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The structural properties of zinc ferrite nanoparticles with spinel structure dispersed in a highly porous SiO2 aerogel matrix were compared with a bulk zinc ferrite sample. In particular, the details of the cation distribution between the octahedral (B) and tetrahedral (A) sites of the spinel structure were determined using X-ray absorption spectroscopy. The analysis of both the X-ray absorption near edge structure and the extended X-ray absorption fine structure indicates that the degree of inversion of the zinc ferrite spinel structures varies with particle size. In particular, in the bulk microcrystalline sample, Zn2+ ions are at the tetrahedral sites and trivalent Fe3+ ions occupy octahedral sites (normal spinel). When particle size decreases, Zn2+ ions are transferred to octahedral sites and the degree of inversion is found to increase as the nanoparticle size decreases. This is the first time that a variation of the degree of inversion with particle size is observed in ferrite nanoparticles grown within an aerogel matrix. [ABSTRACT FROM AUTHOR]

The article highlights the need to standardise measurement of motor complications in clinical trials for early Parkinson's disease. Numerous clinical trials have been conducted to compare treatments for their ability to delay, prevent, or reduce the severity of motor complications. Measuring motor complications is challenging because of the number of potentially important aspects, including frequency, intensity, predictability, phenomenology, as well as the need to rely on a subject's understanding and awareness of these phenomena because they are transient, and often cannot be observed directly by study personnel.

Purpose: Urological management of Cloacal Malformation (CM) focuses on preserving renal function and continence. Study aim was to analyze urinary and intestinal outcomes in CM patients, considering the length of common channel (CC) and presence of occult spinal dysraphism (OSD). Methods: Retrospective review of CM treated at our institution by a multidisciplinary team from 1999 to 2020. Patients with follow-up < 2.5 years were excluded. Length of CC, renal function, urinary and bowel outcomes, presence of associated anomalies (especially OSD) were evaluated. Results: Twenty patients were included, median age at follow-up: 8 years (4–15). A long CC > 3 cm was described in 11 (55%). Chronic kidney disease was found in 3 patients. Urinary continence was achieved in 8/20 patients, dryness (with intermittent catheterization) in 9/20. Fecal continence was obtained in 3/20, cleanliness in 14 (under bowel regimen). OSD was present in 10 patients (higher prevalence in long-CC, 73%). Among OSD, 1 patient reached fecal continence, 7 were clean; 2 achieved urinary continence, while 6 were dry. Conclusions: Length of CC and OSD may affect urinary and fecal continence. An early counseling can improve outcome at long-term follow-up. Multidisciplinary management with patient centralization in high grade institutions is recommended to achieve better results. [ABSTRACT FROM AUTHOR]

Background and purpose There is increasing evidence that deep brain stimulation ( DBS) of the globus pallidus internus ( GPi) is effective in patients with idiopathic or inherited generalized dystonia. There is comparatively less experience about the effects of GPi DBS on acquired dystonia, particularly dystonia due to cerebral palsy ( DCP). Clinical and demographic outcome predictors for DBS in dystonia syndromes are also poorly defined. Our aim was to examine the efficacy and safety of GPi DBS for the treatment of generalized DCP. Methods Fifteen patients with DCP up to 6.2 years after DBS surgery were studied. Only mild limb spasticity or mild static brain magnetic resonance imaging abnormalities were acceptable for inclusion. Dystonia severity and disability were assessed by the Burke−Fahn−Marsden dystonia rating scale (BFMDRS), and health-related quality of life was assessed by the Short Form General Health Survey ( SF-36) scale. The amount of energy delivered was calculated, and adverse events and side effects were collected. Results At last follow-up, BFMDRS motor score improved on average by 49.5%, and the disability score improved by 30%. Health-related quality of life improved in most patients. Age at implant, age at onset and disease duration did not correlate to outcome, whilst higher pre-operative dystonia severity and occurrence of spasticity were associated with poorer outcome. The patients received a stable amount of energy after the first 2 years post-implant and throughout all the observation period. There were few serious adverse events or side effects. Conclusions The outcome was encouraging in the majority of DCP patients, with a stable outlook and a good safety profile. [ABSTRACT FROM AUTHOR]

