Negative MRI and a seizure onset zone close to eloquent areas in FCD type II: Application of MRg-LiTT after a SEEG re-evaluation in pediatric patients with a previous failed surgery.

OBJECTIVE: Negative MRI and an epileptogenic zone (EZ) adjacent to eloquent areas are two main issues that can be encountered during pre-surgical evaluation for epilepsy surgery. Focal Cortical Dysplasia type II (FCD type II) is the most common aetiology underlying a negative MRI. The objective of this study is to present three cases of pediatric patients exhibiting negative MRI and a seizure onset zone close to eloquent areas, who previously underwent traditional open surgery or SEEG-guided radiofrequency thermocoagulations (RF-TC). After seizure seizure recrudescence, pre-surgical SEEG was re-evaluated and Magnetic Resonance-guided laser interstitial thermal therapy (MRg-LiTT) was performed. We discuss the SEEG patterns, the planning of laser probes trajectories and the outcomes one year after the procedure. METHODS: Pediatric patients who underwent SEEG followed by MRg-LiTT for drug-resistant epilepsy associated with FCD type II at our Centre were included. Pre-surgical videoEEG (vEEG), stereoEEG (sEEG), and MRI were reviewed. Post-procedure clinical outcome (measured by Engel score) and complications rates were evaluated. RESULTS: Three patients underwent 3 MRg-LiTT procedures from January 2022 to June 2022. Epileptogenic zone was previously studied via SEEG in all the patients. All the three patients pre-surgical MRI was deemed negative. Mean age at seizure onset was 47 months (21-96 months), mean age at MRg-LiTT was 12 years (10 years 10 months - 12 years 9 months). Engel class Ia outcome was achieved in patients #2 and #3, Engel class Ib in patient #1. Mean follow-up length was of 17 months (13 months - 20 months). Complications occurred in one patient (patient #2, extradural hematoma). CONCLUSIONS: The combined use of SEEG and MRg-LiTT in complex cases can lead to good outcomes both as a rescue therapy after failed surgery, but also as an alternative to open surgery after a successful SEEG-guided Radiofrequency Thermocoagulation (RF-TC). Specific SEEG patterns and a previous good outcome from RF-TC can be predictors of a favourable outcome.

Characterizing structural neural networks in de novo Parkinson disease patients using diffusion tensor imaging.

Parkinson disease (PD) can be considered as a brain multisystemic disease arising from dysfunction in several neural networks. The principal aim of this study was to assess whether large-scale structural topological network changes are detectable in PD patients who have not been exposed yet to dopaminergic therapy (de novo patients). Twenty-one drug-naïve PD patients and thirty healthy controls underwent a 3T structural MRI. Next, Diffusion Tensor Imaging (DTI) and graph theoretic analyses to compute individual structural white-matter (WM) networks were combined. Centrality (degree, eigenvector centrality), segregation (clustering coefficient), and integration measures (efficiency, path length) were assessed in subject-specific structural networks. Moreover, Network-based statistic (NBS) was used to identify whether and which subnetworks were significantly different between PD and control participants. De novo PD patients showed decreased clustering coefficient and strength in specific brain regions such as putamen, pallidum, amygdala, and olfactory cortex compared with healthy controls. Moreover, NBS analyses demonstrated that two specific subnetworks of reduced connectivity characterized the WM structural organization of PD patients. In particular, several key pathways in the limbic system, basal ganglia, and sensorimotor circuits showed reduced patterns of communications when comparing PD patients to controls. This study shows that PD is characterized by a disruption in the structural connectivity of several motor and non-motor regions. These findings provide support to the presence of disconnectivity mechanisms in motor (basal ganglia) as well as in non-motor (e.g., limbic, olfactory) circuits at an early disease stage of PD. Hum Brain Mapp 37:4500-4510, 2016. © 2016 Wiley Periodicals, Inc.

Double-level cervical total disc replacement for adjacent segment disease: is it a useful treatment? Description of late onset heterotopic ossification and review of the literature.

