Cases reported "Intracranial Thrombosis"

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1/2. Emergent decompressive craniectomy in patients with fixed dilated pupils due to cerebral venous and dural sinus thrombosis: report of three cases.

    OBJECTIVE AND IMPORTANCE: Cerebral venous and dural sinus thrombosis is a rare cause of stroke. Although morbidity and mortality have greatly decreased in recent years as a result of early diagnosis and timely medical treatment, when coma occurs the prognosis remains poor. We evaluated whether emergent decompressive craniectomy has a role in the treatment of patients with brain herniation from dural sinus thrombosis and hemorrhagic infarct. CLINICAL PRESENTATION: Three patients developed large hemorrhagic infarct with coma and bilaterally fixed and dilated pupils resulting from aseptic dural sinus thrombosis. INTERVENTION: Two patients underwent emergent surgical decompression as soon as brain herniation developed, and these patients had complete functional recovery. One underwent delayed surgical decompression and remained severely disabled. CONCLUSION: Our results provide preliminary evidence that emergent decompressive craniectomy is effective in patients with brain herniation from dural sinus thrombosis, provided that the clinical onset is recent. We therefore recommend consideration of this aggressive surgical technique for such patients, who may survive with good outcomes.
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2/2. Cerebral ischemic infarction in paroxysmal nocturnal hemoglobinuria report of 2 cases and updated review of 7 previously published patients.

    BACKGROUND: Paroxysmal nocturnal hemoglobinuria (PNH) is an acquired clonal stem cell disorder, leading to a deficient biosynthesis of surface proteins in hematopoetic cells. Clinical symptoms consist of various combinations of intravascular hemolysis, bone marrow failure and mainly venous thrombotic events. Arterial thrombosis has been described only in a few cases.methods: We report two patients with fatal ischemic strokes in PNH caused by arterial occlusion. In addition we also reviewed 7 previously reported cases in the literature and obtained follow-up information on 4 of the 5 survivors.RESULTS: Both patients from our clinic had experienced hemoglobinuria and cytopenia prior to the vascular events but in one case diagnosis of PNH was only established after stroke onset. The causal role of PNH was supported by the absence of other vascular risk factors and by simultaneous symptoms of hemolysis and cytopenia. The diagnosis of PNH was confirmed by a positive result in flow cytometry. Despite extended therapeutic interventions, including antithrombotic treatment (with heparin or tirofiban) and decompressive hemicraniectomy both patients developed further thrombotic complications. The two patients died during their hospital stay from vascular or infectious complications. A review of all 9 published cases revealed that four patients died within a few weeks after the initial thrombotic event. No difference in treatment between patients with good or bad outcome was seen.CONCLUSIONS: PNH is a rare cause of arterial stroke but it should be considered in young stroke patients with abnormal blood findings. As yet, there is no effective antithrombotic treatment.
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