The biological mechanisms associated with the development and rupture of intracranial aneurysms are not fully understood. To clarify the role of VEGF and the related receptors in the pathophysiology of aneurysm, immunostaining for VEGF, VEGFR1 and VEGFR2 was performed on specimens from six unruptured aneurysms and on two specimens of normal arteries wall as a control. The results were correlated with NO concentration of CSF collected during surgery from 8 patients affected by unruptured aneurysms and in 11 control patients. The immunohistochemical data showed a different pattern of VEGF/VEGFR1/VEGFR2 in aneurysms when compared with control. The results of this preliminary study suggest an imbalance of VEGF, VEGFR1 and VEGFR2, and the interaction of VEGF and NO in the pathophysiology of unruptured aneurysms. Our data support the hypothesis of aneurysm formation associated with a loss of expression of VEGFR1, moderate expression of VEGFR2 and high concentration of nitrate. [ABSTRACT FROM AUTHOR]

The objective was to evaluate the outcome of microsurgical “pure” lesionectomy in patients with supratentorial cavernous angiomas presenting with seizures. For this retrospective study 163 patients with cavernoma-related epileptic seizures were selected. They all underwent surgery in a single institution between 1988 and 2003. A microsurgical frame/frameless guided minimally invasive transulcal “pure” lesionectomy was performed. The haemosiderin stained gliotic brain parenchyma that was usually found surrounding the lesion was not removed. Among the 99 patients with epilepsy and longer clinical history, 68 (68.7%) were found completely to be seizure-free, 10 (10.1%) presented sporadic and less frequent seizures and 17 (17.1%) remained unchanged. Sixty-three out of 64 (98.4%) patients who experienced only single or sporadic seizures were found to be completely seizure-free after surgery. Five patients were lost at follow-up (mean 48 months, range 0.5–14 years). Long-term morbidity was 1.8%. Mortality was null. No haemorrhagic episodes were observed during follow-up. Pure lesionectomy prevents bleeding and development of epilepsy in patients that receive early surgery after the epileptic onset. In most of the epileptic patients with a good concordance between the electroclinical data and the location of the angioma, good results can be achieved by this kind of surgery so that more invasive and costly studies to find and remove the epileptogenic cerebral parenchyma seem justified only after lesionectomy fails. [ABSTRACT FROM AUTHOR]

Introduction: Caregiver burden is high among caregivers of PD patients (CPD). Neuropsychiatric symptoms are leading contributors to CPD burden, but whether different symptoms differentially impact domains of caregiver burden is not known. Our objective was to examine which neuropsychiatric symptoms and demographic factors contribute to different domains of caregiver burden in PD. Methods: This was a cross-sectional online survey study. Participants were recruited from the Fox Insight (FI) study and were eligible if they identified themselves as a CPD. The primary outcome was the Caregiver Burden Inventory (CBI) total score and its 5 sub-domain scores. The Neuropsychiatric Inventory Questionnaire (NPI-Q) assessed caregiver-reported neuropsychiatric symptoms in the care recipient. Multivariable linear regression models were used to characterize the associations between NPI-Q symptom severity scores and CBI scores. Covariates were caregiver age, sex, education, and caregiving duration. Results: The sample consisted of 450 CPD, mean age 65.87 (SD 10.39) years, 74% females. After adjusting for covariates, CBI total score was predicted by NPI-Q total score (β = 1.96, p < 0.001); model adjusted R2 = 39.2%. Anxiety severity had the largest effect size [standardized β (sβ) = 0.224] on the time-dependency domain, which was also associated with female sex (sβ = − 0.133) and age (sβ = 0.088). Severity of disinhibition (sβ = 0.218), agitation (sβ = 0.199), and female sex (sβ = 0.104) were associated with greater emotional burden. Conclusion: Our findings indicate that demographic characteristics and specific neuropsychiatric symptoms contribute differentially to domains of caregiver burden. Tailored interventions to support CPD are needed. [ABSTRACT FROM AUTHOR]

