scholarly journals Case Report: Four Cases of Panayiotopoulos Syndrome Evolving to Juvenile Myoclonic Epilepsy

2020 ◽  
Vol 11 ◽  
Author(s):  
Hideo Enoki ◽  
Shinji Itamura ◽  
Shimpei Baba ◽  
Tohru Okanishi ◽  
Ayataka Fujimoto
Keyword(s):  
Focal Epilepsy ◽  
Age At Onset ◽  
Juvenile Myoclonic Epilepsy ◽  
Good Prognosis ◽  
Myoclonic Epilepsy ◽  
Future Evolution ◽  
Generalized Epilepsy ◽  
Long Term Observation ◽  
Panayiotopoulos Syndrome

Panayiotopoulos syndrome (PS) is a self-limited focal epilepsy appearing in childhood. Seizures in PS are self-limiting and do not usually continue into adulthood. Juvenile myoclonic epilepsy (JME) is the most common type of idiopathic generalized epilepsy, developing around puberty and continuing throughout adulthood. We describe four cases of PS in childhood in which JME developed in adolescence. Age at onset ranged from 4 to 8 years for PS, and 11 to 14 years for JME. JME developed after PS subsided, with the interval between last PS seizure and first JME seizure ranging from 1 to 10 years. No link between the two conditions has previously been described. Since PS is considered to show good prognosis and to be self-limiting, long-term observation has been considered unnecessary. No definitive factors were found to predict future evolution to JME in our series, so longer-term follow-up may be warranted for all PS patients.

scholarly journals Long-Term Seizures Outcome in Juvenile Myoclonic Epilepsy (JME): A Retrospective Cohort Study in an Indian Population

2021 ◽  
Author(s):  
Lakshminarayanapuram Gopal Viswanathan ◽  
Ravindranadh Chowdary Mundlamuri ◽  
Kenchaiah Raghavendra ◽  
Ajay Asranna ◽  
Aparajita Chatterjee ◽  
...  
Keyword(s):  
Social Issues ◽  
Tertiary Care ◽  
Clonic Seizure ◽  
Juvenile Myoclonic Epilepsy ◽  
Myoclonic Epilepsy ◽  
First Year ◽  
Medication Usage ◽  
Generalized Epilepsy

Abstract Objectives  Juvenile myoclonic epilepsy (JME) is the most prevalent form of generalized epilepsy in the community. Though the response to medication is good in most patients, long-term medication usage may lead to poor compliance, side effects, and other epilepsy-related social issues. The aim of this study was to describe the demography, clinical features, and therapeutic response in patients with JME who have had at least 10 years of duration of epilepsy. Materials and Methods A retrospective study of 56 patients (M:F=26:30; mean age: 14.5 ± 5.1 years) with JME attending neurological services in a tertiary care neurological center in South India with more than 10 years disease duration was performed. Results All patients had myoclonic jerks, 94% had generalized tonic–clonic seizure, and only 7.1% had features of absence seizures. Seizures were controlled in 60% of cases at the end of 10 years of epilepsy. Thirty-eight patients (67.8%) were on valproate. Control of seizures at the end of the first and fifth years of epilepsy, frequent myoclonic jerks at the onset, and treatment with valproate in the first year of disease resulted in controlled seizures at the end of 10 years. No patient was off medications at the end of 10 years of epilepsy or at the last follow-up. Conclusion About 40% of our patients continued to have seizures even 10 years after the onset of epilepsy. Regular and long-term medication may be required in JME. Early control of seizures, response to valproate, and frequent myoclonia at onset were found to confer better prognosis.

Long-term effectiveness and safety of antiepileptic drugs in children and adults

2021 ◽  
Vol 19 (2) ◽  
pp. 221-228
Author(s):  
Roza M. Shaimardanova ◽  
Rimma G. Gamirova
Keyword(s):  
Valproic Acid ◽  
Antiepileptic Drugs ◽  
Focal Epilepsy ◽  
City Hospital ◽  
Long Term Follow Up ◽  
Short And Long Term ◽  
Long Term Observation ◽  
Patients With Epilepsy

AIM: To conduct a retrospective comparative analysis of the efficacy and safety of epilepsy therapy with antiepileptic drugs. MATERIALS AND METHODS: The analysis of the treatment of 428 patients with epilepsy at the Childrens City Hospital No. 8 in Kazan, receiving antiepileptic drugs. RESULTS: It was found that valproic acid is more effective in the treatment of idiopathic generalized epilepsies compared to focal epilepsies (p = 0.0006). Valproate and carbamazepine were the most effective in the treatment of focal epilepsy with short- and long-term follow-up. Valproic acid is more effective than topiramate (p = 0.02), oxcarbazepine (p = 0.003), and levetiracetam (p = 0.003) in the treatment of focal epilepsy in short- and long-term follow-up. Carbamazepine is more effective than topiramate (p = 0.01), oxcarbazepine (p = 0.02), and levetiracetam (p = 0.001) in the treatment of focal epilepsy in long-term follow-up. It was revealed that more often they complained about side effects when using carbamazepine (63.2%). Levetiracetam was found to be better tolerated compared to valproate (p = 0.0006) and carbamazepine (p = 0.0006). Topiramate is better tolerated than carbamazepine (p = 0.02) and valproate (p = 0.03). Oxcarbazepine is better tolerated than carbamazepine in women (p = 0.04). CONCLUSIONS: When choosing an antiepileptic drug, it is necessary to be guided by the principle: first the basic, and then the drugs of the next generations, in the future, rely on information about the tolerability of the drug. It is necessary to evaluate the therapeutic effect of antiepileptic drugs with long-term observation, and use the criterion of complete absence of seizures as an indicator of the effectiveness of drugs.

