Epileptic Spasms: A Variety of Etiologies and Associated Syndromes

Abstract

Epileptic spasms have been described as a paroxysmal epileptic seizure type that consists of a series of motor movements, involving sudden flexion or extension predominantly of axial and/or proximal limb muscles, occurring with a noticeable periodicity, outside the age of infantile spasms, but have otherwise not been well characterized or described. The purpose of this study was to evaluate patients with epileptic spasms to describe the etiology and best treatment regimen for this seizure type. Twenty-eight children fit the selection criteria for this study, and their charts and electroencephalography (EEG) results were reviewed. Data regarding onset of seizures, characteristics of seizures, duration of seizures, activity at onset, treatments tried and/or failed, genetic or metabolic workup, and results of any imaging studies were collected. The results indicate that the genetic and metabolic workups that were done were most often negative or unrevealing. In addition, treatment regimens varied greatly from patient to patient, and it is evident that these seizures are refractory to many standard anticonvulsants, as well as the ketogenic diet. The results of imaging from this series point to a variety of structural abnormalities that could possibly explain a structural versus metabolic etiology for epileptic spasms. In conclusion, epileptic spasms remain an elusive seizure type to classify, diagnose, and treat. The results of the current series show a relationship between structural abnormalities on magnetic resonance imaging (MRI) and resultant epileptic spasms, which has further implications regarding surgical treatment of these seizures as opposed to the traditional treatment with anticonvulsants, to which epileptic spasms remain refractory.

Dental changes in children with congenital Zika syndrome

“…Epileptic seizures, stiffness, and spasms are symptoms present in CZS. According to Goldstein & Slomski , epilepsy is also present in other congenital infections, about 40% in toxoplasmosis and much less frequent in cytomegalovirus and herpes congenital infection. In the present study, the majority of the children presented these symptoms, corroborating with Alves et al, , who performed a cohort study showing that children with CZS had a high incidence of epileptic seizures with early onset, with spasm being the most observed type of crisis.…”

Revised terminology and concepts for organization of seizures and epilepsies: Report of the ILAE Commission on Classification and Terminology, 2005–2009

“…The term “epileptic spasms,” which includes infantile spasms, was recognized previously (Blume et al, 2001). Because spasms may continue past or even occur de novo after infancy (Camfield et al, 2003, Goldstein & Slomski, 2008), the more general term “epileptic spasms” is used. There was inadequate knowledge to make a firm decision regarding whether spasms should be classified as focal, generalized, or both; consequently, they have been placed in their own group as unknown. For focal seizures, the distinction between the different types (e.g., complex partial and simple partial) is eliminated.…”

Epileptic spasms in epilepsy with myoclonic-atonic seizures (Doose syndrome)

“…Doose identified a specific interictal EEG pattern resembling hypsarrhythmia in some patients with EMAS (Doose, ). However, to the best of our knowledge, ES have been reported in EMAS only in a single case (Goldstein and Slomski, ), without any specific details regarding the clinical presentation of the patient.…”