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1/303. akinetic mutism after fourth ventricle choroid plexus papilloma: treatment with a dopamine agonist.

    BACKGROUND: akinetic mutism is a behavioral state wherein a patient seems to be awake but does not move or speak. Several patients are reported to have developed mutism after posterior fossa surgery. We present a patient who developed akinetic mutism after total excision of a choroid plexus papilloma of the fourth ventricle, and who was treated with bromocriptine. CASE DESCRIPTION: An 18-year-old woman was admitted with akinetic mutism, which had developed 6 days after posterior fossa surgery. She had had no neurologic deficit in the first 5 days after surgery and could communicate with her family. Despite antioedematous therapy and daily lumbar punctures to drain cerebrospinal fluid, there was no clinical improvement after she entered the akinetic mute state. brain magnetic resonance revealed ventriculomegaly; brain single photon emission computed tomography revealed bilateral reduction of perfusion in the frontal region. Because daily lumbar drainage did not result in clinical improvement, shunt placement was not considered. bromocriptine therapy was begun at a dose of 2x2.5 mg; 24 hours later, the patient started to speak and move her upper extremities. Further improvement occurred over the following week when the dose was increased to 3x2.5 mg. bromocriptine was replaced with a placebo to determine whether the neurologic improvement was caused by the medicine. The patient's neurologic status deteriorated progressively; therefore, bromocriptine was restarted and she was discharged from the hospital. During the 6 months of follow-up, the patient has remained in good health. CONCLUSIONS: The etiology of akinetic mutism is not clear. Monoaminergic pathways, particularly dopaminergic cell groups, are most probably involved in this syndrome, because bromocriptine has a dramatic effect on these patients, as demonstrated in our case.
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2/303. Reactivation of herpes virus after surgery for epilepsy in a pediatric patient with mesial temporal sclerosis: case report.

    OBJECTIVE: This case report is presented to raise the awareness of the potential risk of reactivation of herpes simplex virus (HSV) encephalitis after intracranial surgery. CLINICAL PRESENTATION: The case of an 8-year-old male patient who suffered a reactivation of HSV encephalitis after undergoing amygdalohippocampectomy for complex partial seizures is reported. This patient had previously contracted HSV 1 meningoencephalitis at the age of 16 months. Six years later, a left amygdalohippocampectomy was proposed after the development of intractable partial epilepsy associated with left mesial temporal lesions. During the postoperative period, the patient suffered severe clinical deterioration with partial status epilepticus, aphasia, and hyperthermia, which resolved after intensive antiepileptic treatment supported by acyclovir. CONCLUSION: We advise prophylactic pre-, peri-, and postoperative treatment with acyclovir for patients with known histories of HSV encephalitis who undergo intracranial procedures.
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3/303. Suprasellar cystic germinoma.

    We report on a germinoma in the suprasellar region, which had multiple large cystic components. A 13-year-old girl with disturbed visual acuity and growth retardation was admitted to our hospital for treatment of an intracranial tumor. The lesion was difficult to diagnose as a germinoma preoperatively, because of its radiographic characteristics. Histopathological examination revealed that the tumor was a germinoma. Surgery, chemotherapy with carboplatin and etoposide, and radiotherapy (30 Gy) were successful in inducing complete remission of the tumor. The patient's endocrine status remained normal, except for a low GH concentration and diabetes insipidus.
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4/303. Restenosis following carotid endarterectomy--clinical profiles and pathological findings.

    Restenosis following carotid endarterectomy is not a rare condition. Among 122 endarterectomies we experienced, five restenoses (4.1%) were encountered and treated by the second surgery. The present report clarifies the clinical profiles and pathological findings of restenosis following carotid endarterectomy. Mean age of restenosis group (59 years old) was not significantly different from the group without restenosis (62 years old). Average duration between the first endarterectomy and the second surgery was 17 months (8-30 months). Initial symptoms were transient ischemic attack in three sides, minor stroke in one side, and asymptomatic in one. Degree of stenosis was tight (> or = 90%) in two and moderate (70-89%) in three. It is interesting to note that no ulcer was noted in the first endarterectomy specimen. At surgery for restenosis, two cases had symptoms and another two cases were asymptomatic, though all had neck bruits. Four of five lesions were treated by short venous graft from common carotid artery to distal internal carotid artery and another lesion was treated by second endarterectomy and Dacron patch graft. pathology was studied in four and all showed myointimal hyperplasia. Three of four restenosis tissues showed mutant form p53 by immunohistochemistry. The present study indicates that restenosis following carotid endarterectomy is not a rare status. Short venous bypass across the stenotic portion is the treatment of choice. Monoclonal growth of smooth muscle with mutant form p53 might be related to the restenosis.
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5/303. Occipitocervicothoracic fixation for spinal instability in patients with neoplastic processes.