scholarly journals Juvenile myoclonic epilepsy subsyndromes: family studies and long-term follow-up

Brain ◽  
2006 ◽  
Vol 129 (5) ◽  
pp. 1269-1280 ◽  
Author(s):  
Iris E. Martínez-Juárez ◽  
María Elisa Alonso ◽  
Marco T. Medina ◽  
Reyna M. Durón ◽  
Julia N. Bailey ◽  
...  
Keyword(s):  
Family Studies ◽  
Juvenile Myoclonic Epilepsy ◽  
Myoclonic Epilepsy ◽  
Long Term Follow Up

Long-term prognosis of typical childhood absence epilepsy: Remission or progression to juvenile myoclonic epilepsy

Neurology ◽  
1996 ◽  
Vol 47 (4) ◽  
pp. 912-918 ◽  
Author(s):  
E. C. Wirrell ◽  
C. S. Camfield ◽  
P. R. Camfield ◽  
K. E. Gordon ◽  
J. M. Dooley
Keyword(s):  
Juvenile Myoclonic Epilepsy ◽  
Absence Epilepsy ◽  
Myoclonic Epilepsy ◽  
Childhood Absence Epilepsy ◽  
Long Term Prognosis

The Ketogenic Diet and Related Therapies in “Novel” Situations

2016 ◽  
Author(s):  
Sudha Kilaru Kessler
Keyword(s):  
Ketogenic Diet ◽  
Treatment Option ◽  
Juvenile Myoclonic Epilepsy ◽  
Absence Epilepsy ◽  
Myoclonic Epilepsy ◽  
Childhood Absence Epilepsy ◽  
Idiopathic Generalized Epilepsy ◽  
Generalized Epilepsy

The ketogenic diet (KD) is often considered as a treatment option for medication-resistant focal epilepsies and symptomatic generalized epilepsies, but is perhaps less commonly considered for idiopathic generalized epilepsies. The evidence for the use of the KD in two common idiopathic generalized epilepsy syndromes, childhood absence epilepsy and juvenile myoclonic epilepsy, is presented here.

scholarly journals GABAergic Neuron Deficit As An Idiopathic Generalized Epilepsy Mechanism: The Role Of BRD2 Haploinsufficiency In Juvenile Myoclonic Epilepsy

PLoS ONE ◽  
2011 ◽  
Vol 6 (8) ◽  
pp. e23656 ◽  
Author(s):  
Libor Velíšek ◽  
Enyuan Shang ◽  
Jana Velíšková ◽  
Tamar Chachua ◽  
Stephania Macchiarulo ◽  
...  
Keyword(s):  
Juvenile Myoclonic Epilepsy ◽  
Myoclonic Epilepsy ◽  
Gabaergic Neuron ◽  
Idiopathic Generalized Epilepsy ◽  
Generalized Epilepsy

scholarly journals Juvenile myoclonic epilepsy: Under-diagnosed syndrome

2011 ◽  
Vol 64 (7-8) ◽  
pp. 381-385
Author(s):  
Ksenija Bozic ◽  
Ksenija Gebauer-Bukurov ◽  
Petar Slankamenac ◽  
Marija Knezevic-Pogancev ◽  
Slobodan Sekulic
Keyword(s):  
Clinical Manifestations ◽  
Juvenile Myoclonic Epilepsy ◽  
Good Prognosis ◽  
Clinical Syndrome ◽  
Myoclonic Epilepsy ◽  
Clinical Criteria ◽  
Age Related ◽  
Increased Risk ◽  
Epilepsy Diagnosis ◽  
Atypical Presentations

