title: Challenges in identifying Lennox-Gastaut syndrome in adults: A case series illustrating its changing nature.
abstract: The variable presentation and progression of Lennox-Gastaut syndrome (LGS) can make it difficult to recognize, particularly in adults. To improve diagnosis, a retrospective chart review was conducted on patients who were diagnosed as adults and/or were followed for several years after diagnosis. We present 5 cases that illustrate changes in LGS features over time. Cases 1 and 2 were diagnosed by age 8 with intractable seizures, developmental delay, and abnormal EEGs with 1.5-2 Hz SSW discharges. However, seizure type and frequency changed over time for both patients, and the incidence of SSW discharges decreased. Cases 3, 4, and 5 were diagnosed with LGS as adults based on current and past features and symptoms, including treatment-resistant seizures, cognitive and motor impairment, and abnormal EEG findings. While incomplete, their records indicate that an earlier LGS diagnosis may have been missed or lost to history. These cases demonstrate the need to thoroughly and continuously evaluate all aspects of a patient's encephalopathy, bearing in mind the potential for LGS features to change over time.
authors.affiliation: The Children's Hospital at TriStar Centennial Medical Center, 330 23rd Ave North, Suite 450, Nashville, TN 37203, USA.
authors.forename: Jesus Eric
authors.initials: JE
authors.lastname: Piña-Garza
authors.affiliation: Banner University Medical Center, 1012 E Willetta Street, Suite 54, Phoenix, AZ 85006, USA.
authors.forename: Steve
authors.initials: S
authors.lastname: Chung
authors.affiliation: Boston University School of Medicine, 72 E Concord Street C-3, Boston, MA 02118, USA.
authors.forename: Georgia D
authors.initials: GD
authors.lastname: Montouris
authors.affiliation: Duke University Hospital, DUMC 102350, 297 Hanes House, Durham, NC 27710, USA.
authors.forename: Rodney A
authors.initials: RA
authors.lastname: Radtke
authors.affiliation: Department of Neurology, Miami Children's Hospital, 3100 SW 62nd Ave, Miami, FL 33155, USA.
authors.forename: Trevor
authors.initials: T
authors.lastname: Resnick
authors.affiliation: Idaho Comprehensive Epilepsy Center, 1499 W Hays St, Boise, ID 83702, USA.
authors.forename: Robert T
authors.initials: RT
authors.lastname: Wechsler
keywords: Adult; Diagnosis; EEG, electroencephalogram; Features; LGS; LGS, Lennox–Gastaut syndrome; Lennox–Gastaut syndrome; SSW, slow spike–wave discharge; SW, spike–wave discharge; Symptoms