Epilepsy and paroxysmal conditions

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Vol 10, No 4 (2018)
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8-16 2401

Aim. To assess the epileptic, cognitive and autistic manifestations of agenesis of the corpus callosum (ACC) in children.
Material and methods. Clinical characteristics of 31 patients (median age 6.6±0.9 years) with ACC were studied. Group I included patients with combined ACC and epilepsy manifestations (n=12); among them, patients with atypical children’s autism (n=3), genetically confirmed Aicardi syndrome (n=3), microdeletion of the 3X long shoulder (n=1), and Miller-Dieker syndrome (n=1). Group II included patients with combined ACC without epilepsy (n=12); among them: children with autism caused by an organic brain disorder (n=2), genetically confirmed Mowat-Wilson syndrome (n=1), and microcephaly (n=1). In Group III, there were patients with isolated ACC without epilepsy (n=7). All patients underwent a neurologic examination, an assessment of the mental status, and a neuropsychological testing that included diagnostic neuropsychological tests according to Skvortsov et al. (2000), a neuropsychological survey and calculation of the lateral preference according to Semenovich et al. (2002), a test for the dominant hemisphere according to Yassman et al. (1999), as well as brain EEG and MRI.
Results. In 75% of patients in Group I, symptomatic focal epilepsy was diagnosed (frontal-temporal – 4, frontal – 3, temporal – 2). Among other findings: 58% – complex partial seizures, 41.6% – complex partial seizures with secondary generalization, 16.7% – generalized seizures and their combination. In 16.7% of patients, there were atypical febrile seizures at the epilepsy debut with further transformation into symptomatic focal epilepsy of temporal localization. In 25% of patients in Group I, the neurogenetic Aicardi syndrome manifested in infantile (tonic) seizures up 10-20 attacks a day (symptomatic West syndrome). In 3 patients of Group I, atypical infantile autism was associated with symptomatic focal epilepsy (frontal-temporal – 2 and frontal – 1); 2 patients of Group II had infantile autism caused by an organic brain disease. In patients from Group I, the minimum value of the highest mental functions (HMF) score ranged from 25 to 51 (on average, Me =26.5) indicating severe cognitive disorders. In patients of Group III, the total HMF score varied from 77.5 to 87 (on average, Me =81) indicating mild cognitive disorders. Upon an inter-group comparison, patients with ACC associated with cerebral defects showed moderate to severe HMF disturbances (p < 0.002). The focal/multifocal epileptiform activity was recorded in 8 (67%) patients of Group I. In brain MRI scans of all patients of Group I, ACC was combined with various congenital development defects; in 3 (25%) patients of Group II, the structural lesions were dominated by congenital (hemispheric, arachnoid) cysts.
Conclusion. Patients in Groups I and II have combined cerebral pathology with a predominance of combined congenital malformations, mainly associated with abnormal neuronal migration that negatively impacts the prognosis. In 75% of patients with combined ACC associated with temporal or frontal epilepsy, a highly severe cognitive deficiency was found; in 72% of cases of isolated ACC, mild cognitive disorders with a rather favorable prognosis were noted.

17-25 787

Aim – analyze the current practice of management in patients with neonatal seizures and perinatal brain disorders, and determine the ways to prevent long-term neurological complications.
Materials and methods. The study group included 140 children (aged from 2 weeks to 18 months) with neonatal seizures and perinatal brain abnormalities. In these cases, the obstetric and early postnatal history was analyzed; all patients underwent video-EEG monitoring, cervical spine X-ray, neuroimaging, and a Doppler ultrasound test.
Results. We found a number of reliable ante- and intra- partum predictors of neonatal seizures. According to the medical documentation from the neonatal pathology departments, local neonatologists have difficulties in diagnosing and verifying the type and duration of seizures. In most patients with neonatal seizures, we detected epileptiform EEG activity, signs of birth defects (according to X-ray) and marked changes (according to neuroimaging) in the cervical spine.
Conclusion. The results confirm that neonatal seizures are one of the first symptoms of severe brain damage, including intra-natal damage. Evolution of neonatal seizures into drug-resistant epilepsy and further disability is associated with insufficient knowledge of neonatal seizures, standards for their diagnosis, therapy and multidisciplinary observation.

26-38 641

The aim is to develop an antiepileptic drug based on polymer nanoparticles with 2-ethyl-6-methyl-3-oxypyridine succinate to facilitate the drug transport through the blood-brain barrier.

Materials and methods. The nano-drug was created using the biologically active substance 2-ethyl-6-methyl-3-hydroxypyridine succinate and polybutyl cyanoacrylate (PBCA) nanoparticles. The advantages of this nano-form over the active ingredient of the same drug were studied using experimental models: the maximum electroshock test (MES), the antagonism test with corazol, models with a cobaltinduced epileptic focus and secondary generalized convulsions, and models of status epilepticus.

Results. The antiseizure effects of the nanoform on the experimental models of epilepsy are identified.

Conclusion. The nano-drug reduces the number of secondary generalized clonic-tonic seizures by 7.8 times; it also reduces 10-fