Short and long-term outcomes after combined intravenous thrombolysis and mechanical thrombectomy versus direct mechanical thrombectomy: a prospective single-center study.

Recent clinical trials demonstrated that mechanical thrombectomy (MT) using second-generation endovascular devices has beneficial effects in acute ischemic stroke (AIS) due to large vessel occlusion (LVO). However, it remains controversial if intravenous thrombolysis (IVT) prior to MT is superior compared to direct mechanical thrombectomy (DMT). The aims of this study were to compare short and long-term outcomes between IVT + MT and DMT patients. We prospectively recruited AIS patients with LVO in the anterior or posterior circulation eligible for MT with and without prior IVT. Modified Rankin Scale (mRS) and mortality were assessed at baseline, at discharge, 90-days and 1-year after stroke. Favorable outcome was defined as a mRS score ≤2. Of the 66 patients included, 33 (50%) were in IVT + MT group and 33 (50%) were in DMT group. Except for a higher prevalence of patients using anticoagulants at admission in DMT group, baseline characteristics did not differ in the two groups. Procedural characteristics were similar in IVT + MT and DMT group. Rate of favorable outcome was significantly higher in IVT + MT patients than DMT ones both 90-days (51.5 vs. 18.2%; p = 0.004) and 1-year (51.5 vs. 15.2%; p = 0.002) after stroke. DMT patients were six times more likely to die during the 1-year follow-up compared to IVT + MT patients. This study suggests that bridging therapy may improve short and long-term outcomes in patients eligible for endovascular treatment. Further studies with larger patient numbers and randomized design are needed to confirm our findings.

A Novel Protocol for Planning and Navigation in Craniofacial Surgery: A Preclinical Surgical Study.

PURPOSE: In modern craniofacial surgery, the accuracy of osteotomies plays a central role in surgical technique. To reach a higher level of accuracy, many centers use virtual presurgical planning. In the past decade, some surgeons also have applied navigational approaches to craniofacial procedures. In this work, a novel protocol for surgical planning and intraoperative navigation is described and validated in a preclinical setting to determine its accuracy in guiding osteotomies. MATERIALS AND METHODS: This study was based on planning a set of osteotomies using 3-dimensional models of computed tomographic images of human skulls. The planned osteotomies were reproduced on real skulls using an optical infrared navigation system. Positions of the performed osteotomies and planned osteotomies were compared. Results were described as the mean positional error and as a Lin concordance coefficient. The Bland-Altman interval of agreement also was defined to assess a range that could include 95% of possible errors. RESULTS: The mean error was 0.044 mm (95% confidence interval [CI], -0.128 to +0.216), the Lin concordance interval was 0.999 (95% CI, 0.999-1.000), and the Bland-Altman limit of agreement ranged from -1.500 to +1.589 mm. CONCLUSIONS: These results show a submillimetric mean error and a very narrow interval of agreement, providing preclinical validation of this new protocol and suggesting that it could be applied in a clinical setting.

Cochlear Implant Outcomes and Genetic Mutations in Children with Ear and Brain Anomalies.

BACKGROUND: Specific clinical conditions could compromise cochlear implantation outcomes and drastically reduce the chance of an acceptable development of perceptual and linguistic capabilities. These conditions should certainly include the presence of inner ear malformations or brain abnormalities. The aims of this work were to study the diagnostic value of high resolution computed tomography (HRCT) and magnetic resonance imaging (MRI) in children with sensorineural hearing loss who were candidates for cochlear implants and to analyse the anatomic abnormalities of the ear and brain in patients who underwent cochlear implantation. We also analysed the effects of ear malformations and brain anomalies on the CI outcomes, speculating on their potential role in the management of language developmental disorders. METHODS: The present study is a retrospective observational review of cochlear implant outcomes among hearing-impaired children who presented ear and/or brain anomalies at neuroimaging investigations with MRI and HRCT. Furthermore, genetic results from molecular genetic investigations (GJB2/GJB6 and, additionally, in selected cases, SLC26A4 or mitochondrial-DNA mutations) on this study group were herein described. Longitudinal and cross-sectional analysis was conducted using statistical tests. RESULTS: Between January 1, 1996 and April 1, 2012, at the ENT-Audiology Department of the University Hospital of Ferrara, 620 cochlear implantations were performed. There were 426 implanted children at the time of the present study (who were <18 years). Among these, 143 patients (64 females and 79 males) presented ear and/or brain anomalies/lesions/malformations at neuroimaging investigations with MRI and HRCT. The age of the main study group (143 implanted children) ranged from 9 months and 16 years (average = 4.4; median = 3.0). CONCLUSIONS: Good outcomes with cochlear implants are possible in patients who present with inner ear or brain abnormalities, even if central nervous system anomalies represent a negative prognostic factor that is made worse by the concomitant presence of cochlear malformations. Common cavity and stenosis of the internal auditory canal (less than 2 mm) are negative prognostic factors even if brain lesions are absent.

