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

Between 1960 and 1991, 156 episodes of central nervous system (CNS) bleeding were documented in 106 patients from a total population of 1,410 hemophiliacs (7.5%). Ninety-one hemophilia A patients presented 131 bleeding episodes; 15 hemophilia B patients had 25 episodes. 32% of these episodes took place in patients less than 5 years of age. 46% were age 10 or less, and 72% were age 20 or less. The mean age was 14.8 years in hemophilia A and 9 years in hemophilia B patients. A significant increase in the mean age of hemophilia A patients has been observed over the last 10 years; this may be related to HIV infection. A history of recent trauma was documented in 39.7% of the episodes. Spontaneous CNS bleeding was predominant in severe hemophilia (85.2%). One hundred and fifty-four CNS bleeding episodes were intracranial and 2 intraspinal. Of the intracranial episodes, 37.7% were subarachnoid, 29.8 subdural, and 22.7% intracerebral. Factor VIII or IX inhibitors were present in 11.3% of the patients; this figure is slightly lower than that observed in our total hemophilic population. Over 50% of the patients had psychoneurological sequelae; the most frequent were seizure disorders and motor impairment. The overall mortality rate was 29.2%. The mortality was more closely related to the CNS bleeding site than to the severity of hemophilia. Treatment should be based on prompt and prolonged replacement therapy to ensure hemostatic levels of antihemophilia factors.
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PMID:Update of 156 episodes of central nervous system bleeding in hemophiliacs. 147 37

The intracranial haemorrhages presented within a group of 64 haemophiliacs along 25 years were revised. During this period, 10 such episodes were seen in 8 patients with haemophilia A; six of them appeared in children under 10 years of age. In five instances there were traumatic antecedents, whereas in the remaining five the haemorrhage was spontaneous, no vascular abnormalities being demonstrated in these last. The lesions observed were: intracerebral haematoma in three instances, subdural haematoma in four, subarachnoid haemorrhage in two cases and cerebellar haematoma in one case. Only one of the episodes was lethal, death being related with infectious complications. Recurrences were observed in two patients. Two patients had sequelae with seizures, plus mental retardation and motor deficit in one of them. Substitutive therapy shows capable of controlling this severe complication of haemophilia, provided it is started promptly and fair rates of factor VIII are maintained. Such therapy must be kept for longer periods in these patients with traumatic antecedents.
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PMID:[Intracranial hemorrhage in hemophiliacs. Study of 10 episodes]. 158 36

The effectiveness of low-dose intrauterine irradiation for benign diseases and its possible carcinogenic effect on the uterus was studied in 190 patients who were treated during the years 1952-1974. The indications for irradiation were premenopausal functional bleeding, leukemia, hemophilia, fibroids, endometriosis or other benign reason. Radiation was also performed on patients with severe neurologic diseases that contraindicated surgery and on some mentally retarded patients whose restlessness and epileptic seizures were aggravated premenstrually and during menstruation. The mean follow-up period was 15 years. Uterine bleeding recurred in 21% of the patients. No cases of uterine malignant degeneration were found.
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PMID:Uterine malignant degeneration after low- dose endometrial irradiation. 610 64

A patient is reported in whom a meningioma of the lateral one-third of the sphenoid ridge was completely removed and long-term prophylaxis for seizures with diphenylhydantoin was prescribed. One and a half years later, a powerful inhibitor developed that specifically neutralized Factor VIII, the antihemophilic factor, and caused an acquired state of hemophilia. The 4-month hemorrhagic disorder was characterized initially by painless hematuria and later by intracerebral and extradural hematomas at the operative site of the previously excised meningioma. Despite the transfusion of massive quantities of concentrates of clotting factors, and the surgical evacuation of the recurrent hematomas on two occasions, the localized bleeding could not be staunched and the patient died. The types of inhibitors that cause acquired hemophilia and their modes of treatment are examined. Although it is possible that the Factor VIII inhibitor in this patient was induced by the meningioma, most previously reported tumors associated with acquired hemophilia have had an immunological basis. The most probable explanation for the acquired hemophilia in this patient was an inhibitor to Factor VIII from an autoantibody induced by the long-term use of diphenylhydantoin.
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PMID:Acquired hemophilia, meningioma, and diphenylhydantoin therapy. 677 40

Long-term intermittent venous access was established in 77 children by means of a central venous catheter (CVC) with a subcutaneous injection port (Port-A-Cath; PAC). Seventy of these children were included in this follow-up study. Sixty-three were treated for different malignant diseases, five for cystic fibrosis, one for severe hemophilia and one for central nervous system disease with seizures as the main problem. As of April, 1992, PACs had been in place for 3/12 to 8 3/12 years (cumulative 175 5/12 years) with 2,206 entries into the system. The PACs were used for blood sampling and administration of chemotherapy, antibiotics, fluids, total parenteral nutrition (TPN) and blood products. Portal infection was observed in four patients of which two patients had their PAC removed. Catheter dislocation was observed in two and catheter breakage in one. Portal occlusion, extravasation, thrombosis leading to removal of the PAC or other technical or psychological complications were not observed. The children continued normal activities, and the easy venous access decreased emotional stress during treatment. Local doctors were trained to use PACs, through which they administered maintenance chemotherapy. We conclude that long-time use of PACs in children is safe and has many advantages compared to traditional CVCs in use. Strict indications, meticulous implantation techniques and adequate handling are, however, mandatory.
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PMID:Central venous catheter with subcutaneous injection port (Port-A-Cath): 8 years clinical follow up with children. 821 38

