Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038379 (strabismus)
 9,317 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 41-year-old man was doing well until July 1989, when he noted numbness over soles, followed 4 months later by difficulty in walking. These symptoms were progressively getting worse, and he was admitted to our department on June 12, 1990. General physical examination was unremarkable. Neurologically cranial nerves were intact except old right, traumatic strabismus. Muscle tone and deep tendon reflexes were normal throughout, but bilateral Babinski and Chaddock reflexes were present. Mild weakness of lower extremities were found on muscle testing (4/5). In sensory system, superficial sensory disturbance below T10 was seen, and markedly diminished vibration and position senses of lower extremities were noted. Cerebellar test was intact, although unsteadiness was found on heel-shin test. Romberg sign was definitely positive. His gait was wide-based and ataxic. Laboratory data showed no abnormalities in CBC, chemistry, urinalysis, serological tests and endocrinological examinations. Spinal MRI (Siemens 1.5 Tesla) showed abnormal deposition of epidural fatty tissues compressing spinal cord with flattening of cord from T4 to T8. Spinal ataxia as compressive myelopathy due to epidural lipomatosis was considered and he underwent laminectomy from T4 to T8 with improvement in walking. Epidural lipomatosis is an unusual cause of spinal cord compression, presenting compressive myelopathy, radiculopathy, cauda equina syndrome, intermittent claudication, or back pain. Most of cases were associated with long-term administration of adrenocortical steroid hormone, or underlying diseases, except only 3 cases including ours. This is the first case of spinal epidural lipomatosis presenting progressive gait disturbance due to spinal ataxia.
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PMID:[A case of epidural lipomatosis presenting spinal ataxia]. 206 Feb 46

A 6-year-old boy was sent to us due to basal meningoencephalocele detected by MRI. He had had a past history of strabismus and morning glory syndrome since 6 months of age, as well as hypopituitary dwarfism since he was 3 years old. On admission, physical examination revealed hypertelorism and left coloboma. Laryngoscopic examination revealed a round mass covered by normal nasal mucose in the midline of the tegmen of the choana. X-ray tomogram and CT scan demonstrated an isodense mass protruding into the nasopharyngeal cavity through a bony defect of the sella turcica. Right carotid angiogram showed stenosis of the right internal carotid artery and abnormal fine vessels in the basal ganglia similar to basal Moyamoya network. There was an irregular filling of the right anterior cerebral artery. Left carotid angiogram showed an irregular filling of the left middle cerebral artery. MRI showed an anterior part of the third ventricle descending into, and the meningocele protruding into the nasopharyngeal cavity. It also showed a stalk extending from the hypothalamus into the meningocele, but the pituitary gland was not recognized. The meningocele was of the same signal intensity as CSF in both T1 and T2 weighted images. Growth hormone deficiency was confirmed by radioimmunoassay of the peripheral blood. An operation was scheduled transcranial to prevent snoring during sleep at age 9. The stalk was observed behind the chiasm during the operation, but a radical operation was not performed because of a possibility of postoperative hypothalamic-pituitary dysfunction. Transsphenoidal encephalocele is rare. So far as we could see, only 30 cases have been reported.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of transsphenoidal meningoencephalocele]. 224 2

Ocular muscle disturbances (strabismus) are common disorders affecting two to four per cent of the population. Until recently, CT and MRI have not been used extensively in the diagnosis of congenital and acquired conditions causing strabismus. In this article the value of these scanning techniques in terms of more appropriate therapeutic intervention and further insight into the pathophysiology of these conditions is detailed.
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PMID:Computed tomography scanning in the evaluation of ocular motility disorders. 329 74

Twenty infants, diagnosed by cranial ultrasound as having extensive cystic leukomalacia, had visual evoked responses (VER) and electroencephalograms (EEG) in the neonatal period and MRI scans later in infancy. The early ultrasound findings and results from the electrophysiological tests were correlated with later MRI findings and functional abilities. In infants with periventricular leukomalacia (PVL), the cysts were usually no longer visible by ultrasonography, beyond 40 weeks postmenstrual age (PMA), but later MRI scans showed a consistent pattern of delayed myelination around the irregularly dilated occipital horns of the lateral ventricles. VER's were present in the neonatal period and vision was maintained, although all infants developed a marked squint. EEG's were either normal or abnormal initially, but improvement was noted within several weeks. In those with subcortical or mixed lesions, cysts were noted to persist beyond 40 weeks PMA. Later MRI scans showed very poor myelination, with poor progress on subsequent scans and cortical atrophy. VER's were absent and all infants later became cortically blind. EEG's were severely abnormal and recovery was very poor. The infants with PVL developed spastic diplegia with moderate developmental delay, while those with mixed or subcortical lesions developed quadriplegia with severe mental retardation. An integrated approach, consisting of ultrasound imaging and electrophysiological recordings in the neonatal period and MRI imaging later in infancy, may provide a more reliable prediction of the pattern of later deficits.
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PMID:Neurological, electrophysiological and MRI abnormalities in infants with extensive cystic leukomalacia. 360 Sep 97

