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)

A 61-year-old man with lesions of the bilateral medial longitudinal fasciculus (MLF) and left paramedian pontine reticular formation (PPRF) caused by an infarction in the pontine tegmentum was reported. On admission, the ocular position of the left eye on forward gaze was fixed at the midline, while the right eye was abducted, and skew deviation was observed. On leftward gaze, neither eye could pass the midline, and on rightward gaze the right eye was abducted with monocular nystagmus, indicating paralytic pontine exotropia. On the fifth day, the bilateral eyes were abducted on forward gaze. On leftward gaze, the left eye was abducted slightly with monocular nystagmus. On rightward gaze, the right eye was abducted with monocular nystagmus. This issue was considered to be walleyed bilateral internuclear ophthalmoplegia (WEBINO). On the 37th day, right MLF syndrome was noted. About 2.5 months later, the disturbances of ocular movement disappeared. T2-weighted MRI showed high signal intensity lesions in the bilateral paramedian portion of the midpontine tegmentum. It suggests that WEBINO is caused by a lesion in the pons, and that paralytic pontine exotropia, one and a half syndrome and WEBINO are considered to be analogous to one another. The associated abnormality of vergence eye movements suggests that these are also generated by the PPRF in the pons.
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PMID:[A case of infarction in the pontine tegmentum involving the bilateral MLF and unilateral paramedian pontine reticular formation]. 761 50

We report a 79-year-old woman with basilar artery occlusion. She had a sudden onset of tetraplegia and disturbed consciousness, and within four days from the onset she showed a varied, fluctuating eye symptoms. On admission, she showed ocular bobbing, skew deviation with the right eye lower-positioned, upward gaze palsy, one-and-a-half syndrome, and paralytic pontine exotropia (PPE). On the third day after the onset, she showed wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome, and on the fourth day, she showed one-and-a-half syndrome again. Her right-gaze palsy improved repeatedly, and on the 19th day from the onset, only right MLF syndrome remained. Her eye symptoms fluctuated probably according to the distal migration of emboli, there by the responsible lesion and the mechanism of these eye symptoms are considered to be closely inter-correlated. On the fourth day after onset, the magnetic resonance imaging revealed cerebral infarctions in bilateral middle pons, the left paramedian lower pons, and the right paramedian midbrain, and a hemorrhagic infarction in the right inferior cerebellar hemisphere. We believe that that the eye symptoms of this patient were caused by lesions in the paramedian midbrain or pons.
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PMID:[A case of basilar artery occlusion associated with one-and-a-half syndrome, paralytic pontine exotropia and WEBINO-syndrome]. 919 98

Patients with supranuclear and internuclear ocular motility disorders may have nystagmus and oscillopsia, or need to adopt an abnormal head posture to either fixate or maintain binocularity. Many have a cosmetically unsatisfactory appearance. In addition, because of lesions involving ocular motor nuclei or nerve fascicles, double vision is also a common problem. The usual management of these patients is symptomatic with occlusion or prisms. We report on 11 patients who underwent extraocular muscle surgery with the aim of reducing symptoms and restoring or improving binocular single vision. Three patients had bilateral internuclear ophthalmoplegia with exotropia, 3 had dorsal midbrain syndrome, 2 had residual upgaze palsies after cerebral vascular accidents, 2 had oculopalatal myoclonus and one skew deviation. After surgery, symptoms, visual function and cosmesis improved in nearly all patients. We recommend that surgery should be considered more readily in the rehabilitation of these patients.
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PMID:Surgical treatment of supranuclear and internuclear ocular motility disorders. 937 80

The patient is a 72-year-old man who had tonsillitis and underwent incision on March 6, 1998. He complained headache and nausea from March 10 and double vision from March 12. He was admitted to our hospital on March 14. Neurological examination on March 18 revealed bilateral internuclear ophthalmoplegia with exotropia (WEBINO), bilateral ptosis and disturbance of convergence. Lumbar puncture revealed moderately elevated protein (46 mg/dl) with normal pressure and cells. The T2-weighted images of head MRI showed multiple high intensity lesions in anterior commissure and right pons. After he was treated with methylprednisolone, his headache and ptosis disappeared. The lesion in anterior commissure on MRI disappeared also. But WEBINO remained at the time of discharge. WEBINO syndrome is caused by lesion of pons and midbrain. The most common cause of bilateral internuclear ophthalmoplegia is multiple sclerosis in the European and American countries, but the frequency of vascular origin is higher in Japan than in the European and American countries. All the cases of WEBINO syndrome reported in Japan are vascular origin. This is the first case of WEBINO syndrome not of vascular origin.
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PMID:[A case of post-infectious wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome]. 1042 55

