Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology

The International League Against Epilepsy (ILAE) presents a revised operational classification of seizure types. The purpose of such a revision is to recognize that some seizure types can have either a focal or generalized onset, to allow classification when the onset is unobserved, to include some missing seizure types, and to adopt more transparent names. Because current knowledge is insufficient to form a scientifically based classification, the 2017 Classification is operational (practical) and based on the 1981 Classification, extended in 2010. Changes include the following: (1) “partial” becomes “focal”; (2) awareness is used as a classifier of focal seizures; (3) the terms dyscognitive, simple partial, complex partial, psychic, and secondarily generalized are eliminated; (4) new focal seizure types include automatisms, behavior arrest, hyperkinetic, autonomic, cognitive, and emotional; (5) atonic, clonic, epileptic spasms, myoclonic, and tonic seizures can be of either focal or generalized onset; (6) focal to bilateral tonic–clonic seizure replaces secondarily generalized seizure; (7) new generalized seizure types are absence with eyelid myoclonia, myoclonic absence, myoclonic–atonic, myoclonic–tonic–clonic; and (8) seizures of unknown onset may have features that can still be classified. The new classification does not represent a fundamental change, but allows greater flexibility and transparency in naming seizure types.     

Comorbidities and predictors of health-related quality of life in Dravet syndrome

Purpose: Health‐related quality of life (HRQOL) has emerged as a widely accepted measure to evaluate how chronic disease impacts on an individual’s physical, social, and mental well‐being. There is a paucity of data focusing on HRQOL in specific epilepsy syndromes and their associated needs. In this study our aim was to describe the comorbidities and disease‐related predictors for HRQOL in Dravet syndrome, an epileptic encephalopathy, with defined genetic etiology. We anticipate that this will help us to better recognize and understand the needs of children and families and aid treatment planning in this severe epilepsy syndrome. Methods: One hundred sixty‐three individuals with Dravet syndrome and their families participated in the study. Detailed clinical and demographic information was available for each case. HRQOL was evaluated with two epilepsy‐specific instruments, the Impact of Pediatric Epilepsy Scale (IPES) and the Epilepsy & Learning Disabilities Quality of Life Questionnaire (ELDQOL); a generic HRQOL instrument; the Pediatric Quality of Life Inventory (PedsQL); and a behavioral screening tool, the Strength and Difficulties Questionnaire (SDQ). Key Findings: HRQOL was significantly lower for children with Dravet syndrome compared to normative data (p < 0.001). A cross‐sectional evaluation of measures across different age groups revealed that PedsQL generic core and cognitive function scales decreased in older age categories, indicating worse HRQOL (p < 0.001). Assessment of epilepsy severity demonstrated that symptoms were rated very severe in 10 (6%) of 162 cases, somewhat severe in 78 (48%) of 162, moderate in 51 (32%) of 162, and mild in 23 (14%) of 162 cases. The epilepsy severity correlated significantly with the IPES total impact score (r = 0.466, p < 0.001, n = 162). The IPES total impact scores in the Dravet group (n = 162) were significantly higher than scores measured in the original validation sample of epileptic children with and without learning difficulties (± SD) (21.0 ± 8.7 vs. 11.6 ± 5.4, t = 8.95, p < 0.001, n = 46). On the SDQ, 35% of children scored in the abnormal range for “conduct problems,” 66% for “hyperactivity/ inattention,” and 76% for “peer relationships.” Regression analysis revealed that young age at seizure onset (p = 0.019), presence of myoclonic seizures (p = 0.029), motor disorder (p = 0.048), learning difficulties (p = 0.002), epilepsy severity (p < 0.001), and behavioral difficulties (p < 0.001) each independently predicted poorer HRQOL. Behavioral problems such as hyperactivity/inattention were the strongest predictors of poorer HRQOL. Significance: This is the first comprehensive study of HRQOL in an etiologically well‐defined epilepsy syndrome. HRQOL in Dravet syndrome depends on a series of independent factors including seizure control, behavior, cognitive, and motor problems. Identification of specific comorbidities in Dravet syndrome will facilitate a distinct and multidisciplinary approach to management, addressing seizure control, behavior problems, cognitive difficulties, and motor impairment.     

Risk factors for pancreatic fistula after stapled gland transection

The objective of this study was to identify risk factors for pancreatic fistula (PF) after stapled transection in distal pancreatectomy (DP). Patients undergoing DP using a stapler for transection between 2005 and 2009 were identified from a pancreatic resection database. Variables examined included patient and tumor characteristics, staple size, and the use of mesh reinforcement. Univariate and multivariate regression analyses were performed to identify risk factors for postoperative PF. One hundred forty-nine had stapled transection, and of these, 25 (17%) had mesh reinforcement. The overall morbidity and mortality rates were 28 per cent and less than 1 per cent; 34 (23%) were diabetic. The rate of clinically significant PF was 14 per cent. On univariate analysis, diabetes (P = 0.04), a firm pancreas (P = 0.03), use of mesh staple line reinforcement (P = 0.02), use of a 4.1-mm staple cartridge (P = 0.01), and blood loss greater than 100 mL (P = 0.01) were associated with higher pancreatic fistula rates. On multivariate analysis, only the presence of diabetes (OR, 4.17; 95% CI, 1.1-15.3; P = 0.03) and the use of a 4.1-mm cartridge (OR, 8.57; 95% CI, 1.2-60.2; P = 0.03) were independently associated with pancreatic fistula formation. Stapled pancreatic transection provides an acceptable PF rate after DP. Diabetes and staple size influence PF rates. In our experience, use of mesh staple line reinforcement did not reduce the incidence of PF after stapled transection.     

