Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029089 (ophthalmoplegia)
 3,338 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We studied three patients with spontaneous mesencephalic hemorrhages. All presented with some degree of impaired consciousness and abnormal ocular movements. One patient had a convergence-retraction nystagmus with a unilateral hemorrhage confined to the dorsal midbrain. Another patient showed left third nerve palsy due to a unilateral hemorrhage that involved ventral midbrain. The third patient revealed left internuclear ophthalmoplegia with ataxia due to a pinpoint hemorrhage in the tegmentum of the left midbrain. The patients survived and major neurologic deficits recovered, but abnormal ocular movements persisted.
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PMID:Mesencephalic hemorrhage--a report of 3 cases. 278 33

Internuclear ophthalmoplegia is characterized by an adduction deficit on lateral gaze with dissociated nystagmus of the abducting eye. It is seen with lesions of the medial longitudinal fasciculus. In myasthenia gravis, extraocular muscle weakness can cause the same oculomotor pattern, which has been referred to as pseudo-internuclear ophthalmoplegia. We report the additional finding of downshoot in the adducting eye in two patients with pseudo-internuclear ophthalmoplegia and positive Tensilon tests.
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PMID:Pseudo-internuclear ophthalmoplegia with downshoot in myasthenia gravis. 295 87

A case of bilateral internuclear ophthalmoplegia of long duration with autopsy confirmation is reported. The main features of the syndrome included paresis of ocular adduction upon attempted lateral gaze, horizontal nystagmus in the abducting eye, and an absence of converging eye movements. Examination of Weil-stained sections revealed multiple plaques of demyelination. The medial longitudinal fasciculus was demyelinated bilaterally in the upper pons-caudal midbrain. The oculomotor, trochlear, and abducens nuclei appeared relatively well preserved. The internal capsule was severely damaged. Large cyst-like structures were centered in the anterior limb bilaterally and extended caudally to the level of the genu. Plaques of demyelination were present bilaterally in the posterior limb. The left side of the internal capsule was more severely affected than the right. It is thought that convergence in this case may have been eliminated by interruption of fibers from the frontal eye fields and/or other cortical areas in their descent through the internal capsule.
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PMID:Bilateral internuclear ophthalmoplegia with absence of convergent eye movements. Clinicopathologic correlation. 300 54

Internuclear ophthalmoplegia is a gaze disorder characterized by impaired adduction on the side of a lesion involving the medial longitudinal fasciculus with dissociated nystagmus of the abducting eye. Eleven patients with internuclear ophthalmoplegia (nine with clinical multiple sclerosis, two with clinical infarction) underwent MR imaging with spin-echo techniques on a 1.5-T system. Nine patients also had CT. MR showed focal or nodular areas of high signal intensity on T2-weighted images in the region of the medial longitudinal fasciculus in 10 of 11 patients. In one of four patients with internuclear ophthalmoplegia who had MR after intravenous gadolinium-DTPA, an enhancing ring lesion was seen in the region of the medial longitudinal fasciculus on short TR/TE images, indicating active blood-brain-barrier disruption, which correlated with this patient's recent-onset internuclear ophthalmoplegia. CT failed to show the lesions in all nine patients examined. This report demonstrates the superiority of MR in evaluating gaze disorders attributable to brainstem dysfunction, such as internuclear ophthalmoplegia, and correlates MR findings with the relevant neuroanatomy of the medial longitudinal fasciculus.
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PMID:Internuclear ophthalmoplegia: MR-anatomic correlation. 310 83

A new patient with Leigh's syndrome (subacute necrotizing encephalomyelopathy due to pyruvate dehydrogenase complex deficiency) is presented. A Turkish boy of consanguinously married healthy parents developed progressive muscle weakness since infancy. At the age of 3 years he was unable to sit, stand or walk. Clinical examination showed general muscle weakness, hypotonia, muscle hypotrophy, bilateral ptosis, partial bilateral external ophthalmoplegia, nystagmus, intention tremor and hypoactive tendon reflexes. The EEG showed diffuse slowing, the cerebral CT scan disclosed mild hydrocephalus e vacuo. Motor nerve conduction velocity was slightly decreased, the EMG revealed signs of neuropathy. In the biopsied muscle only a mild hypotrophy of type 2 fibres was found, no abnormal mitochondria could be detected. The sural nerve was slightly abnormal: loss of large myelinated axons, loss of unmyelinated nerves. CSF protein was elevated to 80 mg/dl, protein electrophoresis revealed the pattern of markedly impaired blood-CSF barrier. Serum lactate and pyruvate were permanently elevated. In the urine the excretion of alanine was raised. The clinical state deteriorated during intercurrent infections; somnolence, vomiting and Cheyne-Stoke's respiration occurred. At the age of 3 1/2 years the child died of pneumonia. In the liver tissue a decreased activity of the pyruvate dehydrogenase complex was found. Neuropathological examination of the brain demonstrated wide-spread changes of Leigh's spongiform encephalopathy. Several enzyme deficiencies have hitherto been associated with Leigh's syndrome: This patients confirms earlier findings that a subgroup of Leigh's syndrome is caused by pyruvate dehydrogenase complex deficiency.
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PMID:[Leigh's subacute necrotizing encephalomyelopathy due to decreased activity of the pyruvate dehydrogenase complex]. 312 26

