Surgical outcomes in children with drug-resistant epilepsy and hippocampal sclerosis.

BACKGROUND: Hippocampal sclerosis (HS) is a common surgical substrate in adult epilepsy surgery cohorts but variably reported in various pediatric cohorts. OBJECTIVE: We aimed to study the epilepsy phenotype, radiological and pathological variability, seizure and neurocognitive outcomes in children with drug-resistant epilepsy and hippocampal sclerosis (HS) with or without additional subtle signal changes in anterior temporal lobe who underwent surgery. METHODS: This retrospective study enrolled children with drug-resistant focal epilepsy and hippocampal sclerosis with or without additional subtle T2-Fluid Attenuated Inversion Recovery (FLAR)/Proton Density (PD) signal changes in anterior temporal lobe who underwent anterior temporal lobectomy with amygdalohippocampectomy. Their clinical, EEG, neuropsychological, radiological and pathological data were reviewed and summarized. RESULTS: Thirty-six eligible patients were identified. The mean age at seizure onset was 3.7 years; 25% had daily seizures at time of surgery. Isolated HS was noted in 22 (61.1%) cases and additional subtle signal changes in ipsilateral temporal lobe in 14 (38.9%) cases. Compared to the normative population, the group mean performance in intellectual functioning and most auditory and visual memory tasks were significantly lower than the normative sample. The mean age at surgery was 12.3 years; 22 patients (61.1%) had left hemispheric surgeries. ILAE class 1 outcomes was seen in 28 (77.8%) patients after a mean follow up duration of 2.3 years. Hippocampal sclerosis was noted pathologically in 32 (88.9%) cases; type 2 (54.5%) was predominant subtype where further classification was possible. Additional pathological abnormalities were seen in 11 cases (30.6%); these had had similar rates of seizure freedom as compared to children with isolated hippocampal sclerosis/gliosis (63.6% vs 84%, p=0.21). Significant reliable changes were observed across auditory and visual memory tasks at an individual level post surgery. CONCLUSIONS: Favourable seizure outcomes were seen in most children with isolated radiological hippocampal sclerosis. Patients with additional pathological abnormalities had similar rates of seizure freedom as compared to children with isolated hippocampal sclerosis/gliosis.

Which parameters influence cognitive, psychiatric and long-term seizure outcome in mesial temporal lobe epilepsy after selective amygdalohippocampectomy?

BACKGROUND: We aimed to analyze potentially prognostic factors which could have influence on postoperative seizure, neuropsychological and psychiatric outcome in a cohort of patients with mesial temporal lobe epilepsy (MTLE) due to hippocampal sclerosis (HS) after selective amygdalohippocampectomy (SAHE) via transsylvian approach. METHODS: Clinical variables of 171 patients with drug-resistant MTLE with HS (88 females) who underwent SAHE between 1994 and 2019 were evaluated using univariable and multivariable logistic regression models, to investigate which of the explanatory parameters can best predict the outcome. RESULTS: At the last available follow-up visit 12.3 ± 6.3 years after surgery 114 patients (67.9%) were seizure-free. Left hemispheric MTLE was associated with worse postoperative seizure outcome at first year after surgery (OR = 0.54, p = 0.01), female sex-with seizure recurrence at years 2 (OR = 0.52, p = 0.01) and 5 (OR = 0.53, p = 0.025) and higher number of preoperative antiseizure medication trials-with seizure recurrence at year 2 (OR = 0.77, p = 0.0064), whereas patients without history of traumatic brain injury had better postoperative seizure outcome at first year (OR = 2.08, p = 0.0091). All predictors lost their predictive value in long-term course. HS types had no prognostic influence on outcome. Patients operated on right side performed better in verbal memory compared to left (VLMT 1-5 p < 0.001, VLMT 7 p = 0.001). Depression occurred less frequently in seizure-free patients compared to non-seizure-free patients (BDI-II Z = - 2.341, p = 0.019). CONCLUSIONS: SAHE gives an improved chance of achieving good postoperative seizure, psychiatric and neuropsychological outcome in patients with in MTLE due to HS. Predictors of short-term outcome don't predict long-term outcome.

