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)

Purpose: A young patient of ophthalmoplegic migraine with recurrent oculomotor nerve palsy is reported.Case: A 4-year-old girl came to our hospital complaining of recurrent left ptosis and double vision which completely resolved within a few days. She had a history of drowsiness after vomiting every two months. Left oculomotor nerve palsy with internal ophthalmoplegia was observed. Electroencephalography revealed mild changes, but computed tomography, single photon emission computed tomography, and cerebrovascular Doppler-ultrasound examination demonstrated no abnormalities. This oculomotor nerve palsy completely improved on the next day.Results: Thereafter, left episodic mydriasis and oculomotor nerve palsy occurred twice each. However, the frequency and symptoms of migraine were remarkably reduced by topical administration of 0.25% timolol maleate twice daily to both eyes.Conclusion: In isolated oculomotor nerve palsy in childhood, ophthalmoplegic migraine should be also considered. We emphasize that a detailed case history is very important to its diagnosis.
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PMID:A Pediatric Case of Ophthalmoplegic Migraine with Recurrent Oculomotor Nerve Palsy. 1103 60

Painful ophthalmoplegia in childhood has different causes. One is Tolosa-Hunt syndrome, in which a first episode may be difficult to diagnose because of its clinical similarity to ophthalmoplegic migraine. A 10-year-old male with painful ophthalmoplegia and a cavernous sinus inflammation associated with an intracavernous carotid stenosis demonstrated by magnetic resonance imaging and angiography is reported. These findings resolved in follow-up imaging. This report suggests that in the presence of painful ophthalmoplegia, magnetic resonance imaging detection of cavernous sinus inflammation can facilitate the diagnosis of Tolosa-Hunt syndrome when other causes are excluded.
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PMID:Painful ophthalmoplegia with reversible carotid stenosis in a child. 1137 11

The demonstration by magnetic resonance imaging (MRI) scanning of thickening and enhancement of the cisternal part of the oculomotor nerve in patients diagnosed as "ophthalmoplegic migraine" prompts reconsideration of this uncommon disorder. The case histories of five patients, three male and two female, varying in age from 6 to 30 years, are presented here. Recurrent painful ophthalmoplegia started in infancy in two cases, childhood in two instances and adult life in one. One child had his first attacks at 3, 5 and 12 months of age, on each occasion 10 days after an injection of triple vaccine. The possibility of this condition being a recurrent demyelinating neuropathy is considered and its possible relationship to migraine explored.
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PMID:Ophthalmoplegic migraine: a recurrent demyelinating neuropathy? 1142 86

Two patients who presented with ophthalmoplegic migraine underwent electroencephalography, brain magnetic resonance imaging, cerebral angiography, and technetium Tc 99m ethyl cysteinate dimer single photon emission computed tomography (SPECT) during an attack. Follow-up SPECT was performed after neurologic symptoms resolved. In both patients, SPECT during an attack of ophthalmoplegia and headache demonstrated significantly decreased regional cerebral blood flow (rCBF) in the thalamus on the side of the ophthalmoplegia; rCBF reverted to normal on a follow-up SPECT during the symptom-free period. These findings suggest reversible ischemia in the territories of perforating branches of the posterior cerebral artery may accompany ophthalmoplegic migraine and possibly bear some relationship to the clinical features.
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PMID:Ophthalmoplegic migraine with reversible thalamic ischemia shown by brain SPECT. 1200 88

Leigh disease, subacute necrotizing encephalomyelopathy, is a neurodegenerative disorder often seen in infancy or childhood but rarely reported in adults. Genetic heterogeneity is well recognized, and the associated etiologies include both mitochondrial and nuclear DNA defects. We describe an infant presenting with developmental delay and then progressive multisystem disorder and neuroradiologic features of Leigh disease. He and his maternal relatives all have the A8344G mitochondrial DNA mutation. However, only minor clinical features are seen in his maternal relatives, with migraine being the most common problem. Additionally the A8344G mitochondrial DNA mutation is associated with spinocerebellar degeneration, other nonspecific mitochondrial encephalomyopathies, atypical Charcot-Marie-Tooth disease, and progressive external ophthalmoplegia. The A8344G mitochondrial DNA mutation may present with Leigh disease or other different atypical clinical features without myoclonic epilepsy and ragged red fibers.
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PMID:Leigh disease with mitochondrial DNA A8344G mutation: case report and brief review. 1266 41

