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The Sturge-Weber syndrome includes unilateral cerebral cortical angiomatosis, which often leads to progressive cerebral dysfunction and epileptic seizures that are medically difficult to control. Cerebral resections and hemispherectomy have been successfully performed in the past in intractable
Three children with circumscribed unilateral pial angiomatosis had both generalised and partial seizures associated with bilateral synchronous spike-wave complexes. Dramatic control of the seizures was obtained by surgical removal restricted to the angiomas and underlying cortex. There was
Diffuse cerebral angiomatosis is a rare disorder of the brain and only 5 cases have been reported to date. We report a 16-year-old female patient who presented to the emergency department with seizures. The patient had no hereditary syndromes, no epistaxis, no skin lesions and no telangiectasia.
Tuberous sclerosis complex is an autosomal dominant disorder affecting primarily the central nervous system, skin, and kidney caused by mutations in the TSC1 and TSC2 genes. Diagnosis is established with the identification of various neurocutaneous symptoms and multiple organ system hamartomas. The
A 17-year-old boy with epileptic seizures due to meningio-angiomatosis without neurofibromatosis type 2 is presented. Low grade astrocytoma in the left temporal lobe was resected when he was 11 years old. A recurrence was suspected on following-up MRI and a positive PET scan with 11C-methionine PET
A case is reported of an infant affected with Sturge-Weber disease who underwent left hemispherectomy due to untreatable seizures when 97 days old. Pathological analysis of the surgical specimens revealed the presence of four-layered microgyric cortex below the angiomatosis, intense gliosis, and the
Phakomatoses or neurocutaneous syndromes are an important cause of seizures in the pediatric age group. The Sturge-Weber syndrome may affect the eye, skin and brain at different times. The skin lesions need not always manifest. We report a case of isolated affection of the central nervous system in
We report an unusual case of encephalotrigeminal angiomatosis in which the facial and oral angioma was bilateral, and several teeth were congenitally absent. The developmental nature of the anomaly is reviewed. Encephalotrigeminal angiomatosis is commonly referred to as the Sturge-Weber syndrome,
We report six cases of meningio-angiomatosis, a disorder of the cerebral cortex of probable malformative origin frequently associated with neurofibromatosis and either asymptomatic or associated with a seizure disorder. The patients, three males and three females, ranged from 10 to 70 years of age
We present two patients with unilateral occipital gyriform calcification and seizures. Gyriform or serpentine calcification as revealed by computed tomography (CT) scan is rare and is a characteristic finding of Sturge-Weber syndrome (SWS) and celiac disease (CD). These patients had neither the
During the third quarter of her pregnancy, a young woman with Sturge-Weber angiomatosis had a severe right hemiplegia with hemianopia and aphasia, followed 48 hours later by focal seizures. Neuroimaging did not show any cerebral lesion but contrast magnetic resonance imaging revealed a left
Cutaneous vertebral medullary angiomatosis, also known as Cobb syndrome, is a rare segmental neurocutaneous syndrome. This syndrome is considered to be a non-hereditary congenital disease that is usually associated with arteriovenous malformations in the skin and spine. The clinical manifestations
A case of cerebral angiomatosis in a cat was associated with neurologic signs characterized by clusters of severe generalized seizures. Bilaterally in the gray matter, most prominent in the cingulate gyrus, there was focal accumulation of garlandlike arrangements of blood vessels. Vessels exhibited
Two patient with familial cavernous angiomatosis presenting with long lasting variable epilepsy with a poor therapeutic response and variable neurologic impairments are presented here. One of the numerous cavernous angiomas was resected in one case. This last patient remains asymptomatic. Familial
Background: Sturge-Weber syndrome (SWS) is primarily diagnosed in pediatric population, but clinical presentation in late adulthood is rarely reported. Evolution of radiological findings in the adulthood variant of SWS with isolated