Epilepsy surgery for dominant-side mesial temporal lobe epilepsy without hippocampal sclerosis.

Although anterior temporal lobectomy (ATL) is an established surgery for medically intractable mesial temporal lobe epilepsy (MTLE), it can harm memory function, especially in dominant-side MTLE patients without hippocampal sclerosis (HS). To avoid this complication, multiple hippocampal transection (MHT) was developed, but its efficacy has not been fully elucidated. We report the detailed treatment results of MHT compared with that of ATL. We retrospectively analysed the records of 30 patients who underwent surgery for dominant-side MTLE. ATL was completed for 23 patients with HS, and MHT was completed for 7 patients without HS. The seizure control status, number of anti-seizure medicines, neurocognitive function, and psychiatric disorders of each patient were reviewed. The mean follow-up period was 70 months. Seizure control of Engel class I was achieved in 16 patients (70%) in the ALT group versus 5 patients (71%) in the MHT group. The mean number of anti-seizure medicines administered in the ATL group changed significantly from 2.4 to 1.9 (p = 0.01), while that in the MHT group was unchanged (from 2.1 to 2.0, p = 0.77). Eleven patients (48%) in the ATL group developed psychiatric disorders during the postoperative follow-up period, whereas no psychological complications were observed in the MHT group. Neither group showed neurocognitive decline after the surgery in any of the WAIS-III or WMS-R subtests. In conclusion, MHT may achieve reasonable postoperative seizure reduction, preserve neurocognitive function, and reduce postoperative psychiatric complications. Therefore, it can be considered as a therapeutic option for dominant-side MTLE without HS.

Surgical Outcome of Young and Adult Patients with Temporal Lobe Epilepsy Related to Hippocampal Sclerosis in Modern Era.

AIM: To evaluate the preoperative, operative, and postoperative outcomes of young and adult patients who underwent surgery for temporal lobe epilepsy related to hippocampal sclerosis (TLE-HS). MATERIAL AND METHODS: This retrospective study assessed prospectively registered data collected from 2010 to 2020. Clinical, electrophysiological, pathological, and postoperative outcomes were evaluated and compared. Post-surgical seizure outcome was classified into continuous seizure freeness without aura and relapse. RESULTS: In total, 16 young and 48 adult patients with TLE-HS were included in the analysis. The clinical, electrophysiological, pathological, and postoperative outcomes were similar between the young and adult groups. However, the seizure outcome did not significantly differ between the two groups (p=0.38). A significant proportion of patients in both groups were satisfied with the surgical outcomes. CONCLUSION: Surgery is extremely effective against TLE-HS in young patients, as in adults. Furthermore, the clinical, radiological, and pathological outcomes are similar between young and adult patients with TLE-HS.

Sleep macrostructure and cyclic alternating pattern in patients who underwent surgery for hippocampal sclerosis: A prospective controlled polysomnographic study.

