Cases reported "Disease Progression"

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1/151. Translocation (4;15)(p16;q24): a novel reciprocal translocation in a patient with BCR/ABL negative myeloproliferative syndrome progressing to blastic phase.

    A patient with BCR/ABL negative myeloproliferative syndrome with a 46,XY,del(3)(q21), t(4;15)(p16;q24) karyotype is described. fluorescence in situ hybridization performed with chromosomes 4 and 15 painting probes confirmed a novel reciprocal (4;15) translocation. The absence of crkl tyrosine phosphorylation, no activation of the abl kinase as measured by autophosphorylation, and a normal-size abl transcript suggest an alternative mechanism for leukemogenesis to that operative in Ph positive BCR/ABL positive chronic myeloid leukemia. A number of genes potentially relevant to tumorigenesis, some involving the ras signaling pathway, map to the 4p16 and 15q24 chromosome regions.
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2/151. epilepsy surgery in children with pervasive developmental disorder.

    Pervasive developmental disorder (PDD) is occasionally associated with medically intractable complex partial seizures. The outcome of PDD was explored in three males and two females who underwent epilepsy surgery at 32 months to 8 years of age (mean = 4 years) after onset of epilepsy at 1 week to 21 months of age (mean = 11 months). Four children had temporal lobe resections (three right, one left; two for focal cortical dysplasia, and two for tumors), and one had a right temporoparieto-occipital resection (for focal cortical dysplasia). Each child underwent repeated evaluations by a pediatric neuropsychologist and psychiatrist. Fourteen to 47 months (mean = 23 months) after operation, one child with persistent seizures had moderate developmental and behavioral improvement, three children (two seizure free, one with rare staring spells) had mild developmental and behavioral improvement, and the remaining child (seizure free) experienced a worsening of her PDD. The four children with mild-to-moderate improvement in postoperative cognitive and behavioral development still demonstrated persistent delay. Cognitive gains were confirmed by neuropsychologic testing in the oldest patient but were not reflected in test results from the three younger children, who had more modest improvement. The child with worsening of her PDD had cognitive and emotional deterioration to babbling, echolalia, aggressiveness, decreased social interaction, and increased mouthing of objects beginning several months postoperatively. These results suggest that families should be counseled that PDD symptoms in children with focal epileptogenic lesions may or may not improve after epilepsy surgery, even if the surgery is successful with respect to seizure control.
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keywords = operative
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3/151. Laminar and arch fractures with dural tear and nerve root entrapment in patients operated upon for thoracic and lumbar spine injuries.

    OBJECTIVE: To determine the neurological outcome in patients with laminar fractures associated with dural tears and nerve root entrapment, operated upon for thoracic and lumbar spine injuries. PATIENT population: Out of 103 patients operated upon consecutively for thoracic and lumbar spine injuries during the period 1990 to 1994 inclusive, 24 (23.3%) patients had laminar fractures out of whom 3 (2.9%) had an associated dural tear and an other 17 (16.5% or 70.8% of the total patients with laminar fractures) had an associated dural tear and nerve root entrapment. RESULTS: Twelve (70.5%) patients had injury at the thoraculumbar junction, 13 (76.5%) had Magerl's type A3 or above, 10 (58.8%) had a kyphotic angle deformity greater than 5 degrees. Seven (41.1%) had their spinal canal's sagittal diameter reduced by at least 50% and two had dislocations. Nine (52.9%) had initial neurological deficits. Four (50%) out of 8 patients with no initial neurological deficits (Frankel E) worsened to Frankel D. However, one patient among the 3 with initial Frankel A improved to Frankel C while both patients with initial Frankel C usefully improved to final Frankel grades D and E respectively. Two of the four patients with initial Frankel D improved to Frankel E, the other 2 remaining unchanged. All in all five patients neurological status improved, 4 worsened and 8 remained unchanged after neurosurgical treatment. CONCLUSIONS: Vertical laminar fractures with dural tears and nerve root entrapment represent a special group of thoracic and lumbar spine injuries that carry a poor prognosis. However, special operative precautions lead to significant improvement in some of them although a majority remain unchanged or even worsened.
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keywords = operative
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4/151. A single-stage operation for bicuspid aortic valve, annulo-aortic ectasia, hypoplastic aortic arch, and coarctation of the aorta: A case report.

    The patient was an 18-year-old man who had been diagnosed as having a bicuspid aortic valve and dilatation of the ascending aorta six years previously. As he grew up, aneurysmal change of the ascending aorta and hypertension in the upper body gradually progressed. Preoperative evaluation showed annulo-aortic ectasia and the following congenital abnormalities: bicuspid aortic valve, hypoplastic aortic arch, and coarctation of the aorta. Composite graft replacement and extended total aortic arch replacement were carried out.
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5/151. Spinal deformity after selective dorsal rhizotomy in patients with cerebral palsy.

    Selective dorsal rhizotomy is used widely as a means of treating spasticity associated with cerebral palsy. Little is known regarding the effect of the procedure on the development or progression of spinal deformity. The authors reviewed six patients with progressive deformity after rhizotomy. Prerhizotomy and postrhizotomy records of physical examinations and radiographs were reviewed retrospectively in an attempt to identify risk factors for development of and/or rapid progression of, spinal deformity. Detailed preoperative and postoperative evaluation of spinal alignment should be undertaken, particularly in those patients who may be at risk of rapidly progressive deformity.
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ranking = 2
keywords = operative
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6/151. Bilateral contemporaneous posteroventral pallidotomy for the treatment of Parkinson's disease: neuropsychological and neurological side effects. Report of four cases and review of the literature.