We report a rare case of double-level adjacent segment disease (ASD), occurring ten years later an anterior cervical discectomy (ACD) without fusion, treated by cervical arthroplasty, highlighting the outcome at long-term follow-up and focusing on heterotopic ossification. In 1995 a 25-year-old man satisfactorily underwent ACD at C4/C5. At that time MRI also showed signs of degenerative disc disease (DDD) at C3/C4 and C5/C6. Ten years later, a new MRI scan showed a large C3/C4 and a smaller C5/C6 soft disc hernia together with spondylotic changes at the level above and below the site of the first surgery. At C4/C5 imaging revealed a kyphotic stable "pseudoarthrosis" with anterior bridging osteophyte. The patient underwent double-level arthroplasty with ProDisc-C. Clinical and radiological outcome was satisfactory. 3 and 5 years after surgery, X-rays and CT scan documented the progressive development of heterotopic ossification, with gradual reduction of range of motion. A late onset heterotopic ossification can neutralize the theoretical advantages of cervical arthroplasty, which should be considered an effective surgical option only in selected cases. ACDF and restoration of normal lordosis can be a viable alternative in cervical revision surgery, as motion preservation can not be always mantained for a long time.

Anterior, extracanalar, cervical spine osteochondroma associated with DISH: description of a very rare tumor causing bilateral vocal cord paralysis, laryngeal compression and dysphagia. Case report and review of the literature.

INTRODUCTION: Osteochondromas are common benign bone tumors, rarely involving the spine. BACKGROUND: Osteochondroma account for about 35% of bone benign tumors and 9% of all bone tumors. Spinal involvement is described in 1%-4% of cases and their origin from the anterior surface of cervical vertebral bodies is exceedingly rare. AIM: We describe the rare case of an osteochondroma arising from the anterior surface of the C4 and C5 vertebral bodies, and not involving the spinal canal, in a 68-year-old male patient suffering from Diffuse Idiopathic Skeletal Hyperostosis (DISH). MATERIALS AND METHODS: The patient presented with acute onset of respiratory distress due to laryngeal compression exerted by the lesion, dysphagia and paralysis of left vocal cord. Imaging revealed the unusual lesion compressing and dislocating the air ways. An anterior approach to cervical spine was performed to remove the tumor. RESULTS: Postoperatively, the patient sustained a progressive improvement of respiratory function and recovery of the vocal cord paralysis. DISCUSSION: We believe that this case holds some interesting peculiarities: firstly, the anterior location of a cervical osteochondroma could be considered exceptional; yet, the resulting clinical picture should be remembered for differential diagnosis. Secondly, we hypothesized a possible correlation between the tumorigenesis of osteochondroma and the co-existence of DISH. Indeed, the abnormal bone turnover in cervical segments due to DISH could explain the unusual occurrence of osteochondromas in adult age. CONCLUSIONS: The occurrence of an osteochondroma should be considered in patients suffering from DISH and harbouring large osteophytes.

Laminoplasty is better of laminectomy in cervical stenotic myelopathy: myth or truth?

INTRODUCTION: Laminoplasty has been proposed as a treatment for cervical stenotic myelopathy (CSM) as an alternative to standard laminectomy as this has been considered directly associated with an increased risk of postoperative deformity. METHODS: We retrospective reviewed postoperative results of open door laminoplasty (unilateral approach technique) compared with laminectomy in terms of clinical/electophysiological results (somatosensory evoked potentials - SSEP, and motor evoked potentials - MEP); in addition the rate of subsequent spinal deformities was analyzed in both techniques. RESULTS: Postoperative results in terms of late follow up neurological assessment and neurophysiological improvement was substantially comparable in both groups. Postoperative dynamic cervical X-rays showed a kyphotic deformity in (12.5%) in patients undergoing laminectomy; none was unstable. No case of kypothic deformity occurred in patients undergoing open door laminoplasty. Complication rate was similar in both groups. DISCUSSION AND CONCLUSIONS: Standard laminectomy seems to be associated to late cervical spine deformities in a more relevant percentage of patients, possibly leading to severe forms of kyphosis and segmental instability over time compared with open-door expansive laminoplasty. The unilateral approach represents an evolution to standard open door technique that further spares posterior elements, may decrease the incidence of progressive spinal deformity and prevent the need for subsequent spinal stabilization.