Stroke After varicella-zoster infection: report of a case and review of the literature.

Ischemic stroke is a recognized complication of varicella-zoster virus infections. We report on an immunocompetent 5-year-old girl who presented with acute neurologic deficits attributed to cerebral infarction, 3 months after varicella-zoster virus infection. Magnetic resonance imaging of the brain showed subacute ischemic lesions in the territory of the right middle cerebral artery. A literature review of 70 similar cases is reported.

Encoding of human action in Broca's area.

Broca's area has been considered, for over a century, as the brain centre responsible for speech production. Modern neuroimaging and neuropsychological evidence have suggested a wider functional role is played by this area. In addition to the evidence that it is involved in syntactical analysis, mathematical calculation and music processing, it has recently been shown that Broca's area may play some role in language comprehension and, more generally, in understanding actions of other individuals. As shown by functional magnetic resonance imaging, Broca's area is one of the cortical areas activated by hand/mouth action observation and it has been proposed that it may form a crucial node of a human mirror-neuron system. If, on the one hand, neuroimaging studies use a correlational approach which cannot offer a final proof for such claims, available neuropsychological data fail to offer a conclusive demonstration for two main reasons: (i) they use tasks taxing both language and action systems; and (ii) they rarely consider the possibility that Broca's aphasics may also be affected by some form of apraxia. We administered a novel action comprehension test--with almost no linguistic requirements--on selected frontal aphasic patients lacking apraxic symptoms. Patients, as well as matched controls, were shown short movies of human actions or of physical events. Their task consisted of ordering, in a temporal sequence, four pictures taken from each movie and randomly presented on the computer screen. Patient's performance showed a specific dissociation in their ability to re-order pictures of human actions (impaired) with respect to physical events (spared). Our study provides a demonstration that frontal aphasics, not affected by apraxia, are specifically impaired in their capability to correctly encode observed human actions.

Genetic syndromes involving hearing.

OBJECTIVE: The fundamental processes involved in the mechanism of hearing seem to be controlled by hundreds of genes and hereditary hearing impairment may be caused by a large variety of genetic mutations in different genes. Approximately 150 loci for monogenic syndromic and non-syndromic hearing impairment (HI) disorders have been mapped to the human genome. The identification of these genes and functional analysis of the proteins they encode, are paving the way towards a better understanding of the physiology and pathophysiology of the auditory system. To date, approximately 50 causative genes have been identified. METHODS: The clinical and neuroradioldical findings of syndromal hearing impairment are analysed. RESULTS: This paper presents an updated report on genetic syndromes in which a hearing impairment is involved, with a particular attention to the ones associated with external ear and craniofacial malformations. CONCLUSIONS: Concepts in human genetics are rapidly evolving together with technologies. The concept itself of gene is changing. A genetic diagnosis of syndromal hearing impairment has many practical consequences: it can implies specific prognosis, specific management, specific recurrence risk in relatives and, if the diagnosis is confirmed at the molecular level, possibility of a specific early prenatal diagnosis for severe syndromes. It is important to highlight the necessity that the pediatric otolaryngologist must have a close collaboration with a clinical geneticist and a neuroradiologist.

Goldenhar syndrome in association with agenesia of the internal carotid artery.

The association between Goldenhar syndrome and homolateral internal carotid artery agenesis to our knowledge, has never emerged before. We present a case report in a female child that was examined with tonal audiometry, A.B.R., CT scan, MR, digital-subtraction-angiography and color Doppler. The aim of this report is to present an uncommon pathologic association which also provides a brief review of the embryology of the ear and carotid vessels whilst discussing probable pathogenetic causes.

Anencephaly: MRI findings and pathogenetic theories.

We describe the MRI appearances of an anencephalic newborn who survived for 13 h; particularities of this case are male gender and the absence of other associated malformations. Moreover, we discuss the pathogenetic theories of anencephaly, correlating MRI findings with embryological data. An exencephaly-anencephaly sequence due to amnion rupture is hypothesized.