Rare and Symptomatic Cavernous Donut-Shaped Aneurysm Treated by Flow Diverter Deployment.

BACKGROUND: We describe the case of a 62-year-old woman who was admitted to our center for acute diplopia secondary to a left III cranial nerve palsy, left eyelid swelling and ptosis, and mild ipsilateral retroorbital pain. No other motor or sensitive deficits were observed. CASE DESCRIPTION: Computed tomography angiography and digital subtraction angiography were performed, showing a 25-mm left intracavernous aneurysm with a central intrasaccular thrombus, an intrasaccular "swirling" flow with a donut-shape appearance. A flow-diverter stent was deployed bridging the aneurysmal neck. CONCLUSIONS: Twelve months after the procedure the aneurysm was completely occluded, and the patient had totally recovered the cavernous syndrome. A careful literature review has been performed, and the different endovascular approaches have been analyzed.

Extending technology-aided leisure and communication programs to persons with spinal cord injury and post-coma multiple disabilities.

PURPOSE: These two studies extended technology-aided programs to promote leisure and communication opportunities to a man with cervical spinal cord injury and a post-coma man with multiple disabilities. METHOD: The studies involved the use of ABAB designs, in which A and B represented baseline and intervention phases, respectively. The programs focused on enabling the participants to activate songs, videos, requests, text messages, and telephone calls. These options were presented on a computer screen and activated through a small pressure microswitch by the man with spinal cord injury and a special touch screen by the post-coma man. To help the latter participant, who had no verbal skills, with requests and telephone calls, series of words and phrases were made available that he could activate in those situations. RESULTS: Data showed that both participants were successful in managing the programs arranged for them. The man with spinal cord injury activated mean frequencies of above five options per 10-min session. The post-coma man activated mean frequencies of about 12 options per 20-min session. CONCLUSIONS: Technology-aided programs for promoting leisure and communication opportunities might be successfully tailored to persons with spinal cord injury and persons with post-coma multiple disabilities. Implications for Rehabilitation Technology-aided programs may be critical to enable persons with pervasive motor impairment to engage in leisure activities and communication events independently. Persons with spinal cord injury, post-coma extended brain damage, and forms of neurodegenerative disease, such as amyotrophic lateral sclerosis, may benefit from those programs. The programs could be adapted to the participants' characteristics, both in terms of technology and contents, so as to improve their overall impact on the participants' functioning and general mood.

Long-term seizure and behavioral outcomes after corpus callosotomy.

Outcomes of corpus callosotomy (CC) have been mainly focused on seizures. The present study aimed to evaluate the long-term effects of CC on adaptive behaviors and caregivers' satisfaction in addition to seizures and to identify clinical predictors of postsurgical outcomes. Medical records of 26 patients (mean age at study time: 40 years, mean follow-up: 14 years) with childhood-onset epilepsy who underwent anterior or 2-stage complete CC were reviewed. A structured questionnaire was submitted to caregivers asking about relative changes in different seizure types, behavioral functions, and satisfaction with the postoperative outcomes. Formal neuropsychological assessment was carried out in a subgroup of patients. Selected clinical variables including age at surgery, extent of callosal section, length of follow-up, epilepsy syndrome, and presurgical cognitive level were submitted to multiple regression analysis. At the last follow-up visit, a reduction greater than 50% was observed mainly for drop attacks (65% of patients), followed by generalized tonic-clonic seizures (53%), and complex partial seizures (50%). No presurgical variables were significantly associated with seizure outcome. After surgery, more than half of patients showed attention enhancement, which was related to drop seizure improvement. Early age at surgery was associated with better behavioral regulation; complete CC slightly worsened language abilities. Satisfaction with surgery outcomes was expressed by 73% of caregivers and was dependent on drop seizure reduction and improvements in activities of daily living. A long-term positive psychosocial outcome is likely after CC also in severely disabled patients, especially if surgery is performed early.

[ (11) C]-methionine positron emission tomography in the postoperative imaging and followup of patients with primary and recurrent gliomas.