The congenital fibrosis syndromes (CFS), including congenital fibrosis of the extraocular muscles (CFEOM) and Duane syndrome (DS), are rare congenital strabismus syndromes that present with nonprogressive restrictive ophthalmoplegia with or without ptosis. Although historically believed to result from primary extraocular muscle (EOM) fibrosis, our laboratory's work is based on the hypothesis that these disorders result from distinct, but analogous, developmental defects of the oculomotor (nIII), trochlear (nIV), and abducens (nVI) nuclei. We have defined three inherited CFEOM phenotypes (CFEOM1-3) and have mapped each phenotype to a distinct genetic locus (FEOM1-3). Individuals with CFEOM1 are born with bilateral ptosis and both eyes fixed in a downward position with absent upgaze and aberrant horizontal gaze. This disorder maps to the FEOM1 locus on chromosome 12cen.(1,2) Neuropathology studies of CFEOM1 reveal the absence of the superior division of oculomotor nerve and its corresponding alpha motor neurons in the midbrain, with abnormalities of target EOMs.(3) These neuropathology findings parallel those previously identified in Duane syndrome, in which there is an absence of nVI and the abducens nerve.(4,5) Individuals with CFEOM2 are born with bilateral ptosis and exotropia. This atypical form of CFEOM maps to the FEOM2 locus on chromosome 11q13 and results from mutations in ARIX (PHOX2A).(6,7) ARIX encodes a homeodomain transcription factor protein previously shown to be required for nIII/nIV development in mouse and zebrafish.(8,9) Together, these findings support the hypothesis that the congenital fibrosis syndromes result from parallel defects in nIII, nIV, and nVI nuclear development. Functional studies of the CFEOM genes should provide additional insight into the unique features of the extraocular lower motor neuron axis in health and disease. (For full (refs. 1-9), see reference list of the main paper.)
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PMID:Applications of molecular genetics to the understanding of congenital ocular motility disorders. 1196 Jul 93

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is a rare disorder consisting of a bilateral adduction deficit and primary gaze position exotropia. Associated with bilateral medial longitudinal fasciculus lesions, it has been mostly reported in patients with multiple sclerosis and brainstem stroke. A 72-year-old man with characteristic clinical features of progressive supranuclear palsy (PSP) later developed WEBINO. Brain MRI revealed atrophy of the midbrain tegmentum. Caloric irrigation revealed intact horizontal eye movements in both eyes. We believe this to be the first report of WEBINO in PSP. The preservation of vestibulo-ocular horizontal eye movements supports the notion that the WEBINO in this condition was caused by a supranuclear rather than a nuclear lesion and suggests the possibility that even in other causes of WEBINO, the lesion is supranuclear and not in the medial rectus subnucleus as is often suggested.
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PMID:Wall-eyed bilateral internuclear ophthalmoplegia in a patient with progressive supranuclear palsy. 1877 46

Tolosa-Hunt syndrome (THS) is characterized by unilateral painful ophthalmoplegia with oculomotor paresis, associated with an idiopathic granulomatous inflammation involving the cavernous sinus, with a typical relapsing-remitting course. We report a case of an 8-year-old girl who was admitted because of an ophthalmoplegia with exotropia and ptosis of the left eyelid, accompanied by diplopia and left sovraorbital pain. The clinical data, neuroradiological findings and response to steroid treatment suggested THS, as defined by the 2004 International Classification of Headache Disorders (ICHD)-II criteria. THS must be considered a possible cause of painful ophthalmoplegia in childhood, as well as in adults, and confirmed with a focused neuroradiological investigation. The few paediatric cases described in the literature that meet the 2004 ICHD-II criteria are not sufficient to identify possible differences between the paediatric and the adult forms. Every new paediatric case should therefore be reported in order to gather and compare further information.
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PMID:The Tolosa-Hunt syndrome in children: a case report. 1955 37

Carbon monoxide poisoning, which occurs in poorly ventilated areas, is associated with different symptoms and signs. This report describes an 8-year-old girl with severe carbon monoxide intoxication, scattered retinal nerve fiber layer and subhyaloid hemorrhages, and exotropia with bilateral internuclear ophthalmoplegia.
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PMID:Carbon Monoxide Poisoning: A Patient with Bilateral Internuclear Ophthalmoplegia and Exotropia. 1964 81

We report a patient with progressive supranuclear palsy (PSP) with his serial photographs before the onset of ocular symptoms and after the onset with two year intervals. These photographs show his progressive eyeball deviations toward complete exotropia. There were no effective voluntary eyeball movements, Bell's phenomenon, doll's eye movements, and vestibulo-ocular reflexes. These signs indicate the involvement of the oculomotor nuclear complex by the disease. We suggest that PSP may cause not only 'supranuclear' but also 'nuclear' complete ophthalmoplegia with exodeviation of the eyes.
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PMID:Exodeviated ophthalmoplegia in a patient with progressive supranuclear palsy. 1979 6

Wall-eyed monocular internuclear ophthalmoplegia (WEMINO) with contraversive ocular tilt reaction has not been previously reported. A 71-year-old woman suddenly developed blurred vision. Examination revealed left internuclear ophthalmoplegia, left exotropia, right hypotropia, and rightward head tilt. Magnetic resonance imaging showed a tiny infarction at the area of the left medial longitudinal fasciculus in the upper pons. WEMINO with contraversive ocular tilt reaction may be caused by a paramedian pontine tegmental infarction that selectively involves the medial longitudinal fasciculus.
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PMID:Wall-Eyed Monocular Internuclear Ophthalmoplegia (WEMINO) with Contraversive Ocular Tilt Reaction. 2039 78


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