Cognitive outcomes following functional neurosurgery in refractory OCD patients: a systematic review.

Neurosurgery is a therapeutic option for patients with refractory obsessive-compulsive disorder who do not respond to previous treatments. Although its efficacy in reducing clinical symptomatology has been proven, few studies have analyzed its effects at the cognitive level. The aim of this systematic review was to describe the cognitive outcomes of functional neurosurgery in patients that went through capsulotomies or cingulotomies. PubMed, Medline, Scopus, PsycInfo, PsyArticles, and Web of Knowledge were searched for studies reporting cognitive outcomes in refractory obsessive-compulsive patients after capsulotomies and cingulotomies. The risk of bias was assessed with the Assessment Tool for Before-After (Pre-Post) Studies With No Control Group tool; 13 studies met inclusion criteria, including 205 refractory obsessive-compulsive disorder patients for both surgical procedures. Results showed a substantial number of studies that did report significant cognitive improvement after surgery, being this improvement specially related to memory and executive functions. The second-most frequent finding is the maintenance of cognitive performance (nor improvement or worsening). From a neuropsychological point of view, this outcome might be considered a success, given that it is accompanied by amelioration of obsessive-compulsive symptoms. Subtle cognitive adverse effects have also been reported. Neurosurgery procedures appear to be safe from a cognitive point of view. Methodological issues must be improved to draw clearer conclusions, but capsulotomies and cingulotomies constitute an effective alternative treatment for refractory obsessive-compulsive disorder patients.

Gamma Knife® stereotactic radiosurgery as a treatment for essential and parkinsonian tremor: long-term experience.

INTRODUCTION: Unilateral Gamma Knife™ stereotactic radiosurgery on the ventral-intermediate nucleus of the thalamus is a minimally invasive neurosurgical option for refractory tremor. We describe the experience of Gamma Knife™ thalamotomy (GKT) in patients with essential tremor (ET) and tremor-dominant Parkinson's disease (PD) at our specialised stereotactic neurosurgery unit. METHODS: We reviewed the cases of patients treated with GKT between January 2014 and February 2018 with a minimum of 12 months' follow-up. We analysed clinical and demographic variables, indication, radiation dose, effectiveness (based on subscales of the Fahn-Tolosa-Marin [FTM] scale and the Movement Disorders Society-Unified Parkinson's Disease Rating Scale [MDS-UPDRS] motor score), and adverse events. RESULTS: Thirteen patients were registered, 6 with a diagnosis of tremor-dominant PD, four with refractory ET, and three with ET and PD. Median age was 78 years (range, 62-83), with seven patients aged over 75 years. Four patients were receiving anticoagulants and two had history of stroke. The maximum radiation dose administered was 130 Gy. Mean (standard deviation) follow-up duration was 30.0 (14.5) months. Significant tremor improvement was observed on the FTM subscales: 63.6% at 12 months and 63.5% at the end of follow-up; MDS-UPDRS tremor items showed improvements of 71.3% at 12 months and 60.3% at the end of follow up. Eleven patients reported significant improvements in quality of life, and 3 reported mild and transient adverse effects. CONCLUSIONS: This is the largest series of patients with essential and parkinsonian tremor treated with GKT and followed up in the long term in Spain. GKT can be safe and effective in the long term in patients with refractory tremor, including in elderly patients and those receiving anticoagulants.

Stereoelectroencephalography in the preoperative assessment of patients with refractory focal epilepsy: experience at an epilepsy centre.