We investigated the sensitivity and specificity of [(11)C]-methionine positron emission tomography ([(11)C]-MET PET) in the management of glioma patients. We retrospectively analysed data from 53 patients with primary gliomas (16 low grade astrocytomas, 15 anaplastic astrocytomas and 22 glioblastomas) and Karnofsky Performance Status (KPS) > 70. Patients underwent [(11)C]-MET PET scans (N = 249) and parallel contrast-enhanced MRI (N = 193) and/or CT (N = 113) controls. In low grade glioma patients, MRI or CT findings associated with [(11)C]-MET PET additional data allowed discrimination residual disease from postsurgical changes in 96.22% of these cases. [(11)C]-MET PET early allowed detection of malignant progression from low grade to anaplastic astrocytoma with high sensitivity (91.56%) and specificity (95.18%). In anaplastic astrocytomas, we registered high sensitivity (93.97%) and specificity (95.18%) in the postoperative imaging and during the followup of these patients. In GBM patients, CT and/or MRI scans with additional [(11)C]-MET PET data registered a sensitivity of 96.92% in the postsurgical evaluation and in the tumour assessment during temozolomide therapy. A significant correlation was found between [(11)C]-MET mean uptake index and histologic grading (P < 0.001). These findings support the notion that complementary information derived from [(11)C]-MET PET may be helpful in postoperative and successive tumor assessment of glioma patients.

Protracted low doses of temozolomide for the treatment of patients with recurrent glioblastoma: A phase II study.

O(6)-alkylguanine-DNA alkyltransferase (AGAT), involved in temozolomide-induced DNA damage repair, plays a key role in the efficacy of temozolomide. AGAT activity may be reduced by protracted temozolomide doses. On the basis of the preclinical findings, we treated patients with a histologically-proven diagnosis of glioblastoma (GBM) following adjuvant temozolomide failure with a low protracted dose of temozolomide (130 mg/m(2)/day, days 1-7 and 15-21, every 4 weeks). The primary endpoint of the study was 6-month progression-free survival (PFS-6 m). The secondary endpoints were overall survival (OS) from the start of temozolomide alternative schedule and toxicity. Enrolment was ceased at 27 patients due to the lack of effectiveness of this regimen. Results indicate that our schedule is well-tolerated, but ineffective in patients with GBM and further strategies are required to improve the outcome of these patients.

Vagus nerve stimulation (VNS) is effective in treating catastrophic 1 epilepsy in very young children.

The objective of this study is to evaluate the safety and efficacy of vagus nerve stimulation (VNS) in very young children suffering from catastrophic epilepsy and status epilepticus. We reviewed files of 60 VNS-implanted children at our institution and we selected six very young patients, less than 3 years old (mean age at implant 1.6 years). All patients suffered from severe cognitive impairment and catastrophic epilepsy with underlying diagnosis of hemimegalencephaly (1), hypoxic-ischemic encephalopathy (1), tuberous sclerosis complex (1), and malignant migrating partial epilepsy of infancy (3). Three patients were VNS-implanted during admission at intensive care unit (ICU) after developing life-threatening status epilepticus. The mean follow-up time was 41.6 months. The VNS was implanted using a single cervical incision. No surgery-related complications were observed. Four of six children have shown a significant, persistent improvement in seizure control (range, 60-90%). In patients with status, insertion of the vagal nerve stimulator allowed early cessation of status and discharge from ICU. Quality of life and parental satisfaction improved and for three children there was some milestone evolution. Catastrophic epilepsy in infancy can be devastating and difficult to treat with drugs and surgery. If resective surgery is inappropriate or refused, VNS can be considered as a well-tolerated and effective procedure even in toddlers affected by severe epilepsy and multiple developmental disabilities.

Vagus nerve stimulation: clinical experience in drug-resistant pediatric epileptic patients.

