Seizures in Series: Similarities Between Seizures of the West and Lennox‐Gastaut Syndromes

Abstract

We observed seizures resembling infantile spasms in patients with Lennox-Gastaut syndrome (LGS). Infantile spasms, the type of seizures that occurs in patients who have West syndrome, have been well characterized by video-EEG studies and typically occur as a series of sudden generalized flexor or extensor jerks. The seizure types that occur in LGS have not been as clearly delineated. Some patients with West syndrome (WS) in early infancy later develop LGS. Using intensive video-EEG monitoring, we evaluated 14 LGS patients who had seizures that occurred in series. Clinically, the seizures greatly resembled infantile spasms, and the ictal EEG changes were identical to those that occur with infantile spasms. These findings expand the number of features known to be shared by these two syndromes and strengthen the hypothesis that the two syndromes represent age-related manifestations of similar epileptogenic processes.

Lennox-Gastaut sendromlu hastaların klinik özellikleri ve uzun süreli seyri

“…Boys are affected more frequently than girls (5,9). In this study, 15% of the patients were diagnosed as LGS at the age of 1-3 years, 40% were diagnosed at the age of 3-5 years and 45% were diagnosed at the age of 5-8 years.…”

“…Neuromotor development is affected more severely (5). While the risk of development of Lennox-Gastaut syndrome is lower in patients with West syndrome who are given ketogenic diet, prednisolone and ACTH, it has been reported that no relation was found between the age at the time of diagnosis of West syndrome and the etiology (12).…”

“…It is difficult to make a diagnosis, since clasical clinical and EEG findings are not present initially (4). Approximately half of the subjects have a history of infantile spasm (5). It is thought that autoimmunity plays a role, although the pathogenesis is not clear (6).…”

“…LennoxGestaut syndrome may occur related to asphyxia, central nervous system infection, cranial trauma, congenital infection and brain malformation or may be idiopathic. The prognosis is poorer in subjects in whom the etiological cause is found (5,7). Although multiple antiepileptic drugs are used, seizures can not be controlled most of the time.…”

Heterogeneity of Ictal SPECT Findings in Nine Cases of West Syndrome

“…The origin of hypsarrhythmia appears to be the cortical lesion as suggested by Pinard et al (13) in that a corticocortical pathway through the corpus callosum is important in the generalization of hypsarrhythmia. Repetitive tonic spasms have been observed in patients with Lennox-Gastaut syndrome (14) and periodic spasms (15), in which hypsarrhythmia was not demonstrated. We consider this additional evidence support for our proposition.…”