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)

A Blalock-Taussig-anastomosis was performed at the age of 2 years in a boy with transposition of the great arteries, ventricular septal defect, and pulmonary atresia. Nine years later he developed a transient aphasia. Cranial computed tomography (CT-scan) revealed a structure compatible with brain abscess. The boy was conscious and no neurological deficit was found. Initial therapy consisted of Ampicillin, Tobramycin, and Metronidazole, 12 days later an acute hemiplegia developed. 30 ml of pus were aspirated from the brain abscess, and the boy's condition dramatically improved. Streptococcus milleri was found bacteriologically and antibiotic therapy was continued over six weeks. Serial CT-scans during and after therapy demonstrated disappearance of the brain abscess. The presented case shows that after a shunt procedure in cyanotic heart disease right-to-left-shunting and therefore chronic oxygen desaturation and polycythemia are still present as facilitating factors for focal encephalomalacia, cerebritis, and brain abscess. In case of short duration of neurological symptoms and a size of abscess less than 4 cm in diameter antibiotic therapy without total excision may eliminate the infection.
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PMID:[Successful treatment of a brain abscess with antibiotics and drainage puncture in an 11-year-old boy with a complex cyanotic heart defect]. 358 39

A 40-year-old woman with a significant neurological history presented with right hemiparesia, paraesthesia of the right upper member and of the hemiface. Computer tomography scanning revealed hypodensity along the right lateral ventricle which corresponded to the left hemiplegia which had developed when she was 20 years old. Arteriography of the four cervical axes was normal. Echocardiography visualized an aneurysm of the membraneous septum free of thrombosis without ventricular septal defect. The embolism was thought to be of cardiac origin and a decision was taken to correct it by surgery. Ten years after surgical repair no other neurological event has occurred.
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PMID:Aneurysm of the interventricular membranous septum with thrombo-embolism--an indication for surgical repair? 813 73

We described a profoundly intellectually disabled 24-year-old man with Wolf-Hirschhorn syndrome, left hemiplegia, epilepsy, atrophy of the right cerebral hemisphere, and dilatation of the right ventricle. The patient had a small ventricular septal defect, was wheelchair bound, and totally dependent. He had no speech, but vocalised to show his feelings. In this patient, the del(4)(p15) was subtle and arose due to the inheritance of a recombinant chromosome (4) from a maternal pericentric inversion-46,XX,inv(4) (p15.32q35). Fluorescence in situ hybridisation with probe D4S96 confirmed the deletion. This is the second case of Wolf-Hirschhorn syndrome resulting from a large pericentric inversion of chromosome 4.
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PMID:The Wolf-Hirschhorn syndrome in adulthood: evaluation of a 24-year-old man with a rec(4) chromosome. 891 3

Two hundred adults who underwent surgery for congenital heart disease at our institution within a four year period were the basis for this report. Clinical data was obtained, i.e. demographic details, past medical history, physical findings, EKGs, echocardiograms, catheterization and angiography material as well as the New York Heart Association (NYHA) class. Intraoperative findings, perioperative management, complications and morbidity and mortality were assessed. After discharge the patients were followed on an outpatient basis. Again the clinical findings, laboratory results and NYHA class were recorded. Age ranged from 16 to 75 years (mean = 38). There were 114 females and 86 males. 178 patients had primary surgical correction, 18 were reoperated after prior correction and 4 underwent palliation. Eighty-three percent of the patients were symptomatic preoperatively. Seventeen percent were diagnosed per chance, for example by a preemployment physical examination, without a prior history of heart disease. The patients were grouped according to related diagnoses (Table 1). Among the 130 patients with left to right shunts, 112 had an atrial septal defect, 7 a ventricular septal defect, 5 a persistent ductus arteriosus, and 6 partial anomalous pulmonary venous return. Atrial flutter or fibrillation occurred in approximately 10% of all patients with atrial septal defects. It was terminated either by rapid overdrive pacing or DC cardioversion. Postoperatively the patients with pulmonary hypertension were monitored invasively with arterial lines and thermodilution catheters. Therapy consisted of alkalization, hyperventilation and sedation. There was only one postoperative death in this patient group due to marked pulmonary hypertension (1/130 = 0.8%). Nineteen adults had obstruction to right or left ventricular outflow. Surgery included valvotomy, infundibulectomy and valve replacement by homograft or mechanical valve. One patient with multiple previous surgeries expired due to bleeding (3%). Thirteen patients had coarctation. All of them were hypertensive, some on medication. Surgery consisted of aortic patchplasty or interposition of a graft. There was no mortality. Perioperative antihypertensive therapy was necessary in most patients and consisted of nifedipin, nitroprussid or propanolol intravenously. Upon follow up 11 patients became normotensive, 8 of these without the need for medication. Fifteen cyanotic patients underwent 11 corrective and 4 shunt procedures (3 with tricuspid atresia, 10 with Tetralogy of Fallot and 2 with complex cyanotic heart disease. Three died due to low cardiac output or dysrhythmias (20%). The survivors improved their clinical status markedly. Seven adults with Ebstein's disease had valve reconstruction and/or ASD closure. Five had recurrent supraventricular tachycardia, 2 paradoxical emboli with neurological symptoms and 4 out of 7 had decreased exercise tolerance. One patient died postoperatively because of dysrhythmias (14%). Sixteen patients had a variety of defects, i.e. status post Rastelli operation and conduit obstruction, status post Tetralogy of Fallot with pulmonary valvar disease, corrected transposition with left AV valve insufficiency, congenital mitral valve disease and double aortic arch, no deaths. The overall operative mortality was 6/200 = 3%. the late mortality was 4/200 = 2%. The morbidity included 7 reoperations due to bleeding. Five patients needed short-term hemodialysis. One patient developed hemiplegia and two patients had permanent decrease of their left ventricular function. The mean length of follow up was 21 months. The clinical status improved from a NYHA class mean of 2.1 +/- 0.9 to 1.2 +/- 0.45 (p < 0.001). In Germany significant numbers of adults with operated and unoperated congenital heart disease do exist. Detection of these patients can be difficult due to inconspicuous murmurs or stable clinical status.
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PMID:[Adults with congenital heart defects--clinical spectrum and surgical management]. 901 42

A 63-year-old-man with significant left hemiplegia was admitted to hospital. He had experienced a transient cerebral ischemic attack 10 years ago. Computed tomography revealed hypodensity along the right lateral ventricle, which corresponded to the left paralysis. Echocardiography and left ventricular angiography revealed an aneurysm of the membranous septum (AMS) without a ventricular septal defect (VSD). Therefore, the embolism was thought to be of cardiac origin, but surgery revealed that it was not caused by AMS. The aneurysm was created when the septal leaflet of tricupid valve formed a giant capsule during the process of natural closure of the VSD. It was a large pouch, 2.0 cm in diameter, adjacent to the septal leaflet. Anomalies of the tricuspid valve, including pouches, can resemble AMS.
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PMID:Tricuspid pouch can cause systemic embolization in adulthood. 1663 2

Incontinentia pigmenti is a systemic disorder affecting the skin, teeth, eyes, nervous tissue, hair, nails, musculoskeletal system, and heart. We describe an 11-month-old girl with incontinentia pigmenti associated with a ventricular septal defect, left hemiatrophy, hemangiomas, an abnormal labial frenum, and spastic cerebral palsy manifested as left hemiplegia and developmental delay. We believe this patient illustrates that incontinentia pigmenti is a systemic disorder necessitating a multidisciplinary approach to management.
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PMID:A case of incontinentia pigmenti associated with multiorgan abnormalities. 2054 58