    OBJECT: Occipitocervicothoracic (OCT) fixation and fusion is an infrequently performed procedure to treat patients with severe spinal instability. Only three cases have been reported in the literature. The authors have retrospectively reviewed their experience with performing OCT fixation in patients with neoplastic processes, paying particular attention to method, pain relief, and neurological status. methods: From July 1994 through July 1998, 13 of 552 patients who underwent a total of 722 spinal operations at the M. D. Anderson Cancer Center have required OCT fixation for spinal instability caused by neoplastic processes (12 of 13 patients) or rheumatoid arthritis (one of 13 patients). Fixation was achieved by attaching two intraoperatively contoured titanium rods to the occiput via burr holes and Luque wires or cables; to the cervical spinous processes with wisconsin wires; and to the thoracic spine with a combination of transverse process and pedicle hooks. Crosslinks were used to attain additional stability. In all patients but one arthrodesis was performed using allograft. At a follow-up duration of 1 to 45 months (mean 14 months), six of the 12 patients with neoplasms remained alive, whereas the other six patients had died of malignant primary disease. There were no deaths related to the surgical procedure. Postoperatively, one patient experienced respiratory insufficiency, and two patients required revision of rotational or free myocutaneous flaps. All patients who presented with spine-based pain experienced a reduction in pain, as measured by a visual analog scale for pain. All patients who were neurologically intact preoperatively remained so; seven of seven patients with neurological impairment improved; and six of seven patients improved one Frankel grade. There were no occurrences of instrumentation failure or hardware-related complications. In one patient a revision of the instrumentation was required 13.5 months following the initial surgery for progression of malignant fibrous histiosarcoma. CONCLUSIONS: In selected patients, OCT fixation is an effective means of attaining stabilization that can provide pain relief and neurological preservation or improvement.
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6/303. Spontaneous renal allograft rupture attributed to acute tubular necrosis.

    A renal allograft recipient receiving triple immunosuppressive therapy developed spontaneous allograft rupture 5 days after her second cadaveric renal transplantation. Renal biopsy showed interstitial edema with severe acute tubular necrosis (ATN). There was no evidence of acute rejection or renal vein thrombosis. The ruptured renal graft was salvaged by an aggressive fluid resuscitation therapy and surgical hemostasis. The renal function was satisfactory on discharge. We conclude that renal allograft rupture can be the result of interstitial edema solely attributed to ATN in the absence of graft rejection. The ruptured graft kidney is potentially salvageable for those patients whose hemodynamic status can be stabilized by appropriate supportive therapy.
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7/303. Combined Rastelli and atrial switch procedure: a 10-year follow-up.

    We performed a combined atrial (Mustard) and ventricular (Rastelli) repair on a previously palliated patient with situs inversus, atrioventricular discordance, ventricular septal defect, and pulmonary atresia. The suitability and durability of this operative strategy is supported by the satisfactory hemodynamic and functional status of the patient 10 years later.
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8/303. Adjuvant treatment of severe acute pancreatitis with C1 esterase inhibitor concentrate after haematopoietic stem cell transplantation.

    BACKGROUND: With an incidence of 4%, acute pancreatitis is a common complication of bone marrow or peripheral haematopoietic stem cell transplantation, which contributes significantly to morbidity and mortality in these patients. In most cases, the pathogenesis of acute pancreatitis cannot be attributed to a single pathogenetic factor, as treatment toxicity, acute graft versus host disease, infection, and cholestasis may all contribute. Acute pancreatitis is characterised by inflammation and activation of digestive proenzymes leading to autodigestive destruction of the pancreas and systemic activation of protease cascades including the complement system. AIM: To describe the effects of human C1 esterase inhibitor in two children, who developed severe acute pancreatitis with considerable complement activation after allogeneic haematopoietic stem cell transplantation. methods: Both children showed clinical features resembling those observed in capillary leakage syndrome. In both patients, treatment with C1 esterase inhibitor concentrate contributed to a rapid clinical stabilisation. CONCLUSIONS: These observations strongly support the proposed pathophysiological concept that early treatment with C1 esterase inhibitor interferes with the activation of the complement system in acute pancreatitis. Inhibition of complement activation prevents its adverse effects on vascular function and permeability, and thus stabilises intravascular fluid status and prevents multiorgan failure in acute pancreatitis.
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9/303. Pulmonary thromboembolism after spinal instrumentation surgery.

    A 57-year-old woman was hospitalized because of gait disturbance and dysuria. Close examination revealed a cauda equina tumor at the level of L2 and L3. Tumor resection was performed, with posterolateral fusion and spinal instrumentation. On the eleventh day after the surgery, she experienced dyspnea and chest pain during standing and walking exercise. Pulmonary thromboembolism was diagnosed, based on: (1) blood gas analysis findings of hypoxemia and (2) defective images in both of the upper lobes on urgent pulmonary blood flow scintigram. Her clinical status improved with urgent thrombolytic therapy (with tisokinase and urokinase) and anticoagulation therapy (with heparin and warfarin), and her life was saved. When pulmonary thromboembolism occurs, early diagnosis by pulmonary blood flow scintigram and early thrombolytic and anticoagulative therapies are necessary. Special attention should be paid to symptoms of pulmonary thromboembolism in patients after spinal surgery.
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10/303. The failing Fontan with atrial flutter: a successful surgical option.

    Two successful cases of eliminated atrial flutter and improved clinical status for Fontan patients are presented. An operation combining introduction of an extracardiac conduit for the Fontan connection, to direct all systemic venous blood away from the atrium, and atrial pathway division and cryoablation, is a useful surgical option for failing Fontan patients.
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