Ophthalmoplegic migraine is a rare presentation of migraine complicated by an isolated oculomotor paresis. Vasodilation of extracranial vessels is believed to underlie the headache, and vasoconstriction to account for the ophthalmoplegia. Whether the vascular insult involves the central or peripheral portions of the oculomotor nerve is still uncertain. We describe a child who presented with ophthalmoplegic migraine and was demonstrated to have a deficiency of the near triad documented by eye movement and pupillary recordings. Voluntary conjugate eye movements--saccades, smooth pursuit, and optokinetic nystagmus--were normal. Vergence amplitudes appropriate to fixation distance were elicited for Snellen optotypes but not to a point source of light. Concurrent measures of pupillary diameter failed to reveal significant modulation to either stimulus. Brain magnetic resonance imaging scan was normal, and there was no contrast enhancement of the oculomotor nerve at its exit from the midbrain. Both the oculomotor paresis and concurrent presence of a deficiency of the near triad localized the vascular insult to the oculomotor nerve complex in the brainstem.
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PMID:Ophthalmoplegic migraine. 1473 55

Ophthalmoplegic migraine is a rare condition, previously thought to represent a variant of migraine. Recent observations regarding its usual clinical presentation and common magnetic resonance imaging findings have given rise to speculation that this illness is more likely to represent an inflammatory cranial neuropathy. The recent revision of the International Headache Classification has reclassified ophthalmoplegic migraine from a subtype of migraine to the category of neuralgia. In this article, potential pathophysiological mechanisms are discussed. The typical clinical presentation of ophthalmoplegic migraine generally involves transient migraine-like headache accompanied by often long-lasting oculomotor, abducens or, rarely, trochlear neuropathy with diplopia and (if oculomotor nerve is involved) pupillary abnormalities and ptosis. Ophthalmoplegic migraine generally occurs in children, but a number of adult cases have been reported. Prognosis is good because symptoms almost always resolve, but, after several episodes, some deficits may persist. Differential diagnosis is rather large, although most other possible causes of ophthalmoplegia and headache have distinctive presentations or can be excluded with fairly straightforward diagnostic testing. Optimal prophylactic and acute treatment is still unclear, but migraine prophylactic medications such as b blockers and calcium channel blockers have been proposed. Steroids have been used with mixed results.
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PMID:Ophthalmoplegic migraine. 1522 91

Painful ophthalmoplegia is an important presenting complaint to emergency departments, ophthalmologists, and neurologists. The etiological differential diagnosis of painful ophthalmoplegia is extensive and consists of numerous sinister etiologies including vascular (eg, aneurysm, carotid dissection, carotid-cavernous fistula), neoplasms (eg, primary intracranial tumors, local or distant metastases), inflammatory conditions (eg, orbital pseudotumor, sarcoidosis, Tolosa-Hunt syndrome), infectious etiologies (eg, fungal, mycobacterial), and other conditions (eg, microvascular infarcts secondary to diabetes, ophthalmoplegic migraine, giant cell arteritis). A systematic approach to the evaluation of painful ophthalmoplegia can lead to prompt recognition of serious disorders that if left untreated, can be associated with significant morbidity or mortality. Inflammatory conditions such as Tolosa-Hunt syndrome and orbital pseudotumor are highly responsive to corticosteroids, but should be diagnoses of exclusion.
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PMID:Painful ophthalmoplegia: overview with a focus on Tolosa-Hunt syndrome. 1522 94

Brain single photon emission computed tomography (SPECT) studies were conducted in three patients with A3243G mutation of the mitochondrial (mt) DNA tRNA. All were born to mothers suffering from chronic progressive external ophthalmoplegia (CPEO) with the same A3243G point mutation of the mtDNA tRNA. The first case manifested clinically with MELAS, the second case manifested with CPEO, and third case was characterized by recurrent migraine-like headache, tremor, and epilepsy. Brain SPECT of all patients, regardless of whether they had or had not suffered from stroke-like episodes, showed multiple areas of asymmetrical decreased perfusion, particularly in the posterior and lateral head regions, especially the temporal lobes. Crossed-cerebellar diaschisis may occur. Conventional brain magnetic resonance images failed to show some of the lesions. Decreased regional cerebral blood flow, rather than previously proposed hyperemia, is likely to be the cause. We conclude that mitochondrial vasculopathy with regional cerebral hypoperfusion may be seen on brain SPECT in patients with mitochondrial disorders and A3243G mutations, regardless of whether they have or have not suffered from stroke-like episodes.
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PMID:Brain single photon emission computed tomography in patients with A3243G mutation in mitochondrial DNA tRNA. 1596 44

Ophthalmoplegic migraine is a rare syndrome in which episodic fixed unilateral headaches are associated with ipsilateral ophthalmoplegia. Its physiopathology remains obscure. We describe a case in a patient with ophthalmoplegic migraine diagnosed according to the International Headache Society (IH-2004) criteria, who showed an unusual clinical presentation. The first ophthalmoplegic migraine episode occurred in adult life. Pain side changed in one episode. Oculomotor abnormalities were painless during the last crisis. Since diagnosis is made by exclusion, differential diagnosis and need for etiologic investigation are discussed.
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PMID:[Ophthalmoplegic migraine: case report]. 1718 45


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