OBJECTIVE: Stereoelectroencephalography (SEEG) is a technique for preoperative evaluation of patients with difficult-to-localise refractory focal epilepsy (DLRFE), enabling the study of deep cortical structures. The procedure, which is increasingly used in international epilepsy centres, has not been fully developed in Spain. We describe our experience with SEEG in the preoperative evaluation of DLRFE. MATERIAL AND METHODS: In the last 8 years, 71 patients with DLRFE were evaluated with SEEG in our epilepsy centre. We prospectively analysed our results in terms of localisation of the epileptogenic zone (EZ), surgical outcomes, and complications associated with the procedure. RESULTS: The median age of the sample was 30 years (range, 4-59 years); 27 patients (38%) were women. Forty-five patients (63.4%) showed no abnormalities on brain MR images. A total of 627 electrodes were implanted (median, 9 electrodes per patient; range, 1-17), and 50% of implantations were multilobar. The EZ was identified in 64 patients (90.1%), and was extratemporal or temporal plus in 66% of the cases. Follow-up was over one year in 55 of the 61 patients undergoing surgery: in the last year of follow-up, 58.2% were seizure-free (Engel Epilepsy Surgery Outcome Scale class I) and 76.4% had good outcomes (Engel I-II). Three patients (4.2%) presented brain haemorrhages. CONCLUSION: SEEG enables localisation of the EZ in patients in whom this was previously impossible, offering better surgical outcomes than other invasive techniques while having a relatively low rate of complications.

Gamma Knife® stereotactic radiosurgery as a treatment for essential and parkinsonian tremor: long-term experience. / Radiocirugía estereotáctica con Gamma Knife® como tratamiento del temblor esencial y parkinsoniano: experiencia a largo plazo.

INTRODUCTION: Unilateral Gamma Knife™ stereotactic radiosurgery on the ventral-intermediate nucleus of the thalamus is a minimally invasive neurosurgical option for refractory tremor. We describe the experience of Gamma Knife™ thalamotomy (GKT) in patients with essential tremor (ET) and tremor-dominant Parkinson's disease (PD) at our specialised stereotactic neurosurgery unit. METHODS: We reviewed the cases of patients treated with GKT between January 2014 and February 2018 with a minimum of 12 months' follow-up. We analysed clinical and demographic variables, indication, radiation dose, effectiveness (based on subscales of the Fahn-Tolosa-Marin [FTM] scale and the Movement Disorders Society-Unified Parkinson's Disease Rating Scale [MDS-UPDRS] motor score), and adverse events. RESULTS: Thirteen patients were registered, 6 with a diagnosis of tremor-dominant PD, four with refractory ET, and three with ET and PD. Median age was 78 years (range, 62-83), with seven patients aged over 75 years. Four patients were receiving anticoagulants and two had history of stroke. The maximum radiation dose administered was 130 Gy. Mean (standard deviation) follow-up duration was 30.0 (14.5) months. Significant tremor improvement was observed on the FTM subscales: 63.6% at 12 months and 63.5% at the end of follow-up; MDS-UPDRS tremor items showed improvements of 71.3% at 12 months and 60.3% at the end of follow up. Eleven patients reported significant improvements in quality of life, and 3 reported mild and transient adverse effects. CONCLUSIONS: This is the largest series of patients with essential and parkinsonian tremor treated with GKT and followed up in the long term in Spain. GKT can be safe and effective in the long term in patients with refractory tremor, including in elderly patients and those receiving anticoagulants.

Clinical practice of language fMRI in epilepsy centers: a European survey and conclusions by the ESNR Epilepsy Working Group.

PURPOSE: To assess current clinical practices throughout Europe with respect to acquisition, implementation, evaluation, and interpretation of language functional MRI (fMRI) in epilepsy patients. METHODS: An online survey was emailed to all European Society of Neuroradiology members (n = 1662), known associates (n = 6400), and 64 members of European Epilepsy network. The questionnaire featured 40 individual items on demographic data, clinical practice and indications, fMRI paradigms, radiological workflow, data post-processing protocol, and reporting. RESULTS: A total of 49 non-duplicate entries from European centers were received from 20 countries. Of these, 73.5% were board-certified neuroradiologists and 69.4% had an in-house epilepsy surgery program. Seventy-one percent of centers performed fewer than five scans per month for epilepsy. The most frequently used paradigms were phonemic verbal fluency (47.7%) and auditory comprehension (55.6%), but variants of 13 paradigms were described. Most centers assessed the fMRI task performance (75.5%), ensured cognitive-task adjustment (77.6%), trained the patient before scanning (85.7%), and assessed handedness (77.6%), but only 28.6% had special paradigms for patients with cognitive impairments. fMRI was post-processed mainly by neuroradiologists (42.1%), using open-source software (55.0%). Reporting was done primarily by neuroradiologists (74.2%). Interpretation was done mainly by visual inspection (65.3%). Most specialists (81.6%) were able to determine the hemisphere dominance for language in more than 75% of exams, attributing failure to the patient not performing the task correctly. CONCLUSION: This survey shows that language fMRI is firmly embedded in the preoperative management of epilepsy patients. The wide variety of paradigms and the use of non-CE-marked software underline the need for establishing reference standards.

