octreoscan and Correlation with MR Imaging in Graves' Ophthalmopathy
1995
Since the identification of somatostatin receptors on lymphocytes, orbital infiltration with mononuclear cells in Graves' ophthalmopathy has provided a rationale for receptor imaging with the radiolabeled somatostatin analog Octreotide. In 40 patients with Graves' ophthalmopathy and 10 controls, 110 MBq indium-Octreotide were administered i.v., and scans were performed at 4 and 24 h after injection. An uptake ratio between the orbits and the brain was determined. In 20 ophthalmophathy patients, magnetic resonance imaging (MRI) of the orbits was performed and the T2 relaxation time was measured within the rectus muscles. Compared to controls (4 h Octreotide uptake: median 6.0 counts/pixel/MB…
Relationship of eye muscle antibodies with HLA phenotypes and thyroid-stimulating immunoglobulins in endocrine orbitopathy.
1991
The relationship between endocrine orbitopathy and Graves' hyperthyroidism is still not clairified. The purpose of this study was to elucidate the incidence of eye muscle antibodies and the relationship with HLA phenotypes and thyroid antibodies in 65 patients with endocrine orbitopathy classes 1–5. Both bovine and abdominal muscles were used as antigens in ELISA systems in which IgG and IgM antibodies were assayed. Before starting the immunosuppressant therapy, 46/65 patients (71%) showed a positive result for eye muscle antibodies. Those patients with an active disease had such antibodies more frequently. Where the ophthalmopathy was of recent onset IgM antibodies were found, whereas pati…
Direction‐dependent visual cortex activation during horizontal optokinetic stimulation (fMRI study)
2005
Looking at a moving pattern induces optokinetic nystagmus (OKN) and activates an assembly of cortical areas in the visual cortex, including lateral occipitotemporal (motion‐sensitive area MT/V5) and adjacent occipitoparietal areas as well as ocular motor areas such as the prefrontal cortex, frontal, supplementary, and parietal eye fields. The aim of this functional MRI (fMRI) study was to investigate (1) whether stimulus direction‐dependent effects can be found, especially in the cortical eye fields, and (2) whether there is a hemispheric dominance of ocular motor areas. In a group of 15 healthy subjects, OKN in rightward and leftward directions was visually elicited and statistically compa…
Progressive cerebellar ataxia, proximal neurogenic weakness and ocular motor disturbances: hexosaminidase A deficiency with late clinical onset in fo…
1997
Tay-Sachs disease is a genetically determined neurodegenerative disorder, resulting from mutations of the hexosaminidase (Hex) A gene coding for the alpha-subunit of beta-D-N-acetyl-hexosaminidase. Clinically, there is severe encephalomyelopathy leading to death within the first few years of life. Hex A activity is usually absent in tissue and body fluids of these patients. Juvenile and adult Hex A deficiencies are less severe but rare variants with some residual Hex A activity. All these variants are most prevalent among Ashkenazi Jews. We describe a non-Jewish family in which four adult brothers and sisters had markedly reduced Hex A activities and onset of symptoms in the second decade o…
Graves ophthalmopathy: role of MR imaging in radiation therapy.
1991
Twenty-three patients with Graves ophthalmopathy who underwent radiation therapy were monitored by means of magnetic resonance (MR) imaging. T2 relaxation times of extraocular muscles and orbital fat, areas of extraocular muscles, and degree of exophthalmos were measured by means of MR imaging at the beginning, at the end, and 3 months after completion of radiation therapy. As a result, patients with primarily elevated T2 times of extraocular muscles showed a better therapy response regarding muscle thickening than patients with primarily normal T2 times. Elevated T2 times, which probably represent acute inflammatory changes, were markedly decreased at the end of therapy. Therefore, quantit…
Trochlear palsies caused by isolated trochlear schwannomas.
2010
To describe clinical features and management of 4 patients suffering from unilateral superior oblique palsies due to MRI-documented trochlear nerve schwannomas.Chart reviews of 4 patients seen at the departments of ophthalmogy and neurology at the University of Mainz.All four patients were male, aged 36 to 72 years at initial presentation. None suffered from neurofibromatosis. The history of double vision prior presentation was 9 months to 13 years, follow-up time was 9 to 156 months. Two patients didn't receive any intervention: one remained stable over the follow-up time of 9 months. In patient #2, fourth nerve palsy was diagnosed 13 years prior to confirmation of a trochlear schwannoma b…
Ocular pseudomyasthenia: report of a case with a pineal region tumor.
1991
A case presenting with clinical features of ocular myasthenia and a false-positive edrophonium test is reported. Brain CT and MRI scan revealed a pineal region tumor histologically verified as germinoma. We recommend evaluating patients with clinical features of myasthenia gravis (MG) confined to the ocular muscles for intracranial mass lesions.