INTRODUCTION: Temporal lobe epilepsy due to hippocampal sclerosis (TLE-HS) is one of the most common drug-resistant epilepsy. Surgery is currently accepted as an effective and safe therapeutic approach compared to antiseizure medications (ASMs). The study aims to evaluate the effect of surgical treatment of TLE-HS on sleep profile and architecture by subjective and objective evaluation of sleep in basal condition after one month and one year. METHODS: Thirteen patients with TLE-HS were recruited to undergo overnight polysomnography and a subjective evaluation of nocturnal sleep utilizing the Pittsburgh Sleep Quality Index (PSQI) and daytime somnolence through the Epworth Sleepiness Scale (ESS) in basal condition (T0), one month (T1) and one year after surgery (T2), respectively. Thirteen healthy controls (HC) matched for age, sex and BMI were recruited. Scoring and analysis of sleep macrostructure and cyclic alternating pattern (CAP) parameters were performed. RESULTS: The comparison between patients in basal condition (T0) and HC showed a significant lower sleep efficiency (p = 0.003) and REM percentage (p < 0.001). Regarding CAP, patients at T0 showed higher total CAP rate (p < 0.001), CAP rate in N2 (p < 0.001), higher A3 (%) (p = 0.001), higher mean duration of A1 (p = 0.002), A3 index (p < 0.001), cycle in sequences (p < 0.001), lower B duration (p < 0.001), cycle mean duration (p < 0.001) than HC. Surgery did not induce significant changes in nocturnal macrostructural polysomnographic variables in T1 and T2. Lower CAP rate (T1 vs T0 and T2 vs T0 p < 0.001), CAP rate in N3 (T1 vs T0 and T2 vs T0 p < 0.001), A3 (%) (T1 vs T0 and T2 vs T0 p < 0.001); lower phase A2 index (T1 vs T0 p < 0.001) and A3 index (T1 vs T0 p < 0.001), lower phase A1 index (T2 vs T0 p < 0.001) and cycle in sequences (T2 vs T0 p = 0.002) higher B mean duration (T2 vs T0 p = 0.002). No significant differences were found between T1 and T2 in CAP parameters. CONCLUSION: We found a significant NREM sleep instability in patients with TLE-HS compared with HC. In addition, anterior temporal lobectomy (ATL) induced a significant improvement in sleep continuity as evaluated by cyclic alternating pattern already one month later and this effect persisted after one year. ALT seems to restore a more resilient sleeping brain.

Metabolic network is related to surgical outcome in temporal lobe epilepsy with hippocampal sclerosis: A brain FDG-PET study.

BACKGROUND AND PURPOSE: The aim of this study was to investigate differences in metabolic networks based on preoperative fluorodeoxyglucose (FDG)-positron emission tomography (PET) in temporal lobe epilepsy (TLE) with hippocampal sclerosis (HS) between patients with complete seizure-free (SF) and those with noncomplete seizure-free (non-SF) after anterior temporal lobectomy. METHODS: This study was retrospectively performed at a tertiary hospital. We recruited pathologically confirmed 75 TLE patients with HS who underwent preoperative FDG-PET. All patients underwent a standard anterior temporal lobectomy. The surgical outcome was evaluated at least 12 months after surgery, and we divided the subjects into patients with SF (International League Against Epilepsy [ILAE] class I) and those with non-SF (ILAE class II-VI). We evaluated the metabolic network using graph theoretical analysis based on FDG-PET. We investigated the differences in network measures between the two groups. RESULTS: Of the 75 TLE patients with HS, 32 patients (42.6%) had SF, whereas 43 patients (57.3%) had non-SF. There were significant differences in global metabolic networks according to surgical outcomes. The patients with SF had a lower assortative coefficient than those with non-SF (-0.020 vs. -0.009, p = .044). We also found widespread regional differences in local metabolic networks according to surgical outcomes. CONCLUSION: Our study demonstrates significant differences in preoperative metabolic networks based on FDG-PET in TLE patients with HS according to surgical outcomes. This work introduces a metabolic network based on FDG-PET and can be used as a potential tool for predicting surgical outcome in TLE patients with HS.

[Surgical treatment of drug-resistant epilepsy following hippocampal sclerosis]. / Khirurgicheskoe lechenie farmakorezistentnoi epilepsii pri skleroze gippokampa.