INTRODUCTION: Vagus nerve stimulation (VNS) is an effective alternative treatment for patients with partial refractory epilepsy. Nevertheless, information regarding VNS in children is still limited. MATERIALS AND METHODS: The clinical efficacy, safety and neuropsychological effects of VNS in 34 children (mean age 11.5 years) with drug-resistant epilepsy were studied. Mean follow-up was 30.8 months. Nine patients have been diagnosed with Lennox-Gastaut Syndrome, nine patients were affected by severe partial epilepsy with bisynchronous EEG and drop attacks, and 16 patients suffered from partial epilepsy without bisynchronous EEG and fall seizures. Forms were designed for prospective data collection on each patient's history, seizures, implants, device settings, quality of life (QOL), neuropsychological assessment and adverse events. Surgical technique was performed both by standard two incisions and single neck incision. RESULTS: Mean reduction in total seizures was 39% at 3 months, 38% at 6 months, 49% at 12 months, 61% at 24 months and 71% at 36 months. Significant better results were obtained in partial epilepsy, with and without drop attacks, than in Lennox-Gastaut syndrome--three patients being seizure-free. No operative morbidity was reported. Side-effects were minor and transient--the most common were voice alteration and coughing during stimulation. In two patients, electrode breakage occurred 3 years after surgical procedure; in both cases, a new device was implanted after removing the vagal electrode coils and generator. CONCLUSION: VNS can be considered an appropriate strategy as an add-on treatment in children affected by drug-resistant partial epilepsy and ineligible for resective epilepsy surgery.

Bobble head doll syndrome in a child with a third ventricular cyst and hydrocephalus.

INTRODUCTION: The bobble head doll syndrome, mainly affecting children under 10 years of age, is a rare and surgically treatable movement disorder characterized by head bobbing occurring at a rate of 2-3 times/s. Its pathophysiological mechanism is not well known but two main factors are commonly associated with the condition: a dilatation of the third ventricle and, more frequently, a cystic lesion rather than a solid mass in the region of the third ventricle. ILLUSTRATIVE CASE: The illustrative case concerns a child with a third ventricular cystic lesion and hydrocephalus who had experienced abnormal head movements since the age of 1 year as well as ataxia and tremor of the arms. Contrast cranial MRI, at the age of 3, demonstrated enlargement of the third and lateral ventricles, a ballooned cyst inside the third ventricle with compression of all the diencephalic structures, a funnel dilation of the cranial part of the aqueduct, and a cyst in the septum pellucidum. A ventriculoperitoneal shunt (Hakim-Cordis) was placed and the head bobbing, tremor of the arms, and ataxia disappeared immediately. CONCLUSION: The good and immediate clinical result in our case emphasizes the opinion that the reduction of CSF pressure is the best therapeutical option in the bobble head doll syndrome.

Intermittent vagal nerve stimulation in paediatric patients: 1-year follow-up.

OBJECT: Vagal nerve stimulation (VNS) has recently been proposed as a valid treatment for adult patients with drug-resistant partial epilepsy. Little experience in children has been reported. In order to evaluate the clinical efficacy and tolerance of VNS, we studied 13 paediatric patients with drug-resistant partial epilepsy. METHODS: Improvement in seizure frequency was estimated by calculating the percentage of change in seizure frequency during each 3-month period following initiation of VNS, compared with the 3-month period prior to the implantation of the VNS device. The improvement in quality of life (QOL) was evaluated with the Vineland Behavior Adaptive Scale (VBAS). RESULTS: In all patients, the surgical procedure was well tolerated. A recent modification of the implantation technique needing only a single cervical incision, has further reduced the aesthetic damage, particularly in small children who have a reduced muscular mass. Three months after the surgical procedure, 10 of the 13 patients demonstrated a seizure reduction rate greater than 50%. At the 1-year follow-up these positive results were maintained: 6 out of 8 patients continued to demonstrate a seizure reduction rate greater than 50%. Comparison with the pre-implantation period also showed a significant improvement in QOL in 4 out of 8 patients. We conclude that VNS is a valid treatment modality in children with drug-resistant partial epilepsy.