Otoneurological and ultra low field MRI findings in multiple sclerosis patients: 22 patients suffering from multiple sclerosis (MS) underwent thorough neurological and otological examination, extensive ENG testing and Magnetic Resonance Imaging (MRI) examination of the central nervous system. All patients fulfilled the Schumacher criteria for a diagnosis of definitive MS. 20 of the patients had been included in previous otoneurological studies three to five years ago. The general disability assessed according to Hyllested's scale was: Class 1-7, Class 2-4, Class 3-6, Class 4-3, Class 5-2 patients. Thus 11 patients had none or only slight disability. Nevertheless, all patients showed abnormal findings when classified according to the Kurtzke disability scale, which reflects the involvement of separate neuronal entities. The ENG examination revealed abnormal findings in all patients. The most common abnormalities found were as follows: abnormal pendular test 19, ocular fixation index 18, optokinetic nystagmus 14, saccadic eye movements 14 and spontaneous nystagmus 12. 14 patients had uni- or bilateral abnormally slow adduction movements in the saccadic test consistent with internuclear ophthalmoplegia (INO), which is caused by a lesion of the brain stem. MRI examination of the 21 patients studied revealed abnormal findings consistent with MS in sixteen cases. The lesions were unilateral in 5 and bilateral in 11 patients. The most common location for these abnormal findings consistent with MS plaques were in the white matter around the lateral ventricles. Plaques in the brain stem and/or cerebellum were found in only two cases despite numerous clinical and otoneurological findings that indicated the presence of functional lesions in these areas.
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PMID:Otoneurological and ultra low field MRI findings in multiple sclerosis patients. 320 65

An increased compendium of drugs useful in ocular motor system dysfunction has expanded our capacity to treat selected ocular motility disorders. Adjunctive therapeutic modes (e.g., Fresnel prisms and orthoptic exercises) can also be beneficial. PAN and see-saw nystagmus can be treated with baclofen. Downbeat nystagmus may respond to clonazepam therapy, and prisms may help if the nystagmus can be modified with convergence. Congenital nystagmus may respond minimally to drugs (e.g., baclofen), but prisms or surgical procedures, or both, are still the primary treatment modalities. Innovar may be helpful in patients with severe, incapacitating vestibular disorders, and scopolamine alone or in combination with promethazine may be beneficial in patients with milder ambulatory acute peripheral vestibular disorders. Benign positional vertigo is best treated initially with positional exercises before drug therapy is instituted. Opsoclonus and ocular flutter have been treated successfully with corticosteroids, propranolol, and clonazepam, while microflutter, an extremely rare disorder, can resolve with baclofen. Although therapy with carbamazepine, 5-hydroxtryptophan, and scopolamine has been useful in selected patients with ocular palatal myoclonus, most do not respond to drug treatment. It is not usually necessary to treat voluntary nystagmus, but Fresnel prism lenses should be remembered in refractory patients. Potentially reversible and pseudointernuclear ophthalmoplegias also were discussed. Orthoptic exercises can be beneficial in posttraumatic internuclear ophthalmoplegia. Selected supranuclear palsies can be improved completely with the proper drug regimen. Lastly, superior oblique myokymia can be treated successfully with carbamazepine, with tight surveillance for possible adverse side effects. Descriptive phenomenology and pathophysiological localization must be correlated with brain stem neurochemistry and neuropharmacology to medically treat additional ocular motor system disorders.
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PMID:Medical treatment of nystagmus and ocular motor disorders. 349 76

We tested the hypothesis that abnormalities of the abducting eye in internuclear ophthalmoplegia reflect an adaptive process that helps overcome the adduction weakness of the opposite eye. This response operates under the constraints of Hering's law of equal innervation: any attempt to increase the innervation to a weak muscle in one eye must be accompanied by a commensurate increase in innervation to the yoke muscle in the other eye. In 4 patients with internuclear ophthalmoplegia, we patched one eye for 1 to 5 days to allow time for the central nervous system to optimize innervation for the habitually viewing eye. We predicted that there would be a conjugate adjustment of innervation that would diminish the abduction overshoot and backward postsaccadic drift made by the habitually viewing eye. This was the case in 3 of our 4 patients. Our findings show that the abduction nystagmus is a manifestation of a normal adaptive response in some patients with INO.
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PMID:Abduction nystagmus in internuclear ophthalmoplegia. 357 24

Communicating syringes confined to the brain stem are extraordinarily rare. Two patients, presenting with signs and symptoms of cerebellar dysfunction, later developed evidence of brain-stem disease with dysarthria, nystagmus, deafness, and internuclear ophthalmoplegia. The condition of both patients had been diagnosed clinically as multiple sclerosis, but at autopsy they had a striking keyhole-shaped syrinx in the midbrain and upper pons, which communicated with the aqueduct and fourth ventricle without associated syringomyelia. In addition, both patients had marked atrophy and gliosis of the cerebellum, one with extension of the syrinx into cerebellar folia. The unique character of these lesions coupled with the similarity of the clinical features of the cases prompted us to name this disorder--"keyhole aqueduct syndrome."
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PMID:Keyhole aqueduct syndrome. 374 Dec 9

We examined ophthalmologically nine children with lactic acidosis. All showed abnormal ocular findings. Optic atrophy was present in six patients, nystagmus in three, blepharoptosis in one, cataract in one, and limitation on abduction in one. We believe that optic atrophy is the most frequent ocular finding and that nystagmus and ophthalmoplegia are common associations in patients with childhood lactic acidosis.
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PMID:Ocular findings in childhood lactic acidosis. 377 83


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