Idiopathic late-onset absence status epilepticus: A case report with an electroclinical 14 years follow-up

Abstract

Late-onset absence status epilepticus (ASE) may be observed in adult and elderly patients as a late complication of idiopathic generalized epilepsy or de novo, usually related to benzodiazepines withdrawal, alcohol intoxication or psychotropic drugs initiation, but without history of epilepsy. EEG may be highly heterogeneous, varying from the 3 to 3.5 Hz spike-wave discharges typical of idiopathic generalized epilepsy to asymmetric irregular sharp and slow wave complexes. We report the clinical and neurophysiologic 14 years follow-up of a now 86 years-old woman, in whom we observed--at the age of 72--an idiopathic late-onset ASE, with a good clinical response to lamotrigine monotherapy, but with the persistence over years of the same interictal 3-3.5 Hz spike-wave epileptic activity at EEG. This case is singular because, with the available long follow-up, indicates that idiopathic generalized epilepsy may also occur in the elderly, with a late-onset ASE presentation. In this condition, it is particularly important to underline the essential role of EEG (urgent and ambulatory) for the diagnosis, management and monitoring of the disease.

Typical Spike-and-Wave Activity in Hypoxic-Ischemic Brain Injury and its Implications for classifying Nonconvulsive Status Epilepticus

“…This case also implies that extensive hypoxic-ischemic brain injury can spare the thalamocortical-corticothalamic network responsible for generating TSWA/absence seizure 9. As stated above, a genetic predisposition to develop TSWA/ASE may be the reason our patient manifested TSWA 12. It is also possible that all individuals have an inherent ability to produce TSWA and that, in our patient, the pattern of cerebral hypoxic-ischemic injury has “released” such ability 10.…”

The Syndrome of Absence Status Epilepsy: Review of the Literature

“…This is the case of the patients reported by Nightingale and Welch [6], Zambrelli et al [7], Bilo et al [8], and Pro et al [9], who all fulfill the criteria suggested for diagnosis of ASE.…”

“…Pro et al [9] report a female patient who presented recurring episodes of mental confusion and ideomotor slowing since the age of 54, usually lasting 5-6 h, with a frequency of 2/3 per year. Medical observation was sought at 77 years.…”

De novolate‐onset absence status epilepticus or late‐onset idiopathic generalized epilepsy? A case report and systematic review of the literature

“…Furthermore, the patient had no previous history of alcohol abuse or consumption of benzodiazepines, or other seizure triggering factors such as metabolic imbalance, systemic infections, or dehydration, which have been reported to typically occur in association with de novo late-onset ASE (Thomas et al, 1993;Thomas and Andermann, 1994;Bilo et al, 2014). Late-onset ASE occurs as a reactivation of IGE in adults and elderly individuals, in whom epilepsy has remitted after puberty or juvenile age (Andermann and Robb, 1972;Thomas et al, 1992;Fernández-Torre and Díaz-Castroverde, 2004;Trinka, 2005;Zambrelli et al, 2006;Bauer et al, 2007;Fernández-Torre and Rebollo, 2009;Pro et al, 2011). These patients have a previous IGE resolved after puberty or juvenile age, which reactivates in older age after many years of seizure freedom without any AED treatment and in the absence of any identifiable triggering factors.…”

“…without any history of prior seizures. These cases have been more frequently described in women, are typically provoked by benzodiazepine withdrawal, alcohol abuse, or psychotropic drug initiation (Thomas et al, 1992;Thomas et al, 1993;Thomas and Andermann, 1994;Fernández-Torre and Díaz-Castroverde, 2004;Bilo et al, 2014), and are associated with EEG recordings with interictal irregular background activity (Pro et al, 2011). Our patient would appear to add to the list of de novo late-onset ASE in the literature, however, the patient had no triggering factors, and her EEGs showed a persistence of interictal generalized epileptiform discharges on a normal background activity several weeks and months after the ASE.…”