telangiectasis/seizures

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Sudden death from an epileptic seizure due to capillary telangiectasias in the hippocampus.

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Cerebral capillary telangiectasia (CCT) is a type of vascular malformation that is incidentally encountered in clinical practice. Diseased vessels are small and usually clinically benign over the course of a patient's life. Although most CCT patients are asymptomatic, the situation becomes

Expansion of pulmonary arteriovenous malformations after grand mal seizures and other circumstances of PAVM growth.

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A woman with asymptomatic pulmonary arteriovenous malformation (PAVM) discovered incidentally on admission developed recurrent generalised seizures. Immediately after, the PAVM demonstrated marked expansion, and was safely resected. Congenital PAVMs (associated with hereditary haemorrhagic

Vascular malformations of the brain in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease).

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Six patients with vascular malformation of the brain in hereditary hemorrhagic telangiectasia (HHT) were reviewed to determine clinical and radiographic characteristics of these lesions. There were two patients with arteriovenous fistula (AVF), three with arteriovenous malformation (AVM), and one

[A Case of Pial Arteriovenous Fistulae Associated with Hereditary Hemorrhagic Telangiectasia]

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Pial arteriovenous fistulae(pial AVF)are rare vascular lesions. Pial AVF is a complication of hereditary hemorrhagic telangiectasia(HHT)and is associated with a high mortality rate. Here, we report a case of a 14-year-old boy with pial AVF associated with HHT who presented with a seizure. CT and MRI

Bleeding risk of cerebrovascular malformations in hereditary hemorrhagic telangiectasia.

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OBJECTIVE Hereditary hemorrhagic telangiectasia (HHT) is a rare autosomal-dominant vascular dysplasia with a high prevalence of cerebrovascular malformations (CVMs), mostly manifested as arteriovenous malformations (AVMs). The natural history and bleeding risk of these CVMs is unknown. The authors

A novel BMPR2 mutation in a patient with heritable pulmonary arterial hypertension and suspected hereditary hemorrhagic telangiectasia: A case report

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Rationale: BMPR2 mutation is the most common cause of heritable pulmonary arterial hypertension (HPAH), but rare in hereditary hemorrhagic telangiectasia (HHT). ACVRL1, ENG and SMAD4 are the most common gene mutations reported in HPAH

Symptomatic large or giant capillary telangiectasias: management and outcome in 5 cases.

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Brain capillary telangiectasias (BCTs) are usually small and benign with a predilection in the pons and basal ganglion. Reports of large and symptomatic BCTs are rare. Large BCTs have a much higher risk of causing uncontrolled bleeding and severe neurological defects, and they can be fatal if left

[Myoclonic cerebellar dyssynergia (Ramsay-Hunt syndrome) and cerebellar telangiectasia].

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A 8 year-old girl presented with generalized epileptic seizures followed by the progressive onset of myoclonic jerks, sometimes associated with willed movements, and a static and kinetic cerebellar syndrome without conspicuous intellectual impairment. Death occurred 10 years after the onset of the

[Familial brain abscess as a complication of hereditary hemorrhagic telangiectasia].

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The hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease) is an inherited autosomal dominant disease with angiodysplasia of the skin, mucosa, parenchymal organs, and it can affect the central nervous system. In 40% of the cases neurological complications, most frequently intracerebral

Capillary telangiectasis of the brain in chimpanzee.

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The occurrence of capillary telangiectasis of the brain in a chimpanzee is reported. The telangiectases were multiple and diffusely scattered throughout the brain although the cerebral and cerebellar cortex were particulary affected. Hemorrhage into surrounding gliotic parenchyma was present. The

Cerebrovascular manifestations in 321 cases of hereditary hemorrhagic telangiectasia.

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OBJECTIVE Patients with hereditary hemorrhagic telangiectasia (HHT) are at risk for developing cerebral vascular malformations and pulmonary arteriovenous fistulae. We assessed the risk of neurological dysfunction from these malformations and fistulae. METHODS Three hundred twenty-one consecutive

Diffuse capillary telangiectasia of the brain manifested as a slowly progressive course.

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Brain capillary telangiectasia (BCT) are usually small, solitary, benign in clinical manifestation, or found incidentally at autopsy. However, diffuse BCT are rarely reported. A 39-year-old woman had the first generalized seizure 10 years previously. Thereafter, she had sustained progressive spastic

Endometrial ablation for severe menorrhagia in a patient with hereditary hemorrhagic Telangiectasia. A case report.

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BACKGROUND Hereditary hemorrhagic telangiectasia is a rare, inherited disease characterized by abnormal visceral and superficial blood vessel anastomoses. These telangiectasias predispose the patient to a lifelong history of recurrent bleeding for the nasal and gastrointestinal mucosa. Less commonly

Unusual giant cerebral venous varix associated with brain abscess: variant of hereditary hemorrhagic telangiectasia--case report.

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A 35-year-old man suffered secondary generalized tonic-clonic convulsions due to a large brain abscess. Neuroimaging incidentally revealed another tumor-like lesion. Cerebral angiography confirmed that the lesion was an unusual giant venous varix associated with a high-flow pial arteriovenous

Natural history of brain capillary vascular malformations in hereditary hemorrhagic telangiectasia patients.

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OBJECTIVE Brain capillary vascular malformations (CVMs) are known to occur with relatively high frequency in hereditary hemorrhagic telangiectasia (HHT) patients. These lesions are thought to have a benign natural history but this has not been systematically studied. The purpose of our study was to

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