Transient breathing disorders after posterior cervical surgery for degenerative diseases: pathophysiological interpretation.

Central sleep apnea is a breathing disorder that manifests as repetitive cessation of the breath during the sleep. The occurrence of breathing disorders after cervical laminectomy has been exceptionally described as a complication after cervical decompressive laminectomy for cervical stenotic myelopathy. In 1994, Naim-ur-Rahman reported the first case of postoperative central sleep apnea following C3-C6 laminectomy, occurring right after surgery and associated with spyncterial incontinence, that spontaneously recovered three weeks after onset. Recently we described a rare complication of cervical laminectomy for cervical stenotic myelopathy: the onset was delayed from surgery (nearly two weeks later) and cervical stenotic myelopathy was not associated to any other neurological sign of spinal cord damage as demonstrated by the neurophysiological assessment. Possible familiar predisposition can be matter of discussion. No definite interpretation of pathophysiological mechanisms can ultimately explain the occurrence of delayed and isolated central sleep apnea after laminectomy for the treatment of cervical stenotic myelopathy. Such a reversible and benign complication remain unpredictable in the best surgical hands.

Blink reflex recovery cycle distinguishes essential tremor with resting tremor from de novo Parkinson's disease: an exploratory study.

An increased R2 recovery component of the blink reflex (R2-BRrc) has been observed in Parkinson's disease (PD), cranio-cervical dystonia, dystonic tremor and essential tremor with associated resting tremor (rET), while the BRrc was reported normal in patients with essential tremor (ET). Distinguishing rET from tremor dominant PD (tPD) may be challenging especially in the first stages of the diseases, in the absence of DAT-SPECT investigation. We evaluated the possible usefulness of BRrc for differentiating subjects with de novo tPD from those with rET. We investigated R2-BRrc at interstimulus intervals (ISI) of 100, 150, 200, 300, 400, 500 and 750 ms in 11 participants with tPD, 10 with rET and 20 healthy controls. All participants underwent DAT-SPECT and cardiac MIBG scintigraphy. R2 recovery was significantly enhanced in tPD compared to controls at all investigated ISIs (p < 0.001), while in subjects with rET patients BRrc was significantly increased compared to controls at ISI 150, 200, 300, 400, 500 and 750 ms (p < 0.001). At ISI 100 R2-BRrc distinguished patients participants with de novo tPD from those with rET with a sensitivity, specificity and accuracy of 100%. Our findings demonstrate the usefulness of BRrc for differentiating de novo tPD from rET.

Prognostic value of EGFR expression in de novo and progressed atypical and anaplastic meningiomas: an immunohistochemical and fluorescence in situ hybridization pilot study.

AIM: The aim of this study was to assess both the epidermal growth factor receptor (EGFR) protein expression by immunohistochemistry and the EGFR gene amplification by fluorescence in situ hybridization in meningiomas of different grade, in order to evaluate their possible role in the development of the disease. EGFR protein belongs to the family of tyrosine kinase growth factor receptors, which also includes HER2, HER3 and HER4. Elevated expression or activity of EGFR has been reported in several cancers, including brain tumours. EGFR activation can enhance the malignant potential of epithelial tissues. METHODS: We investigated whether there was a difference in the EGFR protein expression and the EGFR gene amplification between the so called de novo malignant meningiomas and recurrent meningiomas with or without malignant progression from a previously lower grade tumor. Our goal was to evaluate if EGFR expression was a useful marker to select patients affected by meningioma with a major risk of recurrences. We also assessed the prognostic value of the EGFR expression on overall survival. RESULTS: Progression from benign meningiomas to atypical or anaplastic meningiomas correlated with an increase in the expression of EGFR protein. Our study shows that EGFR immunostaining in meningiomas directly correlates to the tumor's grade. The EGFR expression did not correlate with the overall survival and the recurrence-free survival of the patients affected by meningioma (de novo, recurrent and progressed). CONCLUSION: We submit that the EGFR expression is not a useful prognostic element to identify patients with a major risk of meningioma recurrence.