Stereoelectroencephalography in the preoperative assessment of patients with refractory focal epilepsy: Experience at an epilepsy centre. / Estereoelectroencefalografía en la evaluación prequirúrgica de epilepsias focales refractarias: experiencia de un centro de epilepsia.

OBJECTIVE: Stereoelectroencephalography (SEEG) is a technique for preoperative evaluation of patients with difficult-to-localise refractory focal epilepsy (DLRFE), enabling the study of deep cortical structures. The procedure, which is increasingly used in international epilepsy centres, has not been fully developed in Spain. We describe our experience with SEEG in the preoperative evaluation of DLRFE. MATERIAL AND METHODS: In the last 8 years, 71 patients with DLRFE were evaluated with SEEG in our epilepsy centre. We prospectively analysed our results in terms of localisation of the epileptogenic zone (EZ), surgical outcomes, and complications associated with the procedure. RESULTS: The median age of the sample was 30 years (range, 4-59 years); 27 patients (38%) were women. Forty-five patients (63.4%) showed no abnormalities on brain MR images. A total of 627 electrodes were implanted (median, 9 electrodes per patient; range, 1-17), and 50% of implantations were multilobar. The EZ was identified in 64 patients (90.1%), and was extratemporal or temporal plus in 66% of the cases. Follow-up was over one year in 55 of the 61 patients undergoing surgery: in the last year of follow-up, 58.2% were seizure-free (Engel Epilepsy Surgery Outcome Scale class I) and 76.4% had good outcomes (Engel I-II). Three patients (4.2%) presented brain haemorrhages. CONCLUSION: SEEG enables localisation of the EZ in patients in whom this was previously impossible, offering better surgical outcomes than other invasive techniques while having a relatively low rate of complications.

SEOM clinical guidelines for diagnosis and treatment of glioblastoma (2017).

Glioblastoma (GB) is the most common brain malignancy and accounts for over 50% of all high-grade gliomas. Radiotherapy (RT) with concomitant and adjuvant temozolomide (TMZ) chemotherapy is the current standard of care for patients with newly diagnosed GB up to age 70. Recently, a new standard of care has been adopted for elderly patients (≥ 65 years) based on short course of RT and TMZ. Several clinically relevant molecular markers that assist in diagnosis and prognosis have recently been identified. The treatment for recurrent GB is not well defined, and decision-making is usually based on prior strategies as well as several clinical and radiological factors. The presence of neurologic deficits and seizures can significantly impact quality of life.

Functional surgery in pediatric drug-resistant posterior cortex epilepsy: Electro-clinical findings, cognitive and seizure outcome.

PURPOSE: Epilepsies originated from the occipital, parietal and/or the posterior edge of the temporal lobe are grouped together into posterior cortex epilepsy (PCE). Our objective was firstly to describe electro-clinical and imaging findings in the presurgical evaluation of children with PCE, and secondly to identify potential factors associated with surgical and cognitive outcomes. METHOD: From the total of patients referred to the Epilepsy Monitoring Unit of 'Hospital Universitario Niño Jesús' from 2003 to 2016, 55 had drug-resistant PCE. Different variables obtained from the multimodal presurgical work-up were analyzed among patients achieving seizure freedom after surgery (ILAE class 1) and patients with persistent seizures. Categorical variables were compared with Fishers exact test and numeric variables with t-Student for independent samples, and multiple logistic regression were used to analyze predictive values. RESULTS: Median duration of epilepsy until surgery was 5 years [3-10 years]. Fifty patients showed lesions in the MRI, and 62.5% had concordant MRI-PET corregistration. 37 (67%) patients were operated (lesionectomy in 21 subjects, tailored resection based on intracranial studies in 16), and 23 (62,2%) reached ILAE class 1, with a mean follow-up period of 3.51 [1-12] years. A lower number of basal seizures and antiepileptic drugs, a well-defined lesion on the MRI, an epileptogenic zone (EZ) restricted to the posterior quadrant and the normalization of postsurgical EEGs were associated with seizure freedom (p<0.05). Additionally, 65% of patients had a long-term improvement of cognitive performances. CONCLUSIONS: Epilepsy surgery should be considered in children with drug-resistant PCE, especially in those with a restricted EZ.

