To conduct a systematic review of the literature regarding rates and predictors of favorable seizure outcome after resective surgery for epileptic spasms (ES) in pediatric patients. The Preferred Reporting Items for Systematic Reviews and Meta-Analyses standards were followed. We searched PubMed, EMBASE, and Cochrane CENTRAL for articles published on the prevalence or incidence of epileptic spasm since 1985.
View Article and Find Full Text PDFPupillary changes can be an important semiologic feature in focal epilepsy. Though the subcortical networks involving pupillomotor function have been described, cortical generators of pupillary dilation and constriction in humans are not well known. In this report, we describe a case of pupillary constriction occurring during seizures in a patient with drug resistant focal epilepsy.
View Article and Find Full Text PDFObjective: The risk of hydrocephalus following hemispherectomy for drug resistant epilepsy (DRE) remains high. Patients with pre-existing hydrocephalus pose a postoperative challenge, as maintaining existing shunt patency is necessary but lacks a clearly defined strategy. This study examines the incidence and predictors of shunt failure in pediatric hemispherectomy patients with pre-existing ventricular shunts.
View Article and Find Full Text PDFBackground: Anti-NMDAR encephalitis is a leading cause of autoimmune encephalitis in children. Untreated disease can lead to long-term neurological disability.
Case Report: We present siblings with pediatric-onset anti-NMDAR encephalitis.
Objective: We aimed to assess the treatment response of infantile-onset epileptic spasms (ES) in CDKL5 deficiency disorder (CDD) vs other etiologies.
Methods: We evaluated patients with ES from the CDKL5 Centers of Excellence and the National Infantile Spasms Consortium (NISC), with onset from 2 months to 2 years, treated with adrenocorticotropic hormone (ACTH), oral corticosteroids, vigabatrin, and/or the ketogenic diet. We excluded children with tuberous sclerosis complex, trisomy 21, or unknown etiology with normal development because of known differential treatment responses.
J Clin Neurophysiol
November 2022
Electroclinical features in surgical candidates with epileptic spasms differ significantly from the other focal epilepsy phenotypes. EEG findings tend to be more diffuse and less localizing in children with epileptic spasms. These are illustrated with five case studies to highlight three different categories of findings on interictal and ictal EEG: lateralizing , nonlateralizing , and false lateralizing .
View Article and Find Full Text PDFBackground: CDKL5 deficiency disorder (CDD) is associated with refractory infantile onset epilepsy, global developmental delay, and variable features that include sleep, behavioral disturbances, and movement disorders. Current treatment is primarily symptom-based and informed by experience in caring for this population.
Methods: We describe medication and non-medication approaches to treatment of epilepsy and additional key neurologic symptoms (sleep disturbances, behavioral issues, movement disorders, and swallowing dysfunction) in a cohort of 177 individuals meeting criteria for CDD, 154 evaluated at 4 CDKL5 Centers of Excellence in the USA and 40 identified through the NIH Natural History Study of Rett and Related Disorders.
Background: To quantitatively evaluate the brain MRI morphological abnormalities in patients with cyclin-dependent kinase-like 5 deficiency disorder (CDD) on a group level and longitudinally.
Methods: We performed surface-based MRI analysis on high-resolution T1-weighted images on three CDD patients scanned at age of three years, and compared with 12 age- and gender-matched healthy controls. We further examined the longitudinal morphological changes in one patient with a follow-up of 5 years.
Background And Purpose: Epilepsy surgery for medically refractory epilepsy secondary to cortical dysplasia in children poses special challenges. We aim to review the current available literature on the outcome after epilepsy surgery for cortical dysplasia in children and critically evaluate the prognostic predictors of outcome.
Methods: A comprehensive review of the literature was performed focusing on the outcome after epilepsy surgery for cortical dysplasia in children.
Purpose: To examine the long-term functional outcomes and their predictors using a patient/family centered approach in a cohort of children who had hemispherectomy. Functional outcome measures studied were the following: ambulation ability, visual symptoms, spoken language, reading skills, and behavioral problems.
Methods: We reviewed 186 consecutive children who underwent hemispherectomy between 1997 and 2009 at our center.
The spectrum of focal epileptogenic lesions and their clinical manifestations in children differ substantially from those seen in adults. In adults, mesial temporal sclerosis is the common lesion in surgical series; but in children, MTS is uncommon, and when it does occur, it exists frequently as dual pathology. The most common lesions in pediatric epilepsy surgery candidates are malformations of cortical development, developmental tumors, or encephalomalacia from infarction, hypoxia, trauma, or infection.
View Article and Find Full Text PDFObjective: Data on longitudinal seizure outcome after hemispherectomy in children are limited. This study explores the posthemispherectomy longitudinal seizure outcome and its predictors.
Methods: We reviewed 186 consecutive children who underwent hemispherectomy between 1997 and 2009 at our center.
Mutations in the smooth muscle-specific isoform of α-actin (ACTA2) cause vascular smooth muscle dysfunction leading to aortic aneurysm and moyamoya syndrome. A unique R179H mutation in ACTA2 has been reported to result in widespread smooth muscle dysfunction affecting vascular and extravascular smooth muscles. We report a 7-year-old girl with an ACTA2 R179H mutation manifesting with neonatal seizures due to multifocal infarcts, asymmetric motor deficits, global developmental delay, spasticity, congenital bilateral mydriasis, and a large patent ductus arteriosus.
View Article and Find Full Text PDFBackground: In patients with medically intractable epilepsy and diffuse unilateral hemispheric disease, functional or disconnective hemispherectomy is a widely accepted and successful treatment option. If recurrent seizures develop after disconnective hemispherectomy, management options become more complex and include conversion to anatomic hemispherectomy.
Objective: To present the outcomes of all patients undergoing reoperative hemispherectomy in 1 institution by 1 surgeon since 1998.