Design and rationale of a Prospective, Observational European Multicenter study on the efficacy of acute surgical decompression after traumatic Spinal Cord Injury: the SCI-POEM study.

OBJECTIVES: Despite many years of research, there is currently no treatment available that results in major neurological or functional recovery after traumatic spinal cord injury (tSCI). In particular, no conclusive data related to the role of the timing of decompressive surgery, and the impact of injury severity on its benefit, have been published to date. This paper presents a protocol that was designed to examine the hypothesized association between the timing of surgical decompression and the extent of neurological recovery in tSCI patients. STUDY DESIGN: The SCI-POEM study is a Prospective, Observational European Multicenter comparative cohort study. This study compares acute (<12 h) versus non-acute (>12 h, <2 weeks) decompressive surgery in patients with a traumatic spinal column injury and concomitant spinal cord injury. The sample size calculation was based on a representative European patient cohort of 492 tSCI patients. During a 4-year period, 300 patients will need to be enrolled from 10 trauma centers across Europe. The primary endpoint is lower-extremity motor score as assessed according to the 'International standards for neurological classification of SCI' at 12 months after injury. Secondary endpoints include motor, sensory, imaging and functional outcomes at 3, 6 and 12 months after injury. CONCLUSION: In order to minimize bias and reduce the impact of confounders, special attention is paid to key methodological principles in this study protocol. A significant difference in safety and/or efficacy endpoints will provide meaningful information to clinicians, as this would confirm the hypothesis that rapid referral to and treatment in specialized centers result in important improvements in tSCI patients.

Osteoporotic vertebral compression fracture pain (back pain): our experience with balloon kyphoplasty.

AIM: The aim of this study is to evaluate the safety and effectiveness of antalgic and functional results after interdisciplinary approach and treatment of vertebral compression fractures (VCF) with percutaneous balloon kyphoplasty (KP) by the pain medicine specialist. METHODS: Between April and December 2004, after informed consent, 13 patients have been treated for a total amount of 15 KP. For L5 - T11 level spinal anesthesia was performed, above T11 local infiltration was used. The following parameters were recorded: intraoperative course, postoperative course, pain before and after treatment, vertebral height restoring and quality of life measuring on visual analogical scale (VAS) and quality of life questionnaire of the European Foundation for Osteoporosis (QUALEFFO) scale. RESULTS: No complications or adverse events were recorded. VAS values for pain were 6.2+/-2.1 preKP vs 3.3+/-1.7 and 4.5+/-1.1 respectively postKP and at follow-up, with statistically significant differences. Vertebral heights were 53.5+/-16%, 71.2+/-21% and 68.1+/-13.5%, preKP, postKP and at follow-up respectively, with statistically significant differences, similarly to quality of life related parameters and QUALEFFO score. CONCLUSION: Back pain due to vertebral compression fractures is a quite frequent diagnosis for the pain medicine specialist; KP is a new technique showing an association of a low incidence of complications with a success rate, both on pain control and on vertebral height restoring. In our study, KP proved to be a safe technique with a high success rate, both for pain relief and for vertebral height restoring, with immediate results and important consequences on the patient's quality of life, physical and mental status, with a low incidence of complications due also to the choice of performing this procedure in locoregional anesthesia.

Isolated, benign, intraorbital schwannoma arising from the supraorbital nerve. Case report and review of the literature.