Surgery is an effective approach for drug-resistant temporal lobe epilepsy following hippocampal sclerosis. There is still no clear and unanimous opinion about advantages and disadvantages of certain surgical technique. MATERIAL AND METHODS: There were 103 surgical interventions in 101 patients. Females prevailed (1.45:1). Age of patients ranged from 16 to 56 years (median 28). Anteromedial temporal lobectomy and selective amygdaloghippocampectomy were performed in 49 (47.6%) and 54 (52.4%) patients, respectively. In the latter group, 30 patients were operated via a 14-mm burr hole-subtemporal approach. Postoperative outcomes were assessed using the Engel grading system. The follow-up period ranged from 2 to 8 years (median 4 years). RESULTS: By the 2nd year, Engel class I was observed in 74 (72%) patients, Engel II, III and IV - in 20 (19.4%), 6 (5.8%) and 3 (2.9%) patients, respectively. Engel class I was achieved after anteromedial temporal lobectomy in 68% of cases, selective amygdaloghippocampectomy via standard approaches in 75% of cases, amygdaloghippocampectomy via subtemporal burr hole approach - in 80% of cases. Neurocognitive impairments after anteromedial lobectomy and selective amygdaloghippocampectomy were similar. At the same time, mental disorders de novo prevailed in the group of anteromedial lobectomy (p<0.05). There were no severe visual field disorders after subtemporal burr-hole access. In other cases, these disorders occurred in 36.2% of patients (p<0.05). There were 8 (7.8%) postoperative complications: 5 (10.2%) - after anterior temporal lobectomy, 3 (5.5%) - after selective surgeries via standard approaches. There were no complications after burr-hole surgery. CONCLUSION: Selective amygdaloghippocampectomy is not inferior to anteromedial lobectomy. Moreover, this procedure is associated with a lower risk of complications and adverse events.

Evaluation of patients with late-onset and medically refractory temporal lobe epilepsy with mesial temporal sclerosis.

OBJECTIVES: Our study aims to compare demographics, clinical features and postsurgical outcomes between early and late-onset patients with medically refractory temporal lobe epilepsy (TLE) related to mesial temporal sclerosis (MTS). PATIENTS AND METHODS: Seventy-one patients admitting to the Epilepsy Clinic of Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine between 1995-2015, who were diagnosed with refractory TLE related to MTS, were included in our study. All of these patients were resistant to medical treatment, and thus candidates for, or underwent surgery, and had no pathology other than MTS in their cranial magnetic resonance imaging (MRI). Based on previous studies, those patients were divided into two categories as "early-onset" and "late-onset", according to the age-onset of afebrile recurrent seizures, where the cutoff was determined as 20 years. Demographics, clinical features, and postsurgical outcomes were compared between both groups. RESULTS: Fifty-three patients included in our study had early-onset MTS-TLE, and 18 patients had late-onset MTS-TLE. Demographics, clinical features, characteristics of electroencephalography (EEG), MRI, PET MRI/CT, neuropsychometric test (NPT) and postsurgical outcomes were similar in both groups. CONCLUSION: In both the early-onset and late-onset groups, the presence of similar demographics, clinical features, and postoperative outcomes have suggested that the course of the disease and the success of surgical treatment were not associated with the age-onset of seizures in TLE related to MTS.

Cervical Hyperextension Treated by Posterior Spinal Correction and Fusion in A Patient with Ullrich Congenital Muscular Dystrophy: A Case Report.

CASE: An 18-year-old man with Ullrich congenital muscular dystrophy (UCMD) noted difficulty of looking forward and discomfort swallowing and breathing because of his hyperextended neck. We treated his cervical deformity with posterior spinal correction and fusion alone. He underwent a tracheotomy because of lung function deterioration 2 years after cervical surgery. The tracheotomy was performed safely because the anterior cervical spine anatomy was normalized and soft tissues around trachea were preserved by the posterior cervical correction. CONCLUSION: Cervical hyperextension can be a problem in patients with UCMD. Posterior spinal correction and fusion may be a preferable solution.

Favorable outcome of extended curettage for the treatment of unifocal chronic sclerosing osteomyelitis of clavicle: a case series.

