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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 54 year-old man, who had a hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease; O-W-R) accompanied by pulmonary arteriovenous fistulas (PAVFs) and congestive heart failure, developed seizure, right hemiparesis and dysphasia. A brain CT scan revealed a cystic lesion with perifocal edema in left frontoparietal lobe. A contrast enhanced CT scan showed a ring-like enhancement. Dynamic CT scans disclosed that the ring in the cortical side was enhanced more thickly than that in the ventricular side. Considering the severity of the cardio-pulmonary condition, and the deep location of the abscess, we performed an echo-guided aspiration and drainage of the abscess under local anesthesia. No bacteria were demonstrated in the culture of the contents of the abscess. After the surgery, the right hemiparesis and dysphasia were much improved and a CT scan showed the marked reduction of the abscess. However, around eight days after the surgery, the patient showed severe pleural effusion due to progressive heart failure and died on the 11th postoperative day. Autopsy disclosed a shrunken brain abscess, multiple cerebral infarction, multiple PAVFs and severe constrictive pericarditis which was regarded as the cause of death in the patient. In this report, we presented the therapeutic advantage of echo-guided surgery for the treatment of brain abscess in a high-risk patient. We also discussed the mechanism of the formation of brain abscess in patients of O-W-R disease by reviewing published cases.
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PMID:[A case of Osler-Weber-Rendu disease with brain abscess; the mechanism of the formation of brain abscess and its treatment in Osler-Weber-Rendu disease]. 194 83

In a retrospective serie the results of 72 cerebral vascular malformations, operated on consecutively were reviewed: 57 AV-malformations (in 55 patients), 1 dura AV-malformation, 9 cavernomas, 1 telangiectasia, 2 venous angiomas and 2 mixed malformations. The most frequent initial symptoms were headache, epileptic seizures and intracranial bleeding. In 5 cases the initial symptom was loss of consciousness with coma. A total resection could be proved angiographically in all cases. Mortality was 0%, neurological morbidity, i.e., new deficits or increase of existing deficits, was 8.6%. There were no recurrences of bleeding in the postoperative period. Quality of life, as assessed by the Karnofsky Index 6 months postoperatively, was satisfactory, approximately 80% of all patients being back at work. In this group of selectively operated patients it could be shown that localization of the malformation in or close to an eloquent brain area (n = 32) is rarely associated with significant persisting new deficits. Taking into consideration the natural history of AV malformations, with an 8% morbidity and 2% mortality each year, as compared to the surgical results, it is concluded that surgery may be more often indicated for AV malformations than was previously thought, even when they are near eloquent brain areas and relatively large in diameter.
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PMID:[Neurologic findings before and after removal of cerebrovascular abnormalities]. 203 13

A case of hemihypertrophy associated with multiple anomalies of the skin, bone and visceral organs is presented. A 31-year-old female was admitted for evaluation of her skin conditions. Her family history is noncontributory, while her past history discloses operations for syndactyly of the right foot, tonsillar hypertrophy, anal prolapse and ovarial cyst. Erythemas of the face and the left upper extremity were noticed during the neonatal period and hypertrophy of the right side of the body started at age 2 months. On admission, hemihypertrophy was observed in the face, trunk and extremities. Multiple faint nevi flammei were seen on the right half of the face and on the left side of the trunk and extremities. Telangiectasis and nevus anemicus were seen in the upper chest. The left upper extremity showed diffuse brown patches that was histologically basal pigmentation with some giant melanosomes. Visceral anomalies consisted of fibromatous tumors of the tip of the tongue and mitral prolapse. Angiography and computed tomography revealed a possible arteriovenous malformation of the right occipital region, small hemangiomas around the patella, dilation of the lateral ventricle, and calcification of the choroid plexus. Tortuous superficial veins were noted in the right leg. She had no seizure, but her IQ was 68. The bone disorders consisted of scoliosis, short forth metacarpus, hypoplastic mandible and peroneal exostosis. Examination revealed a slight diminution of urinary corticosteroid, but no other endocrinological disorders were found. The hemihypertrophy in this case is at least partially due to an arteriovenous shunt, suggested by elevated oxygen saturation of the blood obtained from the internal saphenous vein.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Congenital hemihypertrophy associated with cutaneous pigmento-vascular, cerebral, visceral and bone abnormalities]. 282 Feb 92