Objective and importance. Spinal epidural haematoma (SEH) following implantation of an epidural spinal cord electrode is a very rare complication but one that must not be overlooked. This case is unusual because of the almost “holocord” extension of the haematoma and the excellent recovery obtained by prompt surgical treatment.Clinical presentation. A 69 years old man with normal serum coagulation parameters was submitted to spinal cord stimulation (SCS) for chronic pain syndrome. After a minimal L1 laminotomy the patient developed paraplegia due to a large haematoma at D4-L2.Intervention. Surgical removal of the entire clot by a D4-L2 laminectomy was performed immediately.Conclusion. Large epidural haematoma can result from SCS and this complication may be cured by appropriate and prompt surgery. [ABSTRACT FROM AUTHOR]

Aims: Paediatric low‐grade gliomas (pLGGs) are a heterogeneous group of brain tumours associated with a high overall survival: however, they are prone to recur and supratentorial lesions are difficult to resect, being associated with high percentage of disease recurrence. Our aim was to shed light on the biology of pLGGs. Methods: We performed microRNA profiling on 45 fresh‐frozen grade I tumour samples of various histological classes, resected from patients aged ≤16 years. We identified 93 microRNAs specifically dysregulated in tumours as compared to non‐neoplastic brain tissue. Pathway analysis of the microRNAs signature revealed PI3K/AKT signalling as one of the centrally enriched oncogenic signalling. To date, activation of the PI3K/AKT pathway in pLGGs has been reported, although activation mechanisms have not been fully investigated yet. Results: One of the most markedly down‐regulated microRNAs in our supratentorial pLGGs cohort was miR‐139‐5p, whose targets include the gene encoding the PI3K's (phosphatidylinositol 3‐kinase) catalytic unit, PIK3CA. We investigated the role of miR‐139‐5p in regulating PI3K/AKT signalling by the use of human cell cultures derived from supratentorial pLGGs. MiR‐139‐5p overexpression inhibited pLGG cell proliferation and decreased the phosphorylation of PI3K target AKT and phosphorylated‐p70 S6 kinase (p‐p70 S6K), a hallmark of PI3K/AKT/mTORC1 signalling activation. The effect of miR‐139‐5p was mediated by PI3K inhibition, as suggested by the decrease in proliferation and phosphorylation of AKT and p70 S6K after treatment with the direct PI3K inhibitor LY294002. Conclusions: These findings provide the first evidence that down‐regulation of miR‐139‐5p in supratentorial pLGG drives cell proliferation by derepressing PI3K/AKT signalling. [ABSTRACT FROM AUTHOR]

This article reviews and summarizes 200 years of Parkinson's disease. It comprises a relevant history of Dr. James Parkinson's himself and what he described accurately and what he missed from today's perspective. Parkinson's disease today is understood as a multietiological condition with uncertain etiopathogenesis. Many advances have occurred regarding pathophysiology and symptomatic treatments, but critically important issues are still pending resolution. Among the latter, the need to modify disease progression is undoubtedly a priority. In sum, this multiple-author article, prepared to commemorate the bicentenary of the shaking palsy, provides a historical state-of-the-art account of what has been achieved, the current situation, and how to progress toward resolving Parkinson's disease. © 2017 International Parkinson and Movement Disorder Society. [ABSTRACT FROM AUTHOR]