Kaposi sarcoma secondary to endogenous adrenocorticotropic hormone-dependent Cushing syndrome.

Kaposi sarcoma (KS) is an angioproliferative tumour that develops as a result of an infection by human herpesvirus 8, which is considered a necessary cause but not sufficient. Other factors - genetic, immunological and environmental - might play a role in the development of the disease. We report a case of KS secondary to endogenous Cushing syndrome (ECS) due to a pituitary adenoma, an association that has been reported only once. We also conducted a search through the Medline and PubMed databases for cases involving KS and ECS, finding only three additional cases that shared common clinical and prognostic features with ours. ECS might favour the development of KS due to immunosuppression. Dermatologists and other clinicians should be aware of this association, as it might be an underdiagnosed condition. It also has an important impact on the management of KS, and based on this review it relies on a good prognosis when ECS is well controlled.

Achromatic synesthesias - a functional magnetic resonance imaging study.

Grapheme-color synesthetes experience consistent, automatic and idiosyncratic colors associated with specific letters and numbers. Frequently, these specific associations exhibit achromatic synesthetic qualities (e.g. white, black or gray). In this study, we have investigated for the first time the neural basis of achromatic synesthesias, their relationship to chromatic synesthesias and the achromatic congruency effect in order to understand not only synesthetic color but also other components of the synesthetic experience. To achieve this aim, functional magnetic resonance imaging experiments were performed in a group of associator grapheme-color synesthetes and matched controls who were stimulated with real chromatic and achromatic stimuli (Mondrians), and with letters and numbers that elicited different types of grapheme-color synesthesias (i.e. chromatic and achromatic inducers which elicited chromatic but also achromatic synesthesias, as well as congruent and incongruent ones). The information derived from the analysis of Mondrians and chromatic/achromatic synesthesias suggests that real and synesthetic colors/achromaticity do not fully share neural mechanisms. The whole-brain analysis of BOLD signals in response to the complete set of synesthetic inducers revealed that the functional peculiarities of the synesthetic brain are distributed, and reflect different components of the synesthetic experience: a perceptual component, an (attentional) feature binding component, and an emotional component. Additionally, the inclusion of achromatic experiences has provided new evidence in favor of the emotional binding theory, a line of interpretation which constitutes a bridge between grapheme-color synesthesia and other developmental modalities of the phenomenon.

Usefulness of high b-value diffusion-weighted MRI in the diagnosis of Creutzfeldt-Jakob disease.

BACKGROUND: current diagnostic criteria of probable Creutzfeldt-Jakob disease (CJD) include a combination of clinical, EEG and analytic data. Recent data indicate that brain MRI including fluid-attenuated inversion recovery (FLAIR) and diffusion-weighted imaging (DWI) sequences can be a valid and reliable tool for the diagnosis of CJD. We describe our experience with high b-value (3000s/mm(2)) diffusion-weighted imaging (DWI) in patients with probable or definite CJD and compare it with standard b-value (1000s/mm(2)) DWI. METHODS: we performed a retrospective analysis of patients admitted to our Hospital Service between 2002 and 2008 with a final diagnosis of probable or definite CJD. Patients were examined using either a 1.5 Tesla or a 3 Tesla MRI. The MRI protocol included T1-weigthed spin-echo sequences, T2-weighted fast spin-echo, FLAIR and DWI sequences with high b-value and standard b-value. RESULTS: during the study period there were 7 patients with probable or definite CJD. Only 3 patients (43%) showed changes on FLAIR sequence consistent with CJD. All the cases were detected with high b-value DWI, including 2 cases (28%) that would have been missed using standard b-value (1000s/mm(2)) DWI. In all the patients the changes were more conspicuous and extensive at high b-value DWI (b=3000s/mm(2)). CONCLUSION: our data indicate that high b-value DWI may improve the sensitivity of brain MRI for the diagnosis of CJD, allowing the detection of some cases that would have been overlooked by conventional sequences.