BACKGROUND: Chronic sclerosing osteomyelitis (CSO) of the clavicle, especially its unifocal subset, is scarcely reported, and little is known about its characteristic features and treatment. We aim to describe the characteristic features and outcome of treatment in a series of patients with unifocal CSO of the clavicle. MATERIALS AND METHODS: In a retrospective study, we identified 6 patients with a diagnosis of unifocal CSO of the clavicle. All patients underwent a core needle biopsy, and histologic examination confirmed the diagnosis. Laboratory investigations included the white blood cell count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, and bacteriologic culture. We used plain radiographs, computed tomography scanning, and magnetic resonance imaging for the radiologic evaluation. RESULTS: The median age of the patients was 16.5 years (range, 10-29 years). The ESR and CRP level were elevated in 5 cases. The bacterial culture results were negative in all cases. Sclerosis was the main radiologic symptom. Other radiologic features such as bone expansion, cystic change, periosteal reaction, cortex destruction, and soft-tissue edema could accompany sclerosis. Anti-inflammatory medications temporarily and slightly reduced the symptoms. Four patients underwent extended curettage, in whom the clinical, radiologic, and laboratory symptoms considerably subsided. In the 2 patients who did not agree to undergo surgical intervention, the clinical and radiologic symptoms fluctuated at the follow-up visits. CONCLUSION: Unifocal CSO of the clavicle is associated with negative bacterial culture results, but the ESR and CRP level are frequently elevated. The symptoms fluctuate if not adequately treated. Extended curettage could be regarded as the treatment of choice.

Minimally Invasive Technique (Nummular Craniotomy) for Mesial Temporal Lobe Epilepsy: A Comparison of 2 Approaches.

OBJECTIVE: To describe our series of a minimally invasive technique using a small scalp incision and keyhole craniotomy for the removal of mesial temporal lobe structures through a transcortical approach in patients with medically intractable mesial temporal lobe epilepsy (MTLE). Studies that directly compare the clinical outcomes between minimally invasive and conventional techniques are scarce, and this information is lacking in the literature. METHODS: The study enrolled 73 consecutive patients with refractory MTLE and unilateral hippocampal sclerosis; 30 patients were operated on with standard frontotemporal craniotomy between 2010 and 2013 and 43 patients were operated with a minimally invasive craniotomy (nummular craniotomy) between 2014 and 2016. The preoperative evaluation included clinical history, physical examination, video-electroencephalography, neuropsychologic assessment, and magnetic resonance imaging including thin-section coronal sequences. RESULTS: There were no deaths in either group. Postoperative complications in the standard frontotemporal craniotomy group included temporal muscle atrophy (n = 4; 13.3%), cerebrospinal fluid leakage (n = 1; 3.3%), and wound infection (n = 1; 3.3%). No complications were observed in the keyhole craniotomy group. There was no between-group difference in postoperative seizure control. The mean Engel class I seizure-free outcome was 90.4% in the standard frontotemporal craniotomy group and 90.7% in the nummular craniotomy group (P > 0.05). Lengths of hospitalization (2.81 vs. 4.37 days, P < 0.001) and operative time (85.79 vs. 142.73 minutes, P < 0.001) were lower in the keyhole than in the standard frontotemporal craniotomy group, respectively. CONCLUSIONS: The nummular technique was associated with faster recovery, early hospital discharge, and fewer complications than the standard technique. No differences were observed in postoperative seizure control. Keyhole craniotomy is a safe, easy, and effective treatment option for medically intractable MTLE.

Management of radial scars/complex sclerosing lesions of the breast diagnosed on vacuum-assisted large-core biopsy: is surgery always necessary?