Background and purpose The presentation of Parkinson's disease patients with mutations in the LRRK2 gene ( PDLRRK2) is highly variable, suggesting a strong influence of modifying factors. In this context, inflammation is a potential candidate inducing clinical subtypes. Methods An extensive battery of peripheral inflammatory markers was measured in human serum in a multicentre cohort of 142 PDLRRK2 patients from the MJFF LRRK2 Consortium, stratified by three different subtypes as recently proposed for idiopathic Parkinson's disease: diffuse/malignant, intermediate and mainly pure motor. Results Patients classified as diffuse/malignant presented with the highest levels of the pro-inflammatory proteins interleukin 8 ( IL-8), monocyte chemotactic protein 1 ( MCP-1) and macrophage inflammatory protein 1-β ( MIP-1-β) paralleled by high levels of the neurotrophic protein brain-derived neurotrophic factor ( BDNF). It was also possible to distinguish the clinical subtypes based on their inflammatory profile by using discriminant and area under the receiver operating characteristic curve analysis. Conclusions Inflammation seems to be associated with the presence of a specific clinical subtype in PDLRRK2 that is characterized by a broad and more severely affected spectrum of motor and non-motor symptoms. The pro-inflammatory metabolites IL-8, MCP-1 and MIP-1-β as well as BDNF are interesting candidates to be included in biomarker panels that aim to differentiate subtypes in PDLRRK2 and predict progression. [ABSTRACT FROM AUTHOR]

Objective: To test the hypothesis that variability in SNCA Rep1, a polymorphic dinucleotide microsatellite in the promoter region of the gene encoding α-synuclein, modifies the association between head injury and Parkinson's disease (PD) risk.Methods: Participants in the Farming and Movement Evaluation (FAME) and the Study of Environmental Association and Risk of Parkinsonism using Case-Control Historical Interviews (SEARCH), 2 independent case-control studies, were genotyped for Rep1 and interviewed regarding head injuries with loss of consciousness or concussion prior to Parkinson's disease (PD) diagnosis. Logistic regression modeling adjusted for potential confounding variables and tested interaction between Rep1 genotype and head injury.Results: Consistent with prior reports, relative to medium-length Rep1, short Rep1 genotype was associated with reduced PD risk (pooled odds ratio [OR], 0.7; 95% confidence interval [CI], 0.5-0.9), and long Rep1 with increased risk (pooled OR, 1.4; 95% CI, 0.95-2.2). Overall, head injury was not significantly associated with PD (pooled OR, 1.3; 95% CI, 0.9-1.8). However, head injury was strongly associated with PD in those with long Rep1 (FAME OR, 5.4; 95% CI, 1.5-19; SEARCH OR, 2.3; 95% CI, 0.6-9.2; pooled OR, 3.5; 95% CI 1.4-9.2, p-interaction = 0.02). Individuals with both head injury and long Rep1 were diagnosed 4.9 years earlier than those with neither risk factor (p = 0.03).Interpretation: While head injury alone was not associated with PD risk, our data suggest head injury may initiate and/or accelerate neurodegeneration when levels of synuclein are high, as in those with Rep1 expansion. Given the high population frequency of head injury, independent verification of these results is essential. [ABSTRACT FROM AUTHOR]

F. Orzan, S. Pellegatta, P. L. Poliani, F. Pisati, V. Caldera, F. Menghi, D. Kapetis, C. Marras, D. Schiffer and G. Finocchiaro (2011) Neuropathology and Applied Neurobiology 381-394 ? Proteins of the Polycomb repressive complex 2 (PRC2) are epigenetic gene silencers and are involved in tumour development. Their oncogenic function might be associated with their role in stem cell maintenance. The histone methyltransferase Enhancer of Zeste 2 ( EZH2) is a key member of PRC2 function: we have investigated its expression and function in gliomas. EZH2 expression was studied in grade II-IV gliomas and in glioma stem?like cells (GSC) by quantitative PCR and immunohistochemistry. Effects of EZH2 down?regulation were analysed by treating GSC with the histone deacetylase (HDAC) inhibitor suberoylanide hydroxamic acid (SAHA) and by shRNA. DNA microarray analysis showed that EZH2 is highly expressed in murine and human GSC. Real?time PCR on gliomas of different grade ( n?=?66) indicated that EZH2 is more expressed in glioblastoma multiforme (GBM) than in low?grade gliomas ( P?=?0.0013). This was confirmed by immunohistochemistry on an independent set of 106 gliomas. Treatment with SAHA caused significant up?regulation of PRC2 predicted target genes, GSC disruption and decreased expression of EZH2 and of the stem cell marker CD133. Inhibition of EZH2 expression by shRNA was associated with a significant decrease of glioma proliferation. The data suggest that EZH2 plays a role in glioma progression and encourage the therapeutic targeting of these malignancies by HDAC inhibitors. [ABSTRACT FROM AUTHOR]