Magnetic resonance techniques for the brainstem.

Imaging of the brainstem is a real challenge from the technical point of view, because it is a very complex structure. There are gray matter nuclei with vital functions mixed to white matter tracts with very important connections, as the Cortico-Spinal Tract (CST). On the other hand, the situation of the brainstem, near bony structures and surrounded by vessels adds even more difficulties due to the lower homogeneity of the field and the flow effect of both the vessels and the CSF. Structural imaging with 3-D sequences, either in T1 or T2 has been a major advance in the diagnosis of small lesions of the brainstem. Advanced techniques such as Spectroscopy, Diffusion and Perfusion sequences add important information that can reliably handle many lesions in which biopsy is a very high risk. Therefore, proper management of imaging techniques is particularly important in lesions of the brainstem.

The striatum beyond reward: caudate responds intensely to unpleasant pictures.

The involvement of striatum in affective processes has been consistently reported in recent years. However, studies within this field have mainly focused on positive affect, revealing the involvement of striatum in reward situations. The present research aimed to explore the involvement of striatum in negative affect through fMRI. To that aim, participants (n=18) were presented with negative, positive and neutral pictures while they performed an indirect task, a strategy repeatedly recommended to avoid cognitive interferences. Positive and negative stimuli did not differ in their arousal levels, as assessed by participants themselves. Analyses of variance showed that caudate nucleus was sensitive to the emotional content of stimulation, negative pictures eliciting greater caudate responses than positive and neutral. Regression analyses indicated that both valence and arousal content of visual stimuli synergically contributed to explain the activation of caudate, whose strong response to negative pictures supports models proposing striatum as a key element in withdrawal situations.

[Hypothalamic hamartoma: clinical characteristics. Electroencephalogram and brain magnetic resonance imaging in 10 patients]. / Hamartomas hipotalámicos: características clínicas, electroencefalograma y resonancia magnética cerebral en 10 pacientes.

INTRODUCTION: We describe clinical findings and electroencephalogram (EEG) in patients with hypothalamic hamartoma and epilepsy. METHODS: Our group includes 10 patients (eight males) with mean age of 17.8 years (range: 7-39) and hypothalamic hamartoma in the brain magnetic resonance imaging (MRI). We analyzed clinical data, seizure semiology, MRI and EEG findings of the neuropsychological study. RESULTS: Nine patients had gelastic seizures, that initiated at a mean age of 17.1 months (2 days-5 years). Other types of seizure were observed in seven and five had behavior disorders. Intelligence quotient (IQ) was below the mean range in three. Three children had precocious puberty and thyroid dysfunction. One patient did not have epilepsy. MRI showed a hypothalamic lesion suggesting hamartoma associated to a dysplastic lesion in one case. The interictal EEG was normal in 2 cases and revealed epileptiform abnormalities, consisting of spikes or sharp waves, in temporal regions, frontal, fronto-temporal regions and central-parietal in 8. Three patients had paroxysmal discharges of generalized fast activity (> 10 Hz) during non-REM sleep. Forty seizures were recorded, 31 had an ictal EEG pattern while the EEG was normal in 9. CONCLUSIONS: In our group gelastic seizures were an early and constant finding except in one patient. Partial complex seizures, behavior alteration and cognitive decline were frequent. Video-EEG monitoring allows us to identify interictal and ictal patterns that have been described in hypothalamic hamartomas.

Magnetic resonance angiography shows dilatation of the middle cerebral artery after infusion of glyceryl trinitrate in healthy volunteers.

