Temporal seizure emerging from a cluster of eyelid myoclonia in a teenager with Jeavons syndrome

2020 ◽  
pp. 10.1212/CPJ.0000000000000864 ◽  
Author(s):  
Ifrah Zawar ◽  
Linda Franic ◽  
Elia Pestana Knight

Eyelid myoclonia with absences (EMA) or Jeavons Syndrome is an idiopathic generalized epilepsy (IGE) characterized by eyelid myoclonia with or without absences, eye-closure elicited EEG paroxysms (generalized polyspikes or spike-wave-complexes) and photosensitivity1.

2002 ◽  
Vol 36 (4) ◽  
pp. 534-543 ◽  
Author(s):  
Beng-Yeong Ng

Objective: To review the literature on self-induced epileptic seizures and apply psychiatric knowledge to define possible causes and treatment recommendations. Methods: A review of MEDLINE literature on self-induced epileptic seizures was conducted, followed by cross-referencing with the relevant neurologic, psychiatric and paediatric books and journals. Results: The reported prevalence of self-induction varies depending on the setting. In most instances, self-inducers are people affected by photosensitive primary generalized epilepsy, in whom self-induction is by intermittent photic stimulation or, more rarely, pattern stimulation or eye closure. Self-induced seizures are most commonly absences with spike-wave, though eyelid myoclonia and generalized myoclonic jerks occur. Earlier studies indicate that the majority of self-inducers were of subnormal intelligence but more recent ones indicate that as a group they could not be considered mentally retarded. Monitoring in a well-lit environment is indicated in any therapy-resistant photosensitive patient to determine whether or not selfinduction occurs. Self-induced epilepsy is notoriously resistant to therapy and reasons for this resistance are discussed. An explanatory model for the initiation and maintenance of selfinduction behaviour is also included. Conclusions: It is difficult to determine from published papers just what level of clinical difficulty self-induction of seizures actually posed to the patients and clinicians. In some cases there is contradictory evidence as to the degree of willfulness or self-induction. Self-induced epileptic seizures may be an important aspect of poor seizure control in a subgroup of epilepsy sufferers. These seizures might be modifiable by psychiatric intervention or behavioural modification.


Neurosurgery ◽  
2020 ◽  
Vol 87 (5) ◽  
pp. E578-E583 ◽  
Author(s):  
Vasileios Kokkinos ◽  
Alexandra Urban ◽  
Nathaniel D Sisterson ◽  
Ningfei Li ◽  
Danielle Corson ◽  
...  

Abstract BACKGROUND AND IMPORTANCE At least 25% of patients with idiopathic generalized epilepsy do not obtain adequate seizure control with medication. This report describes the first use of responsive neurostimulation (RNS), bilaterally targeting the centromedian/ventrolateral (CM/VL) region in a patient with drug-refractory Jeavons syndrome (eyelid myoclonia with absences). CLINICAL PRESENTATION A patient, diagnosed with eyelid myoclonia with absences (EMA) and refractory to medication, was offered RNS treatment in the CM/VL region of the thalamus. Stimulation was triggered by thalamic neural activity having morphological, spectral, and synchronous features that corresponded to 3- to 5-Hz spike-wave discharges recorded on prior scalp electroencephalography. CONCLUSION RNS decreased daily absence seizures from a mean of 60 to ≤10 and maintained the patient's level of consciousness during the occurring episodes. This therapy should be evaluated further for its potential to treat patients with pharmaco-refractory generalized epilepsy.


Epilepsia ◽  
2004 ◽  
Vol 45 (1) ◽  
pp. 90-91 ◽  
Author(s):  
Martin J. Gallagher ◽  
Lawrence N. Eisenman ◽  
Kelly M. Brown ◽  
Ebru Erbayat-Altay ◽  
Hrvoje Hecimovic ◽  
...  

2014 ◽  
Vol 45 (S 01) ◽  
Author(s):  
C. von Stülpnagel-Steinbeis ◽  
C. Funke ◽  
C. Haberl ◽  
K. Hörtnagel ◽  
J. Jüngling ◽  
...  

2021 ◽  
pp. 097275312096875
Author(s):  
Haritha Koganti ◽  
Shasthara Paneyala ◽  
Harsha Sundaramurthy ◽  
Nemichandra SC ◽  
Rithvik S Kashyap ◽  
...  

Background: Idiopathic generalized epilepsy is defined as seizures with a possible hereditary predisposition without an underlying cause or structural pathology. Assessment of executive dysfunction in idiopathic generalized epilepsies based on standard Indian battery is not available in the literature. Aims and Objectives: To assess specific executive functions affected in patients with idiopathic epilepsy and their association with various variables. Materials and Methods: Type of observational cross-sectional study, where clinical profile of all idiopathic epilepsy patients attending the neurology OPD was studied and their executive higher mental functions were assessed using the NIMHANS battery. Results: A total of 75 idiopathic generalized epilepsy patients were included in the study. Executive functions that were commonly found abnormal in our study were word fluency ( P ≤ .001), category fluency ( P < .001), verbal n-back ( P < .001), Tower of London ( p < 0.01), and Stroop test ( P < 0.01). Executive functions showed a significant correlation with age at symptom onset, duration of epilepsy, and in those with uncontrolled seizures. Conclusion: Patients of idiopathic generalized epilepsy according to the present study were found to have significant executive dysfunction in multiple domains. This necessitates the screening for executive dysfunctions, which if detected should prompt the clinician to initiate cognitive retraining.


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