Two patients with uncontrollable aggressive behaviour underwent the placement of stimulating leads in the posterior hypothalamus (pHyp). One patient had also multifocal refractory epilepsy. Microrecordings were obtained in both patients during surgery under general anaesthesia. Firing rate, interspike intervals and oscillatory discharge patterns were analysed in 14 neurons. A mean discharge rate of 19 Hz, and oscillatory activity at 7–8 Hz were recorded in the first patient with aggressiveness and epilepsy. In the second patient the mean firing rate was 10 Hz, with evidence of both tonic and random firing patterns. Previous studies in patients with cluster headache showed that a discharge rate around 20 Hz and lack of a specific rhythmic pattern were the most consistent characteristics of neuronal discharge in this area. Our present findings therefore would suggest that the pattern of discharge of neurons in the pHyp should be evaluated with reference to the presence of concurrent pathology. [ABSTRACT FROM AUTHOR]

Trigeminal neuralgia is a disorder characterized by paroxysmal pain arising in one or more trigeminal branches; it is commonly reported in multiple sclerosis. In multiple sclerosis patients the ophthalmic branch may be frequently involved and the risks carried by neurosurgical ablative procedures are higher including major adverse effects such as corneal reflex impairment and keratitis. The objective of this works is to assess the role of posterior hypothalamus neuromodulation in the treatment of trigeminal neuralgia in multiple sclerosis patients. Five multiple sclerosis patients suffering from refractory recurrent trigeminal neuralgia involving all three trigeminal branches underwent deep brain stimulation of the posterior hypothalamus. The rationale of this intervention emerges from our earlier success in treating pain patients suffering from trigeminal autonomic cephalalgias. After follow-up periods that ranged from 1 to 4 years after treatment, the paroxysmal pain arising from the first trigeminal branch was controlled, whereas the recurrence of pain in the second and third trigeminal branches necessitated repeated thermorhizotomies to control in pain in two patients after 2 years of follow-up. In conclusion, deep brain stimulation may be considered as an adjunctive procedure for treating refractory paroxysmal pain within the first trigeminal division so as to avoid the complication of corneal reflex impairment that is known to follow ablative procedures. [ABSTRACT FROM AUTHOR]

Objectives: Our objective is to carry out a clinical study of the performance of the preliminary European classification criteria for Sjögren Syndrome and that of the criteria proposed by the American European Consensus Group.Methods: Eighty-eight patients who had undergone a biopsy of the salivary gland on suspicion that they were suffering from Sjögren Syndrome were studied by two independent rheumatologists. Two pathologists independently revised the biopsies without knowing the diagnosis. With all of this information, the clinicians established, or did not establish, a diagnosis of primary Sjögren Syndrome or secondary Sjögren Syndrome. Finally, it was analysed whether or not the patients met the American European Consensus Group classification criteria and the preliminary European criteria for primary Sjögren Syndrome and secondary Sjögren Syndrome, and their sensitivity and specificity with respect to the clinical diagnosis were determined.Results: Clinicians estimated that 35 cases (39.8%) had primary Sjögren Syndrome (kappa 0.718) and 17 cases (19.3%) had secondary Sjögren Syndrome (kappa 0.761). The specificity and sensitivity of American European Consensus Group criteria, with regard to the clinical diagnosis, for primary Sjögren Syndrome were 97.2% and 48.6%, respectively. For secondary Sjögren Syndrome, the specificity was 97.2% and the sensitivity 64.7%. The preliminary European criteria for primary Sjögren Syndrome demonstrated a lesser specificity (75%), but a higher sensitivity (65.7%). In secondary Sjögren Syndrome the specificity reached 97.2% with sensitivity at 70.6%.Conclusions: These results underline the difficulty in applying the Sjögren Syndrome classification criteria from the American European Consensus Group and the preliminary European criteria, in the diagnosis of individual patients. [ABSTRACT FROM AUTHOR]