The authors describe the rare case of a 65-year-old man, with absent clinical evidence of von Recklinghausen's disease, harbouring an isolated, benign schwannoma of the right supraorbital nerve. The patient presented a progressive, painless proptosis of the eye for the past 2 years, did not complain of any intra- or periorbital pain, and did not experience any form of visual disturbance or field defect. The lesion was completely resected using a fronto-orbitozygomatic approach. The clinicopathological, radiological and surgical features are discussed, and the literature on supraorbital nerve schwannomas reviewed. To the authors' knowledge the fronto-orbitozygomatic approach has not been previously reported for the removal of supraorbital nerve tumors.

Trigeminal palsy caused by triventricular hydrocephalus. A case report.

A case of left trigeminal palsy caused by triventricular hydrocephalus and completely recovered after ventriculo peritoneal shunt is reported. The case was studied by MR before and after the operation. Preoperative MR showed triventricular hydrocephalus with marked reduction of the left Meckel's cave. Following ventriculo peritoneal shunt the patient showed a complete recovery of the left 5th nerve palsy and NR showed the expansion of the left Meckel's cave. The authors suggest that the trigeminal palsy was due to intracranial hypertension probably associated to a weakness of the lateral wall of the left Meckel's cave. No similar case have been previously reported in the literature.

Posterior lumbar interbody fusion for revision disc surgery: review of 50 cases in which carbon fiber cages were implanted.

OBJECT: The authors undertook a study to assess the value of posterior lumbar interbody fusion (PLIF) in which carbon fiber cages (CFCs) were placed in patients undergoing revision disc surgery for symptoms suggesting neural compression with low-back pain. METHODS: The authors followed their first 50 patients for a maximum of 5 years and a minimum of 6 months after implantation of the CFCs. Patients in whom magnetic resonance (MR) imaging demonstrated "simple" recurrent herniation did not undergo PLIF. Surgery was performed in patients with symptoms of neural root compression, tension signs, and back pain with focal disc degeneration and nerve root distortion depicted on MR imaging compatible with clinical signs and symptoms. In 40 patients (80%) pedicle screws were not used. Clinical outcome was assessed using the Prolo Functional Economic Outcome Rating scale. Fusion outcome was assessed using an established classification. Symptoms in 46 patients (92%) improved after surgery, and given their outcomes, 45 (90%) would have undergone the same surgery again. Two thirds of patients experienced good or excellent outcomes (Prolo score > or = 8) at early and late follow up. There was no difference in clinical outcome between those in whom pedicle screws were and were not implanted (p = 0.83, Mann-Whitney U-test). The fusion rate at 2 years postsurgery was 95%. There were minimal complications, and no patients fared worse after surgery. No patient has undergone additional surgical treratment of the fused intervertebral space. CONCLUSIONS: In this difficult group of patients the aim remains to improve symptoms but not cure the disease. A high fusion rate is possible when using the CFCs. Clinical success depends on selecting patients in whom radiological and clinical criteria accord. Pedicle screws are not necessary if facet joints are preserved, and high fusion rates and clinical success are possible without them.

Correlation between histological grade, MIB-1, p53, and recurrence in 69 completely resected primary intracranial meningiomas with a 6 year mean follow-up.

