Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Idiopathic dilated cardiomyopathy complicated with brain cardioembolism is rarely documented by both 2-dimensional echocardiography and cranial computed tomography in pediatric patients. A 2-year-old girl developed hemiparalysis 15 months after diagnosis of idiopathic dilated cardiomyopathy. The 2-dimensional echocardiograms of left ventricular thrombi, computed tomographic findings of brain embolism, clinical course, treatment and outcome are presented. Previous reports of idiopathic dilated cardiomyopathy in children are reviewed.
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PMID:Idiopathic dilated cardiomyopathy complicated with brain embolism: report of one case. 177 61

Dilated cardiomyopathy (DCM) is defined as a syndrome of dilated ventricles with gross impairment of ventricular systolic function. However, few reports on perioperative management of DCM were obtainable. This paper describes perioperative management of two patients with DCM. A 72-year-old man, whose DCM had been treated with medication, was planned for gastrectomy. His cardiac signs indicated NYHA class II. Cardiac function, evaluated prior to the surgery with echocardiography to determine an operative indication, turned out to be well compensated. Minimal cardiac derangements were anticipated perioperatively. The procedure was carried out under general anesthesia with neuroleptanalgesia utilizing butorphanol and vasoactive agents. No circulatory complications were observed throughout the surgery. A 66-year-old man, who had long-standing heart disease, hemiplegia and hydronephrosis due to ureteral stone, was planned for percutaneous nephrolithotomy. However, the planned surgery was withdrawn because he had DCM with minimal compensatory function and perioperative cardiac derangements were anticipated according to the categorized data classified with echocardiography. We conclude that preoperative assessment of cardiac function is essential to minimize perioperative cardiac derangements in patients with DCM.
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PMID:[Perioperative management of patients with dilated cardiomyopathy]. 273 70

Eight cases of intracardiac thrombi in infants and children were compiled in a cooperative study involving five paediatric cardiological centres. Two babies were hospitalised for cardiac failure due to a severe supraventricular arrhythmia. Two-dimensional echocardiography (2D echo) showed a left atrial thrombus which disappeared after anticoagulant therapy. The third case was unusual: 2D echo performed 4 months after a Senning operation for complete transposition of the great arteries showed stenosis of the pulmonary venous canal and a thrombus above the stenosis: the mass was echogenic, rounded, of variable density and in contact with the pulmonary veins. These findings were confirmed at autopsy. The fourth case was a 34 month old child with Fallot's triad in whom 2D echo showed a right ventricular thrombus, confirmed at surgery. The four remaining cases were thrombi detected in patients with congestive cardiomyopathy. The thrombus was adherent to the left ventricular lateral wall or apex. Two of these thrombi disappeared after anticoagulant therapy, one of which after hemiplegia. 2D echo is a useful tool for the diagnosis and surveillance of intraatrial or intraventricular thrombi. Intraatrial thrombi may be due to supraventricular arrhythmias in children; intraventricular thrombi are usually seen in association with poor left ventricular contractility. The diagnosis of thrombosis should lead to institution of anticoagulant or even fibrinolytic therapy in order to avoid systemic embolism.
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PMID:[2-dimensional echocardiographic diagnosis of an intracardiac thrombus in children]. 642 46

Treatment with recombinant tissue plasminogen activator (rt-PA) has been applied in acute cardioembolic stroke to reopen the occluded vessel and improve the patient's neurologic deficit. However, the effect of this therapy on intracardiac thrombus has not been documented previously. A forty-five-year-old man with dilated cardiomyopathy developed acute cardioembolic stroke with disturbance of consciousness, right hemianopia, right hemiplegia, and global aphasia. Cerebral angiography demonstrated occlusion of the left middle cerebral artery trunk. Intravenous administration of 30 megaunits (MU) of recombinant tissue plasminogen activator was commenced two hours after the ictus and completed within sixty minutes. Cerebral angiography was repeated just after this treatment and demonstrated a new occlusion of the left intracranial internal carotid artery along with occlusion of a branch of the left external artery. The authors subsequently performed two-dimensional echocardiography and found a mobile thrombus in the left ventricle. In patients with intracardiac mobile thrombi, recombinant tissue plasminogen activator seems to accelerate breakup or detachment of the thrombi and subsequent recurrent embolization. Therefore, it seems better to pay attention to the presence of mobile intracardiac thrombus before commencing intravenous infusion of rt-PA.
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PMID:Recurrent embolization during intravenous administration of tissue plasminogen activator in acute cardioembolic stroke. A case report. 820 76

Emery-Dreifuss muscular dystrophy (EDMD) is a muscular disorder characterized by 1) early contracture of the elbows. Achilles tendons and post-cervical muscles, 2) slowly progressive muscle wasting and weakness with a humeroperoneal distribution, and 3) life-threatening cardiomyopathy with conduction block. Most of families with EDMD show X-linked recessive inheritance with mutations in the STA gene on chromosome Xq28, which encodes a protein named emerin. A rare autosomal dominant form of EDMD (AD-EDMD) is caused by mutations in lamin A/C gene (LMNA) on chromosome 1q21. Both emerin and lamin A/C are located in the inner surface membrane of the nucleus. A 49-year-old woman was skinny and slow runner from childhood and suspected as having a certain muscular disorder. At 35 years, she was found to have the second degree atrioventricular block. At 45 years, she was admitted to a hospital for right-side hemiplegia after cerebral infarction. Cardiac involvement was also observed including high degree atrioventricular block with chronic atrial fibrillation and frequent paroxysmal ventricular contraction on the electrocardiogram. At 49 years, she was referred to our hospital for further evaluation. She had possible dilated cardiomyopathy with conduction block. She also had muscular atrophy and weakness in all extremities, predominantly in the right-side, and contracture of bilateral Achilles tendon, knee and elbow joints, and postcervical muscles. Biopsied skeletal muscle and electromyogram showed myopathic changes. Since a novel point mutation of Ser303Pro was found in exon 5 of LMNA gene, she was diagnosed as having AD-EDMD and had a permanent pacemaker implantation. Her daughter also had some abnormalities on electrocardiogram. This is the first Japanese case of AD-EDMD. Amiodaron was effective for non-sustained ventricular tachycardia. Early diagnosis and following cardiological examinations and treatments are important and necessary to improve the prognosis of the patients with EDMD.
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PMID:[The first Japanese case of autosomal dominant Emery-Dreifuss muscular dystrophy with a novel mutation in the lamin A/C gene]. 1242 64

We report two cases of Duchenne muscular dystrophy (DMD) complicated with dilated cardiomyopathy (DCM), who were affected with cerebral infarction. Case 1 suddenly developed dysarthria and right facial weakness at age 21. Cranial CT study disclosed a low density area in the left basal ganglia and internal capsule. Case 2 had a history of transient ischemic attack (TIA) at age 21. Five months after the TIA, he developed right hemiplegia and dysarthria, and a low density area in the corona radiate in left cerebral hemisphere was observed in cranial CT. These two cases showed the radiographic cardiomegaly with cardio thoracic ratio (CTR) of 72.8% and 66.6%, the decreased echocardiographic left ventricular ejection fraction below 20%, and the elevated titer of thrombin-anti-thrombin III complex (TAT) and D-dimer. The autopsy of Case 2 at age 26 disclosed the remarkable degeneration and fibrosis of myocardium and old ischemic lesion in the left cerebral frontal cortex. Despite the negative finding of the emboli in the left heart, cardiogenic cerebral infarction secondary to DCM was strongly suspected in both cases.
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PMID:[Two cases of Duchenne muscular dystrophy complicated with dilated cardiomyopathy and cerebral infarction]. 1662 52