Parkinson's disease (PD) is a progressive disturbances of movement that affects mainly the motor system. Prolonged pharmacological administration may result in insufficient control of symptoms and significant side effects. Deep brain stimulation (DBS), targeted at the STN, is a recent surgical procedure that, according to the symptoms response, allows modification of stimulation parameters; its effects are also reversible. In this paper management of surgical patients is reported. It includes patient selection, inclusion and exclusion criteria, postoperative clinical protocol. The evaluation rating scale such as UPDRS, Dyskinesias Rating Scale and Self-Reporting Questionnaire usually administrated on PD patients are analyzed. Surgical inclusion criteria are (1) idiopathic PD, (2) IV or V Hoehn-Yahr stage, (3) severe motor disability, and (4) no dementia or psychiatric abnormalities. Postoperative clinical protocol is analyzed and parameter of stimulation after surgery and at the follow up are reported. Generally DBS allows an improvement of rigidity and tremor; bradykinesia also improves with high frequency stimulation. Results obtained by continuous stimulation show a mean improvement of UPDRS of about 60% and a significant reduction in the drug intake. [ABSTRACT FROM AUTHOR]

The purpose of this study is to evaluate the utility of three-dimensional computed tomographic angiography (3D-CTA) as the primary diagnostic investigation in patients with spontaneous subarachnoid haemorrhage (SAH) from anterior circulation aneurysms, and to correlate the results with digital subtraction angiography (DSA) and intraoperative findings. From May 2005 to May 2007 a total of 38 consecutive patients admitted for SAH, suspicious for ruptured anterior circulation cerebral aneurysm, underwent 3D-CTA. Inclusion criteria were a SAH confirmed by a non-contrast head computed tomographic scan or by lumbar puncture. Exclusion criteria were: previous DSA, severe contrast medium allergy and severe renal failure. All patients underwent early surgical clipping of the aneurysm. The mean time between SAH onset and surgery was 43.6 h (range, 14-74 h). The 3D-CTA was performed in all cases; and in 13 out of 38 patients (34%) represented the only preoperative exam. In all patients that underwent surgery with the sole 3D-CTA, the images collected allowed a good visualisation of the morphology of aneurysms and of the anatomical relationship with the vascular structures. 3D-CTA allows accurate diagnosis with an excellent visualisation of ruptured aneurysms of the anterior circulation. Our results suggest that, in selected cases, ruptured anterior circulation aneurysms could be successfully treated on the basis of 3D-CTA alone. [ABSTRACT FROM AUTHOR]

Recent studies have shown an anti-tumour activity of cannabinoid receptors CB1 and CB2 in gliomas. This effect was mediated by neurotrophins in breast and prostate carcinoma, while in gliomas this relationship has not yet been considered. The aim of this study was to investigate the expression of cannabinoid receptors CB1 and CB2, neurotrophin NGF and NT-3 and their receptors TrkA and TrkC in glioma and endothelial cells. The analysis was performed in 14 gliomas and 2 non-tumour brain specimens by immunohistochemistry and real-time quantitative-polymerase chain reaction (RTQ-PCR). Gliomas showed a weak immunoreactivity for CB1 and CB2 in tumour and in endothelial cells, and for NGF/TrkA mainly in tumour cells, while a moderate/diffuse immunoreactivity was found for NT-3/TrkC. CB2 was expressed on 3 out of 6 low-grade gliomas and in all high-grade gliomas. Non-tumour brain tissues were weakly positive in astrocytes and endothelium for CB1, CB2, NT-3 and TrkC and negative for NGF and TrkA. By RTQ-PCR, gliomas showed low mRNA levels of NGF/TrkA and moderate levels of CB1, NT-3 and TrkC. CB2 mRNA expression was low or absent. A potential role of cannabinoids, particularly of CB2 agonists devoid of psychotropic side effects, in glioma therapy could have a basis in glioblastomas, because they were all positive, though weakly, to CB2. The presence of neurotrophins and their receptors, mainly NT-3 and TrkC, suggests a possible role of these pathways in glioma growth/invasion, but further investigations are required to verify this hypothesis and a potential relationship between cannabinoids and neurotrophins. [ABSTRACT FROM AUTHOR]