Introduction. Juvenile myoclonic epilepsy is an idiopathic, hereditary form of epilepsy. Although juvenile myoclonic epilepsy is a well defined clinical syndrome, attempts at diagnosing it commonly fail. Etiopathogenesis. The exact cause of juvenile myoclonic epilepsy remains unknown. Clinical, morphological and metabolic data suggest a preferential role for frontal regions in this syndrome. Several major genes for juvenile myoclonic epilepsy have been identified, but these genes account for only a small proportions of juvenile myoclonic epilepsy cases, suggesting multifactorial or complex inheritance in most. Clinical Manifestations. Juvenile myoclonic epilepsy is characterized by the triad of myoclonic jerks on awakening (all patients), generalized tonic-clonic seizures (>90% of patients) and typical absences (about one third of patients). Seizures have an age-related onset, circadian distribution and are frequently precipitated by sleep deprivation, fatigue and alcohol intake. Intelligence is normal. Diagnosis. Juvenile myoclonic epilepsy diagnosis is based upon clinical criteria and typical electroencephalographic findings (generalized pattern of spikes and/or polyspikes and waves). All other tests are normal. Treatment and Prognosis. Both medical treatment and counselling are important in the management of juvenile myoclonic epilepsy. Mono-therapy with valproate is the preferred treatment. Some of the newer antiepileptic drugs have been suggested as possible alternatives. Juvenile myoclonic epilepsy has a good prognosis. Lifelong treatment is usually considered necessary in vast majority of patients due to the increased risk of relapse if treatment is discontinued. Conclusion. Juvenile myoclonic epilepsy is a common, although under-diagnosed epileptic syndrome. The clinician should study the occurrence of myoclonic jerks and should consider atypical presentations.

scholarly journals Seizure Exacerbation by Antiepileptic Drugs

2005 ◽  
Vol 5 (5) ◽  
pp. 192-193 ◽  
Author(s):  
Jacqueline A. French
Keyword(s):  
Antiepileptic Drugs ◽  
De Novo ◽  
Focal Epilepsy ◽  
Juvenile Myoclonic Epilepsy ◽  
Absence Epilepsy ◽  
Absence Seizures ◽  
Incorrect Diagnosis ◽  
Juvenile Absence Epilepsy ◽  
Generalized Epilepsy

Worsening of Seizures by Oxcarbazepine in Juvenile Idiopathic Generalized Epilepsies Gelisse P, Genton P, Kuate C, Pesenti A, Baldy-Moulinier M, Crespel A Epilepsia 2004;45:1282–1286 Purpose Several studies have shown that carbamazepine (CBZ) may aggravate idiopathic generalized epilepsy (IGE). Oxcarbazepine (OXC) is a new drug chemically related to CBZ. We report six cases of juvenile IGE with a clear aggravation by OXC. Methods We retrospectively studied all patients with IGE first referred to our epilepsy department between January 2001 and June 2003 and treated with OXC. Results During this period, six patients were identified. All had an aggravation of their epilepsy in both clinical and EEG activities. OXC had been used because of an incorrect diagnosis of focal epilepsy or generalized tonic–clonic seizures (GTCSs) of undetermined origin (no syndromic classification of the epilepsy). Before OXC, only one patient had experienced a worsening of seizures with an inadequate drug (carbamazepine; CBZ). Four had juvenile myoclonic epilepsy, one had juvenile absence epilepsy, and one had IGE that could not be classified into a precise syndrome. OXC (dosage range, 300–1,200 mg/day) was used in monotherapy in all of them except for one patient. Aggravation consisted of a clear aggravation of myoclonic jerks (five cases) or de novo myoclonic jerks (one case). Three patients had exacerbation of absence seizures. One patient had worsened dramatically and had absence status, and one had de novo absences after OXC treatment. The effects of OXC on GTCSs were less dramatic, with no worsening in frequency in three and a slight increase in three. Conclusions OXC can be added to the list of antiepileptic drugs that can exacerbate myoclonic and absence seizures in IGE.

scholarly journals Juvenile Myoclonic Epilepsy. Update

2020 ◽  
Vol 12 (1S) ◽  
pp. 41-49
Author(s):  
I. V. Volkov ◽  
O. K. Volkova
Keyword(s):  
Meta Analysis ◽  
Juvenile Myoclonic Epilepsy ◽  
Myoclonic Epilepsy ◽  
Common Disease ◽  
Advantages And Disadvantages ◽  
Triggering Factors ◽  
Clonic Seizures ◽  
Generalized Epilepsy ◽  
Recommended Therapy

Juvenile myoclonic epilepsy (JME) is a common disease. However, some aspects of etiology and pathogenesis are not yet fully clarified. This publication describes the development of ideas about JME, the types of seizures that occur in JME, provides a list of triggering factors and a prognosis severity scale that depending on these triggering factors. The section on JME diagnostics discusses methods of neuroimaging, electroencephalography, and approaches to assessing the mental status of JME patients. The data of meta-analysis of prevalence and risk factors of refractory JME in the context of assessment of the disease state and prognosis are presented. The data on advantages and disadvantages of various Antiepileptic Drugs (AEDs) for the control of the disease are considered. The results of the own study of AED spectrum and effectiveness in gender-sensitive and ILAE-recommended therapy in adults, as well as the GENERAL study of perampanel efficacy and safety in patients with idiopathic generalized epilepsy, are presented. It confirmed the high efficacy of the perampanel primarily for  myoclonic seizures and generalized tonic-clonic seizures. A case study of a female patient with JME was also described, in which refractory to therapy was noted, including due to low compliance to the prescribed therapy.

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