Need for urodynamic evaluation as a regular follow-up tool in assessment of long-term urological outcomes in patients with sacrococcygeal teratoma

AimTo assess the long-term urologic outcomes in follow-up of patients of sacrococcygeal teratoma (SCT) using urodynamic study (UDS) in addition to clinical and radiologic evaluation.MethodsA prospective study of clinical, radiological and urodynamic evaluation in patients with SCT who underwent resection between January 2002–June 2015 and were followed up till January 2016 was conducted.ResultsTotal 57 patients, 42 (73.7%) females and 15 (26.3%) males with 35 (62.4%) following treatment for benign and 22 (38.5%) for malignant disease were included. Twenty-eight of 57 (49.12%) had urological problems. Clinical complaints in 21 (36.8%) patients included stress urinary incontinence-14 (66.7%), enuresis-9 (42.9%), and poor stream or dribbling of urine-6 (28.6%). Eight of 51 patients (15.7%) had abnormal ultrasound findings, which included contracted, trabeculated thick walled bladder (3), bilateral hydronephrosis (3) and significant post void residue (PVR) (6). Seven of 57 underwent micturating cystourethrogram (MCU), 5 had an abnormal report[significant PVR (4), small trabeculated bladder (3), reflux (2) and large capacity bladder (1)]. Urodynamic study was done in 27 patients, 18/27 (66.7%) had abnormalities. Six patients without any clinical or ultrasonographic abnormalities had abnormal UDS. Total 28 (49.12%) had urological comorbidities. Three patients had overactive bladder, five dysfunctional voiding, one underactive bladder and one had giggle incontinence. Children were managed by behaviour therapy and pharmacotherapy.ConclusionUrodynamic evaluation could detect abnormalities in patients who had no urinary complaints or abnormality on ultrasound. The abnormalities have a potential for progressive upper tract damage. Urodynamics should be an integral part of urological surveillance in patients operated for SCT.Type of studyPrognostic study.Level of EvidenceLevel II (Prospective cohort study).     

Revision Total Hip Arthroplasty Using Imageless Navigation With the Concept of Combined Anteversion

Background

The purpose of the study was to evaluate the implant positions and clinical results of revision total hip arthroplasty (THA) using an imageless navigation with the concept of combined anteversion.

Breast‐Specific Gamma Imaging Influences Surgical Management in Patients with Breast Cancer

Breast‐specific gamma imaging (BSGI) is a physiologic breast imaging modality that provides more sensitive detection of breast lesions than mammography or ultrasound, and appears to have greater specificity than breast MRI. The purpose of this study was to evaluate how often BSGI changed surgical management in patients with breast cancer. Charts were reviewed from 218 consecutive eligible patients who had preoperative evaluation with BSGI or MRI before surgery for breast cancer from January 2008 to May 2010. Patients who were initially considered eligible for breast‐conserving therapy (BCT) were evaluated to determine how many ultimately had mastectomies. Patients who underwent mastectomy because of personal choice or ineligibility for BCT were excluded. Management was changed to mastectomy in 11.9% of those who had BSGI and 28.9% of those who had MRI. Review of pathology demonstrated that all patients who underwent mastectomies were not candidates for breast conservation. 15.4% of patients who underwent BCT based on BSGI findings required a single re‐excision due to positive surgical margins. 14.4% required mastectomy. In the MRI group, 18.8% required a single re‐excision, and 6.3% required mastectomy. Evaluation with BSGI changed management to mastectomy in a substantial proportion of patients believed to be eligible for BCT following standard imaging. BSGI is effective in evaluation of extent of disease in patients with breast cancer, and is comparable to MRI in terms of its influence on surgical management.     

The uncemented isoelastic/isotitan total hip arthroplasty. A 10-15 years follow-up with bone mineral density evaluation

The present study analyses the long-term outcome of isoelastic hip prostheses and their influence on the extent of periprosthetic bone remodeling. Ninety-two patients (102 hips) with Isoelastic/Isotitan uncemented total hip arthroplasty were evaluated after an average of 13.4 years. The average age of patients at surgery was 42.5 years. The clinical outcome was assessed based on the Harris hip score, complications and thigh pain. Bone mineral density (BMD) of the proximal femur in the seven Gruen zones was evaluated by dual-energy X-ray absorptiometry (DEXA) scans with the contralateral hip serving as a control. The average Harris hip score at the most recent follow-up was 72 points, with 72 hips (70%) rating completely pain free. Eight stems required revision whereas none of the cups showed any evidence of loosening. The change in the mean BMD values between the femora on the operated side and the contralateral femora averaged 15% for all zones Although the isoelastic stems are no longer used owing to their high loosening rate, it appears that this prosthesis preserved periprosthetic bone better than reported for cemented or uncemented metallic implants. Besides, the provision of a titanium coating on the isoelastic stem, comparable to that on the RM cup, would presumably have improved its long-term fixation by encouraging bony ongrowth.     

Birmingham hip resurfacing versus cementless total hip arthroplasty in patients 55 years or younger: A minimum five-year follow-up

Objective

The optimal reconstructive method for advanced degenerative hip disease in young adults is a topic of ongoing discussion. The purpose of this study is to report the largest single institution experience from the United States on the outcomes of Birmingham hip resurfacing (BHR) vs. cementless total hip arthroplasty (THA) in patients 55 years or younger at a minimum follow-up of five years. Currently, BHR is the only FDA-approved hip resurfacing implant available in the US.