To study the relationship between initiating therapy with an antipsychotic medication and a subsequent new diagnosis of a drug-induced movement disorder other than parkinsonism in older adults with dementia. Retrospective, population-based cohort study. Ontario, Canada. Ontario residents aged 66 and older with a diagnosis of dementia newly started on treatment with typical or atypical antipsychotic therapy. Estimated relative risk of developing a drug-induced movement other than parkinsonism in the 1-year follow-up period after starting therapy with an antipsychotic medication. From April 1, 1997, to March 31, 2001, 21, 835 older adults with dementia who were newly started on antipsychotic medications were identified. Nine thousand seven hundred ninety subjects were started on atypical antipsychotics and 12,045 subjects started on typical antipsychotics. Demographic characteristics were similar between the groups. There were 5.24 cases of tardive dyskinesia (TD) or other drug-induced movement disorder per 100 person-years on therapy with a typical antipsychotic and 5.19 cases per 100 person-years on therapy with an atypical antipsychotic. The risk of developing drug-induced movement disorder while being treated with an atypical agent was not statistically different from that with a typical antipsychotic (relative risk=0.99, 95% confidence interval=0.86–1.15; P<.93). Older adults with dementia who are treated with typical or atypical antipsychotic therapy are at risk for developing TD and other drug-induced movement disorders. [ABSTRACT FROM AUTHOR]

Epilepsy surgery is recommended in selected patients with Tuberous Sclerosis Complex (TSC). However, reports on predictive factors of seizure outcome are variable. Here we report on seizure and cognitive outcome of 35 TSC patients who received surgery for refractory epilepsy in 7 Italian centers over a period of 22 years (1997–2019). The rate of seizure-free individuals at last follow-up (mean 7.5 years, range 1–21 years) was 51%. Patients with longer follow-up (≥10 years) had a lower rate of Engel I outcome (11.1%) than those who received surgery in the last 10 years (65.4%, p = 0.003). Factors associated with Engel II, III, IV outcome in our cohort included: high number of cortical tubers (≥5); presence of subependymal nodules (SENs); seizure onset before age 1 year; and multifocal interictal epileptic discharges (IEDs) on electroencephalogram (EEG). A subset of patients evaluated with Vineland Adaptive Behaviour Scales (VABS) showed developmental gains, in line with their developmental trajectories, but no improvement in standard scores after surgery was noted. Our study demonstrates that the rates of successful seizure outcome of epilepsy surgery in TSC have improved in the last 10 years. More than half of the patients achieved seizure freedom, and a high proportion of affected individuals experienced a reduction in seizure burden and in antiseizure medications. A comprehensive assessment after surgery should be performed in TSC patients to evaluate the overall neurodevelopmental outcome, as measures that are based only on seizure control do not adequately identify the benefits of surgery on global functioning in these patients. • Epilepsy surgery is a treatment option for patients with refractory seizures in TSC. • More than half of the patients who received surgery achieved seizure freedom. • A worthwhile improvement is observed in up to 77% of surgically treated patients. • Multiple tubers, early seizure onset and multifocal IEDs predict seizure recurrence. • Presurgical assessment is crucial to selecting best candidates for surgery. [ABSTRACT FROM AUTHOR]