AIMS: The diagnosis of radial scars/complex sclerosing lesions (RSs/CSLs) onpercutaneous biopsy carries a risk of histological underestimation. Consequently, surgical excision is often performed in order to exclude a possible associated malignancy. The aim of this study was to assess the rate of 'upgrade to carcinoma' upon subsequent surgical excision of RS/CSL cases diagnosed on vacuum-assisted large-core biopsy (VALCB). We also analysed the risk factors for upgrade in order to determine a subset of patients who could avoid surgery and benefit from conservative management with clinical and imaging follow-up. METHODS AND RESULTS: This was a retrospective observational single-centre study on 174 consecutive RS/CSL cases diagnosed on VALCB from May 2008 to October 2015. Univariate analysis was performed to identify clinical, radiological and histological risk factors for upgrade. Surgical excision was performed following VALCB diagnosis of 88 RS/CSL cases with or without associated atypia. The overall rate of surgical upgrade to carcinoma was 9.1% (8/88). None of the benign biopsies without atypia was surgically upgraded. Additional to atypia, risk factors for upgrade were non-incidental finding of the RS/CSL, the mammographic appearance, and the number of fragments obtained during the biopsy procedure (P < 0.05). CONCLUSION: We demonstrate that VALCB revealing an RS/CSL is reliable for excluding malignancy when there is no associated atypia and when radiological and histological findings are concordant. In such cases, surgery can be avoided in favour of clinical and imaging follow-up. When an RS/CSL is associated with atypia, the decision to perform surgical excision depends on other associated risk factors.

The degree of sclerosis is associated with prognosis in well-differentiated liposarcoma of the retroperitoneum.

BACKGROUND AND OBJECTIVES: Well-differentiated liposarcomas (WDL) are often partly composed of sclerotic tissue, however, the amount varies widely between tumors, and its prognostic significance is unknown. We hypothesized that tumors with more sclerosis would behave more aggressively. METHODS: Primary retroperitoneal WDL from 29 patients resected at our institution with follow-up were histologically evaluated by soft tissue pathologists blinded to outcome. Tumors with ≥ 10% sclerosis were designated "sclerotic" while tumors with < 10% sclerosis were designated as "minimally sclerotic". Cellular and dedifferentiated tumors were excluded. Clinical parameters and radiologic assessments on computed tomography (CT) were recorded. RESULTS: Histological evaluation identified 13 minimally sclerotic WDL and 16 sclerotic WDL. Median follow-up was 9 years (range, 3-20). Median recurrence-free survival (RFS) and median overall survival (OS) were 6.16 and 13.9 years, respectively. Compared with patients with sclerotic WDL, those with minimally sclerotic WDL had superior RFS (HR = 0.17 [95% CI, 0.06-0.53], P = .002) and OS (log-rank test, P = .002). Sclerotic WDL exhibited higher Houndsfield Units than minimally sclerotic WDL (26 vs 1, P = .040). CONCLUSIONS: Minimally sclerotic WDL were associated with more favorable outcome compared with sclerotic tumors. Assessment of sclerosis in WDL is likely a useful prognostic marker.

Ressecção cirúrgica de calcinose sintomática em paciente com esclerose sistêmica / Surgical resection of symptomatic calcinosis in a patient with systemic sclerosis

Introdução: A esclerose sistêmica é uma doença rara, autoimune, com evolução progressiva, que afeta os tecidos conectivos e órgãos internos por inflamação, podendo causar calcinose de subcutâneo. Podem evoluir para quadros dolorosos e incapacitantes, podendo tornar-se infectados, principalmente quando ulceram pela pele. Objetivo: Apresentar caso de calcinose em região inguinal e sua evolução cirúrgica. Relato de Caso: Paciente feminina portadora de calcinoses em região inguinal bilateral, apresentando algia moderada/grave com falha de tratamento clínico. Realizada ressecção cirúrgica das calcinoses, que formavam cordões de fibrose com aderência na fáscia do músculo oblíquo externo. Realizado fechamento primário com nylon 2.0 pontos simples subdérmicos e ponto intradérmico continuo nylon 3.0 para fechamento estético e menor reação inflamatória. Boa evolução pós- operatório. Conclusão: O melhor tratamento da calcinoses ainda não é claro. O tratamento das complicações se torna essencial para reduzir a morbidade e aumentar a qualidade de vida do paciente.