Sixty-nine intracranial, totally excised meningiomas were immunostained for MIB-1 and p53 protein expression. According to the 1993 WHO criteria, revised by Perry et al., the 69 meningiomas were classified into: grade I = 54 benign meningiomas, grade II = 10 atypical meningiomas, grade III = 5 malignant meningiomas. The patients were followed until death or for an average of 6.7 years. The 69 meningiomas were divided into two groups, according to the absence (n = 42) or presence (n = 27) of recurrences. In the last group we included 3 patients who died of meningioma recurrence. According to the percentage of MIB-1 positively stained cells, meningiomas were divided into three groups: <1% (n = 36), 1-10% (n = 28), >10% (n = 5). We found the MIB-1 labeling index (LI) <1% in 33 grade I (61%) and in 3 grade II (30%) meningiomas. On the other hand, 7 grade II (70%) and all grade III (100%) meningiomas presented a MIB-1 LI >1%. Correlation between histological grade and MIB-1 LI was statistically significant (p = 0.0006). The correlation between MIB-1 LI and follow-up was also highly significant (p < 0.001): the majority of meningiomas which did not recur (32/42 equal to 76%) were characterized by a low (<1%) MIB-1 LI. In the recurrence group MIB-1 LI was significantly higher than in the disease-free patients' group. Moreover, MIB-1 appeared to be a prognostic parameter not strongly related to the histological grade. In fact, it was significantly higher in recurrent histologically benign meningiomas, as compared with benign meningiomas without recurrence (p = 0.0006). Positive p53 protein expression (>1%) was shown in 26/45 meningiomas (57%), with an LI of 1-10% in 18 (40%) and an LI of >10% in 8 (17%) meningiomas. Although the p53 LI tended to be higher in atypical and malignant meningiomas, no significant correlation was found between the p53 expression and the recurrence (p = 0.05). The authors conclude that quantitative MIB-1 labeling is a useful technique in the routine diagnostic assessment of meningiomas, and helpful in obtaining more information about prognosis and thereby in planning the most suitable treatment.

Group-I metabotropic glutamate receptors: hypotheses to explain their dual role in neurotoxicity and neuroprotection.

The role of group-I metabotropic glutamate receptors (mGlu1 and 5) in neurodegeneration is still controversial. While antagonists of these receptors are consistently neuroprotective, agonists have been found to either amplify or attenuate excitotoxic neuronal death. At least three variables affect responses to agonists: (i) the presence of the NR2C subunit in the NMDA receptor complex; (ii) the existence of an activity-dependent functional switch of group-I mGlu receptors, similar to that described for the regulation of glutamate release; and (iii) the presence of astrocytes expressing mGlu5 receptors. Thus, a number of factors, including the heteromeric composition of NMDA receptors, the exposure time to drugs or to ambient glutamate, and the function of astrocytes clearing extracellular glutamate and producing neurotoxic or neuroprotective factors, must be taken into account when examining the role of group-I mGlu receptors in neurodegeneration/neuroprotection.

[Dynamic obstruction to left ventricular outflow during dobutamine stress echocardiography: the probable mechanisms and clinical implications]. / Ostruzione dinamica all'efflusso ventricolare sinistro durante ecocardiografia da stress con dobutamina: probabili meccanismi ed implicazioni cliniche.

We observed the development of left ventricular outflow tract dynamic obstruction in some patients during dobutamine stress echocardiography. The purpose of this study was to identify the possible mechanisms and to consider the clinical implications. From 11/04/94 to 01/09/97 we studied 547 patients; 42 patients developed dynamic obstruction, defined as a late peak Doppler velocity profile that exceeded baseline outflow velocity by at least 1 m/s. The encountered mechanisms were: increased myocardial contractility; systolic anterior motion of the mitral valve; decreased venous return to the left ventricle, and peculiar characteristics of the left ventricular geometry. The results of this study show that the dynamic obstruction is mainly due to the first mechanism and secondarily to some characteristics of the left ventricular geometry. The hypotension observed in a few cases is not related to the dynamic obstruction but to beta 2 receptor hypersensibility to dobutamine. The symptoms, like dyspnea and chest pain, experienced by these patients are related to the dynamic obstruction rather than to the presence of coronary artery disease. In conclusion, we think that patients who develop dynamic obstruction, without wall motion abnormalities, during dobutamine stress echocardiography, may behave pathophysiologically as patients with obstructive hypertrophic cardiomyopathy, in whom diastolic dysfunction and outflow tract obstruction are responsible for symptoms. Therefore, these patients require a pharmacological treatment with beta blockers and/or non-dihydropyridine calcium channel blockers.