During a 5-month period, four patients developed contracture of the inferior rectus muscle following local anesthesia for cataract surgery in the practice of one ophthalmologist. Two anesthesiologists administered retrobulbar anesthesia. All patients had persistent vertical diplopia caused by a large hypotropia of the operated eye. Forced duction testing revealed marked restriction to elevation of the eye. In two patients, MRI demonstrated segmental thickening of the inferior rectus muscle, just posterior to the globe. All patients had normal thyroid function tests. No patient had a previous history of strabismus. Three of the four have had strabismus surgery consisting of recession of the markedly restricted inferior rectus muscle on an adjustable suture. These three patients have regained fusion and are now asymptomatic.
J Pediatr Ophthalmol Strabismus
PMID:A cluster of patients with inferior rectus restriction following local anesthesia for cataract surgery. 825 42

We present the work-up and treatment for 25 patients with inferior oblique palsy, including 2 with bilateral inferior oblique palsy and 23 with unilateral inferior oblique palsy. Nineteen patients were adults over the age of 21 years, and six were children under the age of 10 years. Sixteen adults and two children underwent CT scanning of the head. All scans were normal. Eight adults also underwent MRI evaluation of the head. These also were all normal. All adults had a negative Tensilon test for myasthenia gravis. The majority presented with a head tilt to the side of the paretic muscle. None of these patients complained of tilting images, but incyclotorsion was measurable in all cases that were tested. The most important conclusion from this study is that inferior oblique palsy is a benign entity, with none of these patients having a brain tumor or myasthenia gravis.
J Pediatr Ophthalmol Strabismus
PMID:Diagnosis and treatment of inferior oblique palsy. 801 89

Thirteen preterm children, aged 4 to 14 years, with visual impairment due to periventricular leukomalacia (PVL) were evaluated for visual function, intellectual level, cognitive profile and motor function. Their visual impairment was characterized by low acuity, crowding, visual field defects and ocular motility disturbances. Their cognitive profile was uneven, often with considerably higher scores on verbal than on visual-spatial tasks. Nine children had normal intelligence, three had mild mental retardation and one had severe mental retardation. In all the children, visual impairment was complicated by visual perceptual difficulties, accounting for their greater visual handicap than would be expected from their visual acuities and strabismus alone. Though CT or MRI revealed bilateral PVL in all the children, six had no motor impairment consistent with cerebral palsy, which is an unexpected finding.
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PMID:Visual impairment in preterm children with periventricular leukomalacia--visual, cognitive and neuropaediatric characteristics related to cerebral imaging. 876 Nov 68

Although systemic steroids or orbital radiotherapy are effective in limiting the inflammatory response in thyroid eye disease (TED), there are reports of over 70% of treated patients requiring subsequent rehabilitative surgery: either orbital decompression or strabismus correction. This study investigated whether combined immunosuppression with primary orbital radiotherapy together with azathioprine and low-dose prednisolone, applied early in the active disease state, was more effective in treating TED. Forty consecutive patients with active TED were recruited. Orbital MRI (STIR sequence) was used to assess disease activity. Median duration of symptoms was 1.0 year. Subjects were treated with bilateral orbital radiotherapy (20 Gy in 10 fractions) and oral prednisolone and azathioprine. Pre- and post-treatment activity was measured clinically, including uniocular field of fixation, Mourits score and total eye score, until TED became inactive off all treatment. Before treatment, 15 subjects had signs of dysthyroid optic neuropathy, 35 had significant motility restriction and 38 had marked soft tissue signs. On average TED became inactive after 1.2 years (SD 0.7) of immunosuppression, and treatment was well tolerated. One patient required subsequent cosmetic orbital decompression, 6 had successful strabismus surgery and 13 required minor cosmetic lid surgery. Compared with previously reported treatment regimes we think that combined orbital radiotherapy and medical immunosuppression is far more effective than either treatment alone in the management of active TED, and led to fewer side effects of high-dose steroids. In particular there was more than a four-fold reduction in the requirement for orbital decompression and strabismus surgery.
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PMID:Combined radiotherapy and medical immunosuppression in the management of thyroid eye disease. 947 1

We report a peculiar case of congenitally restricted sursumduction caused by an anomalous band-like structure within the right orbit which was completely distinct from the extraocular muscles. Imaging (static and dynamic ultrasound studies of the orbit and globe, CT and MRI) revealed the origin of the anomalous structure in Zinn's ring, its intraconal course and bulbar insertion. The ultrasound characteristics were similar to those of the extraocular muscles and optic nerve, and contractile activity appeared to occur during eye movements. The seemingly muscular nature of this structure and its total autonomy with respect to the normal extraocular muscles distinguish this case from previously reported cases of congenital restrictive strabismus.
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PMID:Restrictive limitation of sursumduction caused by an anomalous muscular structure. 978 36

The action of the superior oblique muscle depends on both the contractile function of the muscle belly as well as the force transmission function of the tendon trochlea complex. Brown's syndrome is a restriction to elevation in adduction presumed due to an anomaly of the superior oblique tendon trochlea complex. This report presents two patients with an unusual restriction to elevation in adduction in the affected eye, and a clinical picture consistent with a Brown's syndrome. A non-invasive analysis of the functional anatomy of the superior oblique was performed using a high-resolution MRI with surface coils. Imaging in the first case revealed a cyst in the area of the right superior oblique tendon just posterior to the trochlea in primary position. The second patient had undergone reattachment of an avulsed trochlea after a dog bite (canine tooth syndrome). Imaging in the second case revealed scarring and connective tissue disruption around the tendon impeding muscle function. These findings provide distinct mechanical explanations for Brown's syndrome.
Strabismus 1998 Mar
PMID:Magnetic resonance imaging features of two cases of acquired Brown's syndrome. 1062 44


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