Introduction: Systemic sclerosis is a rare, autoimmune, progressive disease that affects connective tissues and internal organs by inflammation, which can cause calcinosis cutis. It can progress to painful and disabling conditions, and can become infected, especially when skin ulceration is present. Objective: To present a case of calcinosis in the inguinal region and its surgical recovery. Case Report: A female patient with calcinosis in the bilateral inguinal region presenting with moderate/severe pain had a failed clinical treatment. We performed surgical resection of the calcinosis cutis, which had formed clusters of fibrosis with adhesion to the fascia of the external oblique muscle. We used simple nylon 2.0 sutures along the subdermal plane to perform primary closure and continuous nylon 3.0 sutures along the intradermal plane for aesthetic closure and minimal inflammatory reaction. Her postoperative recovery was positive. Conclusion: The best treatment for calcinosis cutis is still unclear. Treating complications becomes essential for reducing patients' morbidity and increasing their quality of life.

Pulmonary Arterial Bypass Surgery for Fibrosing Mediastinitis Causing Severe Pulmonary Hypertension.

This case report describes a patient with mediastinal fibrosis. The mass lesion was expanding from the mediastinum to the cervical region and surrounding the major vascular structures. The patient had symptomatic dyspnea at rest and pulmonary hypertension secondary to stenosis of the bilateral main pulmonary arteries. To decrease pulmonary artery pressure and improve blood flow to the lungs, grafting from the pulmonary trunk to the bilateral interlobar pulmonary arteries was performed using cardiopulmonary bypass.

FCR Interposition Arthroplasty for Concomitant STT and CMC Arthritis.

Arthritis of scaphotrapeziotrapezoid joint is common and can be seen in association with first carpal metacarpal (CMC) arthritis. Untreated scaphotrapeziotrapezoid arthritis may be a cause of residual pain after CMC arthroplasty. There are various treatment options described in the literature ranging from arthrodesis, resection arthroplasty, interpositional arthroplasty, and implant arthroplasty. We describe a novel technique of flexor carpi radialis (FCR) tendon interposition to tackle this condition. Our common treatment for CMC arthritis is trapezium resection with ligament reconstruction and tendon interposition with half of the FCR. With this procedure, through the same exposure, the proximal part of the trapezoid is resected. An anchor is placed into the trapezoid and the sutures are passed through a remnant of the FCR. Thus the FCR is pulled in between the scaphoid and the trapezoid and secured in place. We performed this procedure in 13 hands with average follow-up of 36 months. All patients were satisfied with the procedure and were pain free at their last follow-up. The advantage of this procedure is the use of the FCR, which is already available in the field and requires very little additional procedure.

Visual field defects after radiosurgery versus temporal lobectomy for mesial temporal lobe epilepsy: Findings of the ROSE trial.

PURPOSE: Stereotactic radiosurgery (SRS) may be an alternative to anterior temporal lobectomy (ATL) for mesial temporal lobe epilepsy (MTLE). Visual field defects (VFD) occur in 9-100% of patients following open surgery for MTLE. Postoperative VFD after minimally invasive versus open surgery may differ. METHODS: This prospective trial randomized patients with unilateral hippocampal sclerosis and concordant video-EEG findings to SRS versus ATL. Humphries perimetry was obtained at 24 m after surgery. VFD ratios (VFDR = proportion of missing homonymous hemifield with 0 = no VFD, 0.5 = complete superior quadrantanopsia) quantified VFD. Regressions of VFDR were evaluated against treatment arm and covariates. MRI evaluated effects of volume changes on VFDR. The relationships of VFDR with seizure remission and driving status 3 years after surgery were evaluated. RESULTS: No patients reported visual changes or had abnormal bedside examinations, but 49 of 54 (91%) of patients experienced VFD on formal perimetry. Neither incidence nor severity of VFDR differed significantly by treatment arm. VFDR severity was not associated with seizure remission or driving status. CONCLUSION: The nature of VFD was consistent with lesions of the optic radiations. Effective surgery (defined by seizure remission) of the mesial temporal lobe results in about a 90% incidence of typical VFD regardless of method.

