Desmoplastic gangliogliomas (DIG) are rare primary neoplasms that comprise 0.5-1.0% of all intracranial tumors. Clinically, there are two forms of DIG, the infantile and the non-infantile. These tumors invariably arise in the supratentorial region and commonly involve more than one lobe, preferentially the temporal and frontal. On neuroimaging are seen as large hypodense cystic masses with a solid isodense or slightly hyperdense superficial portion. The histologic diagnosis is characterized by the presence of three different cell lines: astrocytic, neuronal, and primitive neuroectodermal marker sites, which were demonstrable. The treatment of choice is radical surgical excision, and if this is done, achieved complete healing of the patient does not require additional treatment. A literature review of DIG was compiled through Medline/Ovid using the keywords "desmoplastic infantile ganglioglioma", "desmoplastic non-infantile ganglioglioma" covering the years 1984-2009. We present a review of a total of 113 cases of infantile (94) and non-infantile gangliogliomas (19) published to date, examining the clinical, radiologic, surgical, and pathological aspects, as well as the outcome. Desmoplastic gangliogliomas represent a rare tumor group with two well-defined age groups, the children and non-children. Desmoplastic infantile gangliogliomas are the most common and occur in children below 5 years of age, and the large majority of them present within the first year of life. Surgery is the treatment of choice and no complementary treatment is needed in cases of complete tumor resection.
Twiddler syndrome occurs when a patient intentionally or unintentionally manipulates an implantable generator (usually a pacemaker) and dislodges the pacing leads, causing malfunction of the device. Though the syndrome has been described in patients with pacemakers, to our knowledge only one spontaneous case has been described in patients undergoing deep brain stimulation for movement disorders. We report the clinical cases of two patients with Parkinson's disease who had subthalamic bilateral electrodes implanted and presented the twiddler syndrome 2 and 3 years after surgery. We analysed the possible mechanisms of this syndrome and note that twiddler syndrome should be suspected in patients undergoing deep brain stimulation and showing hardware dysfunction.
BACKGROUNDFlow-diverter technology has become an important stent-based embolization tool in the treatment of complex cerebrovascular pathology. We report here the experience of 4 Spanish centers with using the SILK flow-diverter (SFD) device.OBJECTIVETo evaluate the safety and efficacy of using the SFD in the endovascular treatment of intracranial aneurysms with complex morphology.METHODSWe retrospectively examined a prospectively maintained database of patients treated with SFD devices between July 2008 and December 2013 at 1 of 4 institutions in Spain. Data regarding patient demographics, aneurysm characteristics, and technical procedure were analyzed. Angiographic and clinical findings were recorded during the procedure and at 12 months postoperatively.RESULTSA total of 175 SFD devices were implanted in 157 patients (women/men: 119/38; mean, median, and range of age: 56.2, 56.7, and 19-80 years, respectively), who were treated in a delayed manner (3-6 months from the event) for 180 aneurysms (165 unruptured and 15 ruptured). Adverse events (acute and delayed) were observed in 28.7% of cases (45/157), and most were resolved (19.1%; 30/157). Six months after the procedure, total morbidity and mortality were 9.6% (15/157) and 3.2% (5/157), respectively. Long-term imaging follow-up showed complete occlusion, neck remnants, and residual aneurysm in 78.1% (100/128), 14.0% (18/128), and 7.8% (10/128) of cases, respectively.CONCLUSIONSThe SFD device is an effective tool for the treatment of challenging aneurysms, and allows complete occlusion within a year of the procedure in most patients, with morbidity and mortality comparable to those previously reported for similar devices.
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