Takayasu arteritis (TA) is a rare large vessel arteritis, affecting primarily aorta and its major branches. Its clinical manifestations can vary significantly - from asymptomatic to serious vascular ...events. Acute neurological complications are frequent at the onset of the disease and in relapses. Anxiety and depression are more frequent in TA patients than in general population as well as during relapses. Prevalence of transient ischemic attack or ischemic stroke in TA patients is approximately 10-20%.
We presented a patient with TA that began with a depressive episode resulting in attempted suicide by bromazepame poisoning. This was subsequently followed by major ischemic stroke caused by thrombosis of the left middle cerebral artery (probably due to aortic arch embolism) successfully treated with intravenous thrombolysis.
Intravenous thrombolysis appears to be safe and effective in patients with TA and stroke.
Introduction. Synchronous multicentric cerebral gliomas are uncommon brain tumors, mostly malignant, with unknown pathogenesis, unfavorable prognosis and still controversial management. Preoperative ...differentiation from other multiple brain pathologies by conventional magnetic resonance imaging (MRI) is often difficult, but supplemental use of advanced magnetic resonance techniques should allow the tumor biology to be predicted and an appropriate treatment strategy planned. Case report. We reported a 59-yearold man with double synchronous multicentric cerebral lesions, which had initial MRI and diffusion-weighted imaging presentation as left parietal metastasis and ipsilateral amygdalo- hippocampal low-grade glioma. However, magnetic resonance spectroscopy (MRS) of both lesions showed different metabolite profiles of malignant glioma. En bloc resection of the easily accessible parietal lesion revealed glioblastoma with methylated O6-methylguanine-DNA methyltransferase (MGMT) gene promoter. Subsequently, the patient was treated with temozolomide (TMZ)-based chemoradiation according to Stupp?s protocol, with continuous standard (5/28) adjuvant TMZ in 12 courses. Despite prolonged stabilization of the disease with good life-quality during treatment, the patient died 19 months after diagnosis. The time to tumor progression estimated by MRI was 17 months. Conclusion. MRS significantly improved the differential diagnostic accuracy of conventional MRI in our patient. In accordance with reviewed literature data, the younger age, good initial performance status and methylated MGMT gene promoter were all favorable predictors of longer survival in the reported case. Resection of at least one easily accessible tumor lesion, followed by TMZ-based chemoradiation, with continuous adjuvant TMZ in more than 6 standard courses, seems currently to be the most beneficial therapeutic option for such cases.
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Erdheim-Chester disease (ECD) is a rare, systemic form of non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family with characteristic bilateral symmetrical long bone osteosclerosis, ...associated with xanthogranulomatous extra skeletal organ involvement. In ECD, central nervous system (CNS) and orbital lesions are frequent, and more than half of ECD pa tients carry the V600E mutation of the protooncogene BRAF. The synchronous or metachronous development of ECD and Langerhans cell histiocytosis (LCH) in the same patients is rare, and the possible connection between them is still obscure. Cladribine is a purine substrate analogue that is toxic to lymphocytes and monocytes with good hematoencephalic penetration.
We presented a 23-year-old man successfully treated with cladribine due to BRAF V600E-mutation-negative ECD with bilateral orbital and CNS involvement ECD developed metachronously, 6 years after chemotherapy for multisystem LCH with complete disease remission and remaining central diabetes insipidus. During ECD treatment, the patient received 5 single-agent chemotherapy courses of cladribine (5 mg/m2 for 5 consecutive days every 4 weeks), with a reduction in dose to 4 mg/m2 in a fifth course, delayed due to severe neutropenia and thoracic dermatomal herpes zoster infection following the fourth course. Radiologic signs of systemic and CNS disease started to resolve 3 months after the end of chemotherapy, and CNS lesions completely resolved within 2 years after the treatment After 12-year follow-up, there was no recurrence or appearance of new systemic or CNS xanthogranulomatous lesions or second malignancies.
In accordance with our findings and recommendations provided by other authors, cladribine can be considered an effective alternative treatment for ECD, especially with CNS involvement and BRAF V600E-mutation-negative status, when interferon-alpha as the first-line therapy fails.
Multiple sclerosis (MS) is an immune-mediated disease of the central nervous system that affects young individuals and leads to severe disability. High dose immunoablation followed by autologous ...hemopoietic stem cell transplantation (AHSCT) has been considered in the last 15 years as potentialy effective therapeutic approach for aggressive MS. The most recent long-time follow-up results suggest that AHSCT is not only effective for highly-aggressive MS, but for relapsing-remitting MS as well, providing long-term remission, or maybe even cure. We presented a 10-year follow-up of the first MS patient being treated by immunoablation therapy and AHSCT.
A 27-year-old male experienced the first symptoms--intermitent numbness and paresthesia of arms and legs of what was treated for two years by psychiatrist as anxiety disorder. After he developed severe paraparesis he was admitted to the Neurology Clinic and diagnosed with MS. Our patient developed aggressive MS with frequent relapses, rapid disability progression and transition to secondary progressive form 6 years after MS onsetthe Expanded Disability Status Scale (EDSS) 7.0 Ambulation Index (AI) 7. AHSCT was performed, cyclophosphamide was used for hemopoietic stem cell mobilization and the BEAM protocol was used as conditionig regimen. No major adverse events followed the AHSCT. Neurological impairment improved, EDSS 6.5, AI 6 and during a 10-year follow-up remained unchanged. Brain MRI follow-up showed the absence of gadolinium enhancing lesions and a mild progression of brain atrophy.
The patient with rapidly evolving, aggressive, noninflammatory MS initialy improved and remained stable, without disability progression for 10 years, after AHSCT. This kind of treatment should be considered in aggressive MS, or in disease modifying treatment nonresponsive MS patients, since appropriately timed AHSCT treatment may not only prevent disability progression but reduce the achieved level of disability, as well.
Acute aortic dissection (AD) is the most common life-threatening disorder affecting the aorta. Neurological symptoms are present in 17-40% of cases. The management of these patients is controversial.
...We presented a 37-year-old man admitted for complaining of left-sided weak-ness. Symptoms appeared two hours before admission. The patient had no headache, neither thoracic pain. Neurological examination showed mild confusion, left-sided hemiplegia, National Institutes of Health Stroke Scale (NIHSS) score was 10. Ischemic stroke was suspected, brain multislice computed tomography (MSCT) and angiography were performed and right intrapetrous internal carotid artery dissection noted. Subsequent color Doppler ultrasound of the carotid arteries showed dissection of the right common carotid artery (CCA). The patient underwent thoracic and abdominal MSCT aortography which showed ascending aortic dissection from the aortic root, propagating in the brachiocephalic artery and the right CCA. Digital subtraction angiography was performed subsequently and two stents were successfully implanted in the brachiocephalic artery and the right CCA prior to cardiac surgery, only 6 hours after admission. The ascending aorta was reconstructed with graft interposition and the aortic valve re-suspended. The patient was hemodynamically stable and with no neurologic deficit after surgery. Unfortinately, at the operative day 6, mediastinitis developed and after intensive treatment the patients died 35 days after admission.
In young patients with suspected stroke and oscillatory neurological impairment urgent MSCT angiography of the brain and neck and/or Doppler sonography of the carotid and vertebral artery are mandatory to exclude carotid and aortic dissection. The prompt diagnosis permits urgent carotid stenting and cardiosurgery. To the best of our knowledge, this is the first published case of immediate carotid stenting in acute ischemic stroke after the diagnosis of carotid and aortic dissection and prior to cardiac surgery
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