Altered transforming growth factor beta/SMAD3 signalling in patients with hippocampal sclerosis.

Transforming growth factor beta (TGFß) signalling cascade has been implicated in enhancing neuronal excitability and excitatory synaptogenesis following blood brain barrier (BBB) damage and inflammation. We aimed to study if TGFß signalling expression is altered in patients with Hippocampal Sclerosis (HS). We probed into the protein expression level of the ligand transforming growth factor beta 1 (TGFß1), transforming growth factor beta receptor II (TGFßRII) and downstream signalling molecule SMAD3 and phosphorylated SMAD3 (pSMAD3) on surgically resected hippocampal samples of thirty-four patients with HS through immuno-blotting. The increase in protein expression level of the ligand TGFß1 was 285 ± 1.15% higher and its receptor TGFßRII was 170 ± 0.98% higher in hippocampus of patients with HS in comparison to the autopsy hippocampal control samples. The expression of the downstream signalling molecules, SMAD3 is 157 ± 0.13% and 106 ± 0.17% higher in patients with HS as compared to both types of non-seizure controls. The expression of active form of SMAD3, pSMAD3 (2.6010 ± 1.2735) was significantly upregulated in hippocampus of patients with HS compared to autopsy hippocampal controls (0.7899 ± 0.3688). While the expression of pSMAD3 (1.527 ± 0.9425) was significantly upregulated in hippocampus of patients with HS with another type of non-seizure control viz. tumour periphery tissue (0.5791 ± 0.2679), hence strongly supporting the altered expression of the pathway. This study provides the first evidence of alteration of TGFß pathway in patients with HS which could be a potential therapeutic target.

New clinicopathological associations and histoprognostic markers in ILAE types of hippocampal sclerosis.

Mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS) is a heterogeneous syndrome. Surgery results in seizure freedom for most pharmacoresistant patients, but the epileptic and cognitive prognosis remains variable. The 2013 International League Against Epilepsy (ILAE) histopathological classification of hippocampal sclerosis (HS) has fostered research to understand MTLE-HS heterogeneity. We investigated the associations between histopathological features (ILAE types, hypertrophic CA4 neurons, granule cell layer alterations, CD34 immunopositive cells) and clinical features (presurgical history, postsurgical outcome) in a monocentric series of 247 MTLE-HS patients treated by surgery. NeuN, GFAP and CD34 immunostainings and a double independent pathological examination were performed. 186 samples were type 1, 47 type 2, 7 type 3 and 7 samples were gliosis only but no neuronal loss (noHS). In the type 1, hypertrophic CA4 neurons were associated with a worse postsurgical outcome and granule cell layer duplication was associated with generalized seizures and episodes of status epilepticus. In the type 2, granule cell layer duplication was associated with generalized seizures. CD34+ stellate cells were more frequent in the type 2, type 3 and in noHS. These cells had a Nestin and SOX2 positive, immature neural immunophenotype. Patients with nodules of CD34+ cells had more frequent dysmnesic auras. CD34+ stellate cells in scarce pattern were associated with higher ratio of normal MRI and of stereo-electroencephalographic studies. CD34+ cells were associated with a trend for a better postsurgical outcome. Among CD34+ cases, we proposed a new entity of BRAF V600E positive HS and we described three hippocampal multinodular and vacuolating neuronal tumors. To conclude, our data identified new clinicopathological associations with ILAE types. They showed the prognostic value of CA4 hypertrophic neurons. They highlighted CD34+ stellate cells and BRAF V600E as biomarkers to further decipher MTLE-HS heterogeneity.