Previous studies have reported dilatation of the middle cerebral artery (MCA) during acute glyceryl trinitrate (GTN)-induced headache, using imaging techniques such as transcranial Doppler (TCD), positron emission tomography (PET) and single photon emission computerized tomography (SPECT). In the present study we aimed to evaluate whether magnetic resonance angiography (MRA) may be used to examine the effect of GTN on the MCA, with respect to changes in diameter and cross-sectional area in healthy volunteers. In addition, we wanted to determine the intra- and inter-observer variation of the method. In a randomized, double blind, crossover study 12 healthy volunteers received intravenous infusion of GTN (0.5 microg/kg/min for 20 min) or placebo. Using 1.5 Tesla MRA, we recorded changes in the diameter and cross-sectional area of MCA before, during and after infusion of GTN. The MRA images were evaluated by two blinded, independent observers/neuroradiologists. The primary endpoints were the differences in the AUC for diameter and cross-sectional area of the MCA between the two experimental conditions and the intra- and inter-observer variation. The areas under the curve (AUC) of the MCA diameter and cross-sectional area were significantly greater after GTN than after placebo (P < 0.05). The intra-observer variation (day-to-day) at baseline was 8.3% and 10.9% for the two observers. The mean inter-observer variation of the cross-sectional MCA area was 15.5% and for the diameter measurements 8%. The present study shows that the MRA method gives a reliable semi-quantitative index of the vascular changes in the intra-cerebral arteries after infusion of GTN and may be useful for headache research.

[Regional correlations between MR imaging perfusion and SPECT in Alzheimer's disease]. / Correlaciones regionales entre el estudio de la perfusión mediante resonancia magnética y SPECT en la enfermedad de Alzheimer.

INTRODUCTION: Dynamic susceptibility contrast (DSC) is a magnetic resonance (MR) technique that provides an estimation of cerebral blood flow (CBF) through the obtention of a cerebral blood volume map. As observed with nuclear medicine methods, DSC MR studies have demonstrated a temporoparietal hypoperfusion in Alzheimer's disease (AD). However, the concurrent validity of DSC and nuclear medicine techniques has not been sufficiently investigated. PATIENTS AND METHODS: A single-photon emission computed tomography (SPECT) and a DSC MR perfusion study were performed consecutively in 14 AD patients. Expert based qualitative assessments of CBF were carried out in eight regions of interest (ROI). RESULTS: Level of agreement in the studied ROI was highly variable. Presence of large blood vessels and patient head movement were possibly the main causes of this variability. Nevertheless, when interrater variability was eliminated, intraclass correlation coefficients became more uniform, ranging from 0.32 to 0.71. CONCLUSIONS: DSC MR imaging was concordant with SPECT at the different cerebral lobes of AD patients. The positive findings should be confirmed under better technical and methodological conditions.

Neurosyphilis masquerading as corticobasal degeneration.

We report on a patient with a syndrome resembling corticobasal ganglionic degeneration (CBD), including slight cognitive impairment, asymmetric akinesia, rigidity with myoclonus, and arm levitation, which can be one of the features of alien limb phenomenon; however, further diagnostic testing was consistent with neurosyphilis. Syphilis, "the great imitator," may also masquerade as CBD. Because neurosyphilis is treatable, it should be considered in the workup of patients with cognitive impairment and motor signs of CBD.

[Functional neuroimaging applied to the study of neuropsychological rehabilitation]. / Aplicación de la neuroimagen funcional al estudio de la rehabilitación neuropsicológica.

OBJECTIVE: We review the main aspects of functional recovery after brain injury as well as neuroimaging characteristics that make it relevant and useful to assess these changes. We also review some issues regarding recovery of motor and sensory functions, language and visuo spatial processes, and we discuss the methods used in this field, the difficulties found, and future implications. DEVELOPMENT: One of the main aspects in the study of the brain is the capacity to reorganize different functions in order to compensate for the deficits after a lesion in the central nervous system. The study of these adaptive processes is important in a clinical field as well as for basic research, as it is a clear example of brain plasticity. All the findings show how the study of the plastic phenomena or functional reorganization will allow us to better know how the brain works after a lesion. CONCLUSION: The use and combination of the new functional neuroimaging techniques gives the opportunity to register the reorganization of the brain with a high temporal and spatial resolution. It will give also an objective measure to assess the effectiveness of the rehabilitation programs. It will allow to identify different variables related to rehabilitation outcome and will guide effectively the selection of different rehabilitation approaches.