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Query: UMLS:C0018681 (headache)
 56,091 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A patient aged 39 years with Wyburn-Mason syndrome is presented. He had an apple-sized intracranial arteriovenous angioma racemosum of the left temporo-medical region, which was inoperable, as it extended to the basal ganglia. It was diagnosed by carotid angiography and by sequential brain szintigraphy. More than 50 retinal arteriovenous anastomoses were observed in the left eye, involving all quadrants and the macular region. Some of these anastomoses were only found by fluorescence angiography. Clinical signs were hemiparesis and decreased sensibility of the right side, headaches, speech disorders and right homonymous hemianopia. Vision was decreased to 0.4. By partial embolisation via the right common carotid, it was possible to reduce the size of the intracranial hemangioma.
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PMID:[The Wyburn-Mason syndrome (author's transl)]. 99 84

Wyburn-Mason's syndrome is a rare neurocutaneous disorder consisting mainly of unilateral arteriovenous malformations of the midbrain and retina with multiple cutaneous nevi. The authors report on the clinical presentation, neurologic phenotype, and long-term neurologic follow-up of two unrelated children. The first patient had recurrent epistaxis during early childhood. At the age of 7 years, he developed acute hemianopsia and right hemiplegia. Angiography revealed large bilateral arteriovenous malformations involving the midbrain thalamic area and the right optic nerve. During the following years, he had recurrent episodes of headache, right hemiplegia, and cognitive deterioration. The second patient had some episodes of epistaxis in the first years of life. At the age of 5 years, he presented with sudden onset of headache, followed by a loss of consciousness, vomiting, and, subsequently, visual disturbances. Angiography revealed deeply located arteriovenous malformations involving the right temporal, frontobasal, capsulonuclear, insular, and parietal areas and the right optic nerve. During the following years, he had an acute strokelike episode followed by transient hemiplegia and slow progressive signs, with mild worsening of cognitive abilities. Early onset of neurologic manifestations is a poor prognostic factor for long-term outcome.
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PMID:A neurocutaneous disorder with a severe course: Wyburn-Mason's syndrome. 1565 97

Wyburn-Mason syndrome is a rare, non-hereditary congenital neurocutaneous disorder leading to arteriovenous malformations. Malformations are characterized by an artery that is directly connected to veins without a capillary system and forms a fragile mass of abnormal vessels. It can be found in the midbrain, in the eyes, orbit, and rarely in cutaneous nevi. Neurological and ocular symptoms are the most common. Ocular signs and symptoms include abnormally dilatated vessels of conjunctiva, nystagmus, strabismus, vitreous hemorrhage, vein occlusions, retinal detachment, etc. Neurological symptoms may include headaches, paralysis, epistaxis, hydrocephalus, and hemiparesis. Imaging modalities such as MRI/CT angiography, optical coherence angiography, and fluorescein angiography are the most important for the identification of arteriovenous malformations. In our case report, we present an eight-month-old girl with an incidental finding of retinal angiomatosis on the left eye and was subsequently diagnosed with Wyburn-Mason syndrome. We compare the findings from the first visit to her clinical findings 20 years later.
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PMID:The Natural History of Retinal Vascular Changes from Infancy to Adulthood in Wyburn-Mason Syndrome. 3318 56