Etiology, clinical features and outcome of epilepsia partialis continua in cohort of 51 children

Kravljanac, Ružica; Djurić, Milena; Jović, N.; Djordjevic, Maja; Zamurović, D.; Pekmezović, Tatjana

doi:10.1016/j.eplepsyres.2012.09.003

Cited by 22 publications

(35 citation statements)

References 18 publications

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“…The high percentage of EPC is in line with previous reports 6 and confirms that MDs are a frequent cause of this peculiar condition also in very young children 6,30,31. By contrast, in our series, seizures F I G U R E 4 A, Patient with non-syndromic mitochondrial encephalopathy carrying mutation in DNM1L gene had epileptic encephalopathy with focal seizures: Video-EEG recording at 5 years old detected epilepsia partialis continua (EPC) of the right arm, the EEG showed a generalized slowing and multifocal epileptic abnormalities not related to jerks.…”

supporting

confidence: 91%

“…In our series, we chose not to force the inclusion of epilepsy in of EPC is in line with previous reports 6 and confirms that MDs are a frequent cause of this peculiar condition also in very young children. 6,30,31 The occurrence of EPC confirms that the motor area is particularly vulnerable to the energy failure resulting from mitochondrial dysfunction, 2,32 which may be worsened by SE. Mechanisms triggering SE may be related to the oxidative stress, the impaired calcium homeostasis, and defects of specific factors, depending on the type of MD.…”

Section: Discussionmentioning

confidence: 95%

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Epileptic phenotypes in children with early‐onset mitochondrial diseases

et al. 2019

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Objectives:To determine the prevalence of epilepsy in children with early-onset mitochondrial diseases (MDs) and to evaluate the epileptic phenotypes and associated features. Materials and Methods:Children affected by MD with onset during the first year of life were enrolled. Patients were classified according to their mitochondrial phenotype, and all findings in patients with epilepsy versus patients without were compared. The epileptic features were analyzed. Results:The series includes 129 patients (70 females) with median age at disease onset of 3 months. The median time of follow-up was 5 years. Non-syndromic mitochondrial encephalopathy and pyruvate dehydrogenase complex deficiency were the main mitochondrial diseases associated with epilepsy (P < 0.05). Seizures occurred in 48%, and the presence of epilepsy was significantly associated with earlier age at disease onset, presence of perinatal manifestations, and early detection of developmental delay and regression (P < 0.001). Epileptic encephalopathy (EE) with spasms and EE with prominent focal seizures were the most detected epileptic syndromes (37% and 27.4%). Several seizure types were recorded in 53.2%, with the unusual association of generalized and focal epileptic pattern. Disabling epilepsy was detected in 63% and was associated with early seizure onset, presence of several seizure types, epileptic syndrome featuring EE, and the recurrence of episodes of status epilepticus and epilepsia partialis continua (P < 0.05).Conclusions: Epilepsy in children with early-onset MD may be a presenting or a prominent symptom in a multisystemic clinical presentation. Epilepsy-related factors could determine a worst seizure outcome, leading to a more severe burned of the disease. K E Y W O R D Schildren, early onset, epileptic encephalopathy, epileptic spasms, mitochondrial diseases, mitochondrial epilepsy | 185 MATRICARDI eT Al.

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confidence: 91%

Section: Discussionmentioning

confidence: 95%

Epileptic phenotypes in children with early‐onset mitochondrial diseases

et al. 2019

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“…There are other neurologic conditions that may present with EPC in childhood and mimic RE including focal cortical dysplasia, 46 Sturge-Weber syndrome, 47 and hemimegalencephaly. 48 Brain MRI features may help differentiate these disorders from RE.…”

Section: Differential Diagnosismentioning

confidence: 99%

Rasmussen's Encephalitis

Luat

2018

J Pediatr Epilepsy

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Rasmussen's encephalitis (RE) is a childhood epilepsy syndrome characterized by drug-refractory focal seizures especially epilepsia partialis continua, progressive hemiparesis, and neurocognitive decline. Diagnosis is mainly clinical and can be ambiguous during the early stages of the disease. Its underlying etiology is still elusive, but is suspected to be immune mediated. Treatment of seizures and disease progression is challenging, and although immunotherapy may delay its course, to date, surgical disconnection of the diseased hemisphere remains to be the only effective treatment providing seizure freedom in 60 to 85% of cases. The following review summarizes the current knowledge of the pathophysiology, clinical presentation, management, and treatment of RE.

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“…Kravljanac et al (2013) conducted a cohort study of 51 children with epilepsia partialis continua and recorded neurological consequences in 64.7% and fatal outcomes in 15.7% of the patients. 6 On the contrary, in adult-onset epilepsia partialis continua, the disorder maybe self-limiting [Trauma, stroke], remediable [Cortical dysplasia, AV malformation] or progressive (e.g, tumor, carcinomatous meningitis); the outcome is determined by the primary disease.…”

Section: Prognosismentioning

confidence: 99%

The Dance of Doom : A Rare Case of Epilepsia Partialis Continua

Gopalakrishnan¹,

Kandasamy²,

Shanmugam³

2015

jemds

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BACKGROUND: "Epilepsia Partialis Continua" is considered as the status epilepticus equivalent of simple partial motor seizures. First described by Kojewnikoff in 1894, this unique type of prolonged focal seizure continues to perplex neurologists for over a century now. Controversy looms large over the site of origin of this seizure. The unequivocal cortical origin of epilepsia partialis continua is substantiated by clinical, electrophysiologic and neurosurgical evidence. However, distant, or even subcortical sites of origin have been documented earlier. Despite our progressive knowledge about this disorder, to date, neither Antiepileptic drugs (AEDs) nor invasive/surgical treatment approaches have altered the course of this disease. We herein report a case of EPC who is admitted to our hospital. CASE REPORT: On the 31 st of July, Ms A, 20/female, educated upto XII Std, was brought to the hospital for h/o 4 episodes of seizures since morning. On examination, patient was drowsy, obeyed simple commands and largely responsive. She had persistent tachycardia

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Etiology, clinical features and outcome of epilepsia partialis continua in cohort of 51 children

Cited by 22 publications

References 18 publications

Epileptic phenotypes in children with early‐onset mitochondrial diseases

Epileptic phenotypes in children with early‐onset mitochondrial diseases

Rasmussen's Encephalitis

The Dance of Doom : A Rare Case of Epilepsia Partialis Continua

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