The clinical, radiological, and histopathological features of 21 cases of angiographically occult intracranial vascular malformations (AOIVM's) are analyzed, and a review of 241 additional appropriately documented, histologically verified cases collected from the literature is presented. In all, there were 115 (43.8%) arteriovenous malformations, 82 (31.2%) cavernous angiomas, 26 (9.9%) venous angiomas, 10 (3.8%) cases of capillary telangiectasis, and 29 (11%) mixed or unclassified angiomas. The result of the analysis shows that there are no essential differences in the patterns of clinical presentation, the computerized tomography (CT) appearance, or the surgical prognosis among these pathological types of vascular malformations. Certain histological features common to all AOIVM's (such as the small caliber, the more or less complete thrombosis of the malformed vessels, and the changes induced in the surrounding brain tissue by repeated microhemorrhages) seem to determine the biological behavior of the anomaly rather than the predominant type of vessel involved. Thus, subdivision of AOIVM's into the four classical pathological types has little practical value. Most AOIVM's are visualized by the CT scan and show a rather typical appearance. Surgical removal, which prevents rebleeding and ameliorates or suppresses seizure activity, is usually easy to perform and represents the treatment of choice for patients with clinically symptomatic AOIVM's.
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PMID:Clinical, radiological, and pathological spectrum of angiographically occult intracranial vascular malformations. Analysis of 21 cases and review of the literature. 328 Jul 47

The clinical courses of 31 episodes of brain abscess and one episode of meningitis occurring in patients with hereditary hemorrhagic telangiectasia are reviewed. Pulmonary arteriovenous malformations were demonstrable in all but two patients and presumably permitted septic microemboli to evade the normal pulmonary capillary filter and lodge in the brain. Obtundation, headache, visual disturbances, hemiplegia, and seizures were the most common presenting features. Cyanosis, clubbing, polycythemia, and hypoxemia were routinely encountered, but leukocytosis and fever were present in a minority of cases, and all blood cultures were sterile. Anaerobic and microaerophilic streptococci were the commonest pathogens found in the brain abscesses. Thirteen patients died, and patients without abscess drainage or with delayed diagnosis had a higher mortality rate. A brain abscess may develop in approximately 1 percent of patients with hereditary hemorrhagic telangiectasia, and awareness of this risk should lead to early investigation of any patient with hereditary hemorrhagic telangiectasia who has neurologic symptoms.
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PMID:Central nervous system infections associated with hereditary hemorrhagic telangiectasia. 637 93

Presented case is a 27-year-old male with remarkable enlargement of an arteriovenous malformation (AVM) in the left frontal lobe, four years after subtotal removal operation. First operation was carried out in February, 1973, because of 5 times adversive seizure to the right. Post-operative angiogram disclosed a tiny telangectatic residual AVM. Four years later of first operation, follow-up angiography showed a remarkable enlargement of AVM. The second operation was performed in March, 1979, with angiographically complete disappearance of AVM. A lot of theory for enlargement of AVM have been presented. Author summarized possible factor's, which influence enlargement of the size of AVM, as follows (Table 3). The first of all, histopathological nature of AVM with hemodynamic factor is most important. And, second important factor is a existence of space of adjacent AVM and multiple telangiectasia of the internal and external on nidus of AVM. At last, biological factors, such as a autonomic growth with individual ageing growth, and a size of AVM, should be regarded. It is author's conclusion, the growth of AVM of our case has occurred in the dead-space originated by previous operation, that continuous expansion of residual small vessels (telangiectasia) in vicinity of the operated region. Therefore, authors emphasizes that at the first time, radical removal of AVM should be operated with meticulous care of existent telangiectatic small vessels in surrounding brain tissue of AVM complex.
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PMID:[A case of remarkable enlargement of cerebral arteriovenous malformation after operation -special reference to their enlargement- (author's transl)]. 724 89