    The authors report the underestimated cognitive, mood, and behavioral complications in patients who have undergone bilateral contemporaneous pallidotomy, as seen in their early experience with functional neurosurgery for Parkinson's disease (PD) that is accompanied by severe motor fluctuations before pallidal stimulation. Four patients, not suffering from dementia, with advanced (Hoehn and Yahr Stages III-IV), medically untreatable PD featuring severe "on-off" fluctuations underwent bilateral contemporaneous posteroventral pallidotomy (PVP). All patients were evaluated according to the Core Assessment Program for Intracerebral Transplantations (CAPIT) protocol without positron emission tomography scans but with additional neuropsychological cognitive, mood, and behavior testing. For the first 3 to 6 months postoperatively, all patients showed a mean improvement of motor scores on the Unified Parkinson's disease Rating Scale (UPDRS), in the best "on" (21%) and worst "off" (40%) UPDRS III motor subscale, a mean 30% improvement in the UPDRS II activities of daily living (ADL) subscore, and 60% on the UPDRS IV complications of treatment subscale. Dyskinesia disappeared almost completely, and the mean daily duration of the off time was reduced by an average of 60%. Despite these good results in the CAPIT scores, one patient experienced a partially regressive corticobulbar syndrome with dysphagia, dysarthria, and increased drooling. No emotional lability was found in this patient, but he did demonstrate severe bilateral postoperative pretarsal blepharospasm (apraxia of eyelid opening), which interfered with walking and which required treatment with high-dose subcutaneous injections of botulinum toxin. No patient showed visual field defects or hemiparesis, but postoperative depression, changes in personality, behavior, and executive functions were seen in two individuals. Postoperative abulia was reported by the family of one patient, who lost his preoperative aggressiveness and drive in terms of ADL, speech, business, family life, and hobbies, and became more sleepy and fatigued. One patient reported postoperative mental automatisms, such as compulsive mental counting, and circular thoughts and reasoning during off phases; postoperative depression was found in two patients. However, none of the patients demonstrated these symptoms during intraoperative microelectrode stimulation. These findings are compatible with previous reports on bilateral pallidal lesions. A progressive lowering of UPDRS subscores was seen after 12 months, consistent with the progression of the disease. Bilateral simultaneous pallidotomy may be followed by emotional, behavioral, and cognitive deficits such as depression, obsessive-compulsive disorders, and loss of psychic autoactivation-abulia, as well as disabling corticobulbar dysfunction and apraxia of eyelid opening, in addition to previously described motor and visual field deficits, which make this surgery undesirable even though significant improvement in motor deficits can be achieved.
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ranking = 8
keywords = operative
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7/151. Left extended hemihepatectomy with preservation of large inferior right hepatic vein: a case report.

    For hepatic function to be preserved after an extended hemihepatectomy adequate venous drainage of the remaining liver is required. Most metastases close to the confluence of the superior hepatic veins are considered unresectable because hepatic venous outflow after resection would be compromised. In 10-25% of people, the inferior right hepatic vein is of large calibre. Thus the superior hepatic veins may be sacrificed and hepatic function preserved if a large inferior right hepatic vein is present. A patient with involvement of segments 2, 4 and 8 by metastatic colorectal cancer is presented. This patient had a large inferior right hepatic vein, and so was able to undergo an extended left hemihepatectomy with ligation of all superior hepatic veins. Subsequent quality of life was maintained. This case illustrates that an 'unresectable' hepatic lesion can be actually resectable if an alternative venous drainage is present. A pre-operative search for a prominent inferior right hepatic vein by ultrasound, computerised tomography, or even magnetic resonance imaging should be considered in these cases.
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ranking = 1
keywords = operative
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8/151. Functional recovery after bilateral pallidotomy for the treatment of early-onset primary generalized dystonia.

    This report describes the successful treatment of dystonia musculorum deformans with bilateral stereotactic pallidotomy in a 14-year-old girl in whom the dystonia was diagnosed when she was 7 years old. The patient presented with dystonia of the right upper extremity that progressed to generalized dystonia. Preoperatively, she required maximal assistance with all activities of daily living and transfers. She was not a functional ambulator. Postoperatively, she had remarkable functional recovery. At discharge, she was at modified independence level for all basic activities of daily living and required supervision for household ambulation. No postoperative complications were noted. We propose that bilateral stereotactic lysis of globus pallidus interna may be an alternative treatment for dystonia musculorum deformans. The technique of bilateral pallidotomy and theories of its effectiveness are discussed.
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ranking = 3
keywords = operative
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9/151. Allograft reconstruction of the extensor mechanism for progressive extensor lag after total knee arthroplasty and previous patellectomy: a 3-year follow-up.

    Major extensor lag after total knee arthroplasty may follow operative damage to the patellar tendon or its insertion. It may also occur in a late progressive form postoperatively. A successful allograft reconstruction of the extensor mechanism for progressive extensor lag after total knee arthroplasty is described. Patellectomy had been carried out earlier on the same knee for patellofemoral osteoarthritis.
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ranking = 2
keywords = operative
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10/151. Reversible sensorineural hearing impairment induced by a carotid body tumor.

    A case of a 62-year-old Austrian man having a 25-year history of a right-sided carotid body tumor (CBT) is presented. Three months before being transferred to the University of Vienna for tumor resection the patient developed symptoms of tinnitus, progressive ipsilateral hearing loss and dysphagia. Pure-tone audiometry demonstrated a 50 dB right sensorineural hearing loss. A 6 x 6 x 4 cm firm, pulsatile mass was found in the right carotid triangle and extending towards the base of the skull. One week after radical tumor resection all preoperative symptoms disappeared and hearing of the right ear recovered. review of the available literature showed that hearing loss and tinnitus are unusual symptoms of a CBT. Our findings suggest that routine audiometric evaluations in such cases of CBT patients should be obtained in order to determine the real incidence of audiological disorders.
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ranking = 1
keywords = operative
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