Intracranial hemorrhage occurs in 2-8% of patients with hemophilia and can result in neurologic sequelae including seizure disorders. There is a paucity of data concerning the surgical treatment of epilepsy in children with hemophilia. We review our experience with 2 children who developed medically refractory seizure disorders. Both children underwent hemispherotomy, at 18 months (case 1) and 13 years of age (case 2), respectively. Perioperative management included continuous factor replacement. Both children tolerated surgical intervention without complications or increased neurologic deficit. Case 2 showed a 90% reduction in seizure frequency, and case 1 is seizure free. Surgical management of intractable epilepsy in children with hemophilia is safe and effective.
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PMID:Surgical management of intractable epilepsy in children with hemophilia. 1085 8

Factor XIII (FXIII) deficiency is a rare autosomal recessive congenital disorder of haemostasis, associated with a high risk of intracranial haemorrhage. Intracranial haemorrhage can result in neurological sequelae including seizure disorders. In some cases, medically intractable epilepsy led to epilepsy surgery. Little has been reported on the management of FXIII deficiency during surgery, and there is only a few data on the management, safety and efficacy of epilepsy surgery in the patients with haemostatic disorder. We report here an epilepsy neurosurgery in a case of severe FXIII deficiency.
Haemophilia 2003 Jan
PMID:Surgery in severe factor XIII deficiency: report of a case of epilepsy neurosurgery and review. 1255 90

A middle-aged woman was admitted to the hospital after being found unconscious at home. A brain CT scan excluded an intracranial bleed or other focal abnormality. Laboratory analysis showed hyponatraemia (sodium: 121 mmol L(-1)) and a low plasma osmolality, with normal sodium excretion and urine osmolality. A diagnosis of hyponatraemic coma was made. The patient was treated with water restriction; 24 h later the sodium was 135 mmol L(-1) and the patient was neurologically fully recovered. The patient, who suffered from von Willebrand's disease, had received desmopressin and ibuprofen for analgesia 2 days before after a dental intervention. She had received desmopressin several times in the past without any complications. A few patients treated with desmopressin for coagulation abnormalities have been reported to develop water intoxication and severe hyponatraemia resulting in seizures and coma. By inhibiting prostaglandin synthesis, non-steroid anti-inflammatory agents (NSAIDs) potentiate the effect of water reabsorption in the renal tubules of vasopressin, therefore enhancing water retention. Desmopressin and NSAIDs should not be used in combination in patients with bleeding disorders, but it is often followed in clinical practice. In addition, this is probably not an unusual situation in patients treated with desmopressin for other 'non-haemorrhagic' indications. This report emphasizes the need for practitioners to be aware of this rare but severe complication.
Haemophilia 2003 Mar
PMID:Hyponatraemic coma induced by desmopressin and ibuprofen in a woman with von Willebrand's disease. 1261 77

We investigated 52 of 457 patients with congenital factor deficiencies with 57 episodes of intracranial haemorrhage (ICH) between 1998 and 2007. There were 38 severe haemophiliacs, 6 with factor XIII deficiency, 5 with factor X deficiency, 2 factor V-deficient patients, and 1 with type 3 von Willebrand disease (VWD). The median age was 8 years (range 1 month-22 years). Most patients were below 15 years of age (86.5%). All patients with factor X deficiency were between 1 and 5 months of age. ICH was the primary bleeding episode leading to detection of factor deficiency in 19.2% (five patients with severe haemophilia and all patients with factor X deficiency). Trauma caused bleeding in 66%. None of the patients with factor X deficiency had history of prior trauma. Surgery was performed in five patients with subdural haematomas, all of whom survived. Conservative factor replacement with 100% correction for 3 days followed by 50-60% correction for 7 days was possible in 60% patients. Seizures requiring prolonged therapy were noted in eight patients. Death was recorded in 15 patients (29%). Inadequate therapy in the form of delay or insufficient replacement was noted in 7/15 deaths. ICH was seen in 11.3% of all patients with coagulation factor deficiencies. Factor X deficiency presented with ICH at an earlier age. Inadequate replacement therapy including delayed treatment caused nearly 50% of all deaths. Most patients can be managed satisfactorily with adequate replacement therapy alone, with surgery being reserved for those with worsening neurological conditions.
Haemophilia 2008 Sep
PMID:Intracranial haemorrhage in patients with congenital haemostatic defects. 1863 45

Central nervous system (CNS) bleeding is one of the most severe and debilitating manifestations occurring in patients with rare bleeding disorders (RBDs). The aim of this study was to retrospectively collect data on patients affected with RBDs who had CNS bleeding, to establish incidence of recurrence, death rate, neurological sequences, most frequent location, type of bleeding and efficacy of treatments. Results pertained to 36 CNS bleeding episodes in 24 patients with severe deficiency except one with moderate factor VII (FVII) deficiency. Six patients (25%) experienced a recurrence and two had more than one recurrence. Seven patients (29%) had an early onset of CNS bleeding before the first 2 years of life, others (71%) later in life. In 76% of cases, CNS bleeding was spontaneous. CNS bleeding was intracerebral in 19 cases (53%), extracerebral in 10 (28%) and both intracerebral and extracerebral in two cases (6%). Neurosurgery was performed in 11 cases, in association with replacement therapy in seven cases. Seizures were noted in four patients. Residual psychomotor abnormalities were seen in two patients. No death was recorded. To prevent recurrence, 17/24 patients (71%) were put on secondary prophylaxis. In conclusion, recurrence of CNS bleeding was confirmed to be relatively frequent in patients with severe FV, FX, FVII and FXIII deficiencies. Most patients were managed with replacement therapy alone, surgery being reserved for those with worsening neurological conditions. Our results indicate that some RBDs require early prophylactic treatment to prevent CNS bleeding. Optimal dosage and frequency of treatment need further evaluation.
Haemophilia 2012 Jan
PMID:Central nervous system bleeding in patients with rare bleeding disorders. 2153 94


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