Three children with hair-shaft abnormalities and neurological disorders are reported. The first case is a young girl with monilethrix and Friedreich's ataxia that presented IgG and IgA deficiency, without telangiectasis. The second patient is a young girl with monilethrix and HMSN I. The third patient is a boy with a peculiar face, hypotrichosis and pili torti; he was mentally retarded and had epileptic seizures. His diagnosis was compatible with Noonan's syndrome or with a cardio-facio-cutaneous syndrome, because of the existence of hyperkeratosis and the neurological features. It is our aim to emphasize the heterogeneity of neurotrichosis, whose limits are not well established and whose manifestations are shared by some of them.
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PMID:[Hair disorders and neurological impairments (neurotricosis): three new cases]. 749 1

We present four cases of pial arteriovenous fistula (AVF) in children as the presenting manifestation of Rendu-Osler-Weber disease (ROW). The common clinical manifestations of ROW in adults, such as skin telangiectasia and mucosal haemorrhagic complications, seldom occur in children, since telangiectases develop with age. Pial AVF in ROW also conform to the usual age incidence and are therefore present in childhood. Of the four children in this series, three had multiple AVF. Two presented with central nervous system haemorrhage, one with seizures and the other with progressive neurological deficit. There were no clinical or angioarchitectural differences between the AVF associated with ROW and sporadic AVF. The diagnosis was based in all cases on the family history. Transarterial embolisation to obliterate the AVF was carried out in all patients. One patient had early rebleeding after partial embolisation of the AVF, with a fatal outcome. Three patients were cured and one asymptomatic in long-term follow up. No exhaustive search was conducted for multiorgan telangiectases, since there is no indication for treatment of asymptomatic telangiectasia in ROW. No pulmonary fistulae were found. ROW should be suspected in children with multiple pial AVF; they may be the only manifestation of the disease, since epistaxis and telangiectasia are unusual in early life.
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PMID:Pial arteriovenous fistula in children as presenting manifestation of Rendu-Osler-Weber disease. 770 92

Between 1988-1993 we performed CT-stereotactic guided microsurgical resection as a one-session-procedure in 46 patients bearing small (< 30 mm) cerebral vascular malformations (SCVM). The location of the SCVM was deep subcortical in 38 patients, temporal medio- basal in 3 and brainstem in 5. The surgical technique intended to minimise invasiveness by reducing the operative approach to a size less than the diameter of the lesion concerned. The mean diameter of our SCVM's was 20 mm ranging from 10 to 30 mm. Histologically we found 23 arteriovenous malformations, 22 cavernous malformations and 1 capillary telangiectasia (capillary malformation). Clinical symptomatology consisted mainly of seizures, (progressive) neurological deficit and (minute) acute intracerebral bleeding. The SCVMs could be demonstrated by contrast-enhanced CT as well as by MRI. 15 of the AVM's revealed as angiographically occult. Complete microsurgical resection of the SCVM was accomplished in all cases with a surgical morbidity of 6.5% and no operative mortality. In 14 patients, most of them with initial acute intracerebral haemorrhage, the pronounced focal neurological deficit improved. During the follow-up period (1/2-5 years) no rebleeding occurred. As far as epileptic seizures were concerned 13 patients became seizure-free without anticonvulsants and 11 patients seizure-free with anticonvulsant, in the remaining 4 patients seizures were reduced in frequency.
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PMID:Stereotactic microresection of small cerebral vascular malformations (SCVM). 772 39

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 with multiple AVMs associated with AVF. Seizures were the most common presenting symptom (seen in three patients), and two of them had intracerebral hematomas (ICH). In the remainder, the malformations were incidentally found in the course of evaluation of other diseases. Their locations were variable, but the majority was superficially confined to the cerebral cortex. Arterial supply was from mostly one feeding artery that was a cortical branch of either anterior, middle, or posterior cerebral artery. In six of nine malformations, the venous drainage was through a superficial cerebral vein into either the superior sagittal sinus or transverse sinus. Direct surgery was done on two patients with ICH, artificial embolization on one, and stereotactic radiosurgery on one. The cerebral vascular malformations in HHT are not infrequent, and in particular the importance of computed tomography and cerebral angiography should be recognized in patients with pulmonary AVF associated with HHT.
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PMID:Vascular malformations of the brain in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease). 800 11


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