Abstract
Introduction
Generalized paroxysmal fast activity (GPFA) is a rare and underreported EEG pattern known to be related to epileptic encephalopathy. We aimed to investigate the electroclinical spectrum of GPFA along with other atypical EEG features in patients without epileptic encephalopathy in routine EEG practice.
Methods
Outpatient EEG records of Hacettepe University Hospital were retrospectively reviewed between 2010 and 2020. Patients ≥ 18 years old with GPFA without epileptic encephalopathy were included. Electroclinical features of GPFA were analyzed. Atypical EEG features, epileptiform K-complexes and sleep spindles, and generalized polyspike train (GPT) were also investigated in this cohort.
Results
Most of the 19 included patients had refractory epilepsy (68%), while 16% were seizure-free. Generalized epilepsy (GE) was present in 58% of patients, and the rest had structural-focal epilepsy (26%), combined generalized and focal epilepsy (11%), or childhood occipital epilepsy (COE) (5%). Atypical EEG features with full atypical morphology were found in 91% of patients with GE. All patients with GPFA provoked by sleep had epileptiform K-complexes. The presence of GPT was not different between the GE and non-GE groups and was higher in patients with GPFA occurring only during sleep (p = 0.017). In two patients, GPFA frequency increased postictally. A transition from fixation-off sensitivity to GPFA occurred in a patient with COE.
Conclusion
In this study, GPFA had a wide diagnostic range from focal to generalized epilepsy. The association of GPFA with other electroclinical features was of importance mostly for sleep outcomes; this finding might lead to a better understanding of epileptogenesis.
Similar content being viewed by others
References
Gastaut H et al (1966) Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise known as “petit mal variant”) or Lennox syndrome. Epilepsia 7(2):139–179
Halasz P (1991) Runs of rapid spikes in sleep: a characteristic EEG expression of generalized malignant epileptic encephalopathies. A conceptual review with new pharmacological data. Epilepsy Res Suppl 2:49–71
Guye M et al (2001) Absence epilepsy with fast rhythmic discharges during sleep: an intermediary form of generalized epilepsy? Epilepsia 42(3):351–356
Halasz P et al (2004) Generalised paroxysmal fast activity (GPFA) is not always a sign of malignant epileptic encephalopathy. Seizure 13(4):270–276
Seneviratne U et al (2016) Atypical EEG abnormalities in genetic generalized epilepsies. Clin Neurophysiol 127(1):214–220
Aydin-Ozemir Z et al (2014) Long-term follow-up of adult patients with genetic generalized epilepsy with typical absence seizures and generalized paroxysmal fast activity in their EEG. Seizure 23(8):607–615
Bansal L et al (2019) Electroclinical features of generalized paroxysmal fast activity in typical absence seizures. J Clin Neurophysiol 36(1):36–44
Mohammadi M et al (2015) Asymmetrical generalized paroxysmal fast activities in children with intractable localization-related epilepsy. Brain Dev 37(1):59–65
Gastaut H et al (1963) An electro-clinical study of generalized epileptic seizures of tonic expression. Epilepsia 4:15–44
Markand ON (1977) Slow spike-wave activity in EEG and associated clinical features: often called “Lennox” or "Lennox-Gastaut’ syndrome. Neurology 27(8):746–757
Arzimanoglou A et al (2009) Lennox-Gastaut syndrome: a consensus approach on diagnosis, assessment, management, and trial methodology. Lancet Neurol 8(1):82–93
Sagi V et al (2017) Generalized paroxysmal fast activity in EEG: an unrecognized finding in genetic generalized epilepsy. Epilepsy Behav 76:101–104
Brenner RP, Atkinson R (1982) Generalized paroxysmal fast activity: electroencephalographic and clinical features. Ann Neurol 11(4):386–390
Scheffer IE et al (2017) ILAE classification of the epilepsies: position paper of the ILAE Commission for Classification and Terminology. Epilepsia 58(4):512–521
Karakis I et al (2014) Prognostic value of EEG asymmetries for development of drug-resistance in drug-naive patients with genetic generalized epilepsies. Clin Neurophysiol 125(2):263–269
Seneviratne U, Cook M, D’Souza W (2016) Epileptiform K-complexes and sleep spindles: an underreported phenomenon in genetic generalized epilepsy. J Clin Neurophysiol 33(2):156–161
Sun Y et al (2018) Generalized polyspike train: An EEG biomarker of drug-resistant idiopathic generalized epilepsy. Neurology 91(19):e1822–e1830
Pack AM (2019) Epilepsy overview and revised classification of seizures and epilepsies. Continuum (Minneap Minn) 25(2):306–321
Chin PS, Miller JW (2004) Ictal head version in generalized epilepsy. Neurology 63(2):370–372
Seneviratne U, Cook M, D’Souza W (2014) Focal abnormalities in idiopathic generalized epilepsy: a critical review of the literature. Epilepsia 55(8):1157–1169
Kim DW, Lee SY, Lee SK (2016) Focal epileptogenic lesions in adult patients with epilepsy and generalized epileptiform discharges. J Epilepsy Res 6(2):75–78
Seneviratne U, Cook M, D’Souza W (2012) The electroencephalogram of idiopathic generalized epilepsy. Epilepsia 53(2):234–248
Niedermeyer E (1966) Generalized seizure discharges and possible precipitating mechanisms. Epilepsia 7(1):23–29
Geyer JD, Carney PR, Gilliam F (2006) Focal epileptiform spikes in conjunction with K-complexes. J Clin Neurophysiol 23(5):436–439
Steriade M, McCormick DA, Sejnowski TJ (1993) Thalamocortical oscillations in the sleeping and aroused brain. Science 262(5134):679–685
Bartolomei F et al (1997) Prognostic factors for childhood and juvenile absence epilepsies. Eur Neurol 37(3):169–175
Acknowledgments
We appreciate M. Yasir Pektezel M.D. and all Hacettepe University Medical Faculty Department of Neurology EEG technicians for their contributions to this study.
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Ethical approval
None
Informed consent
None
Conflicts of interest
None of the authors have potential conflicts of interest to be disclosed.
Additional information
Publisher’s note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Rights and permissions
About this article
Cite this article
Das Pektezel, L., Tezer, F.I. & Saygi, S. Electroclinical spectrum of generalized paroxysmal fast activity in adults without epileptic encephalopathy. Neurol Sci 43, 3857–3866 (2022). https://doi.org/10.1007/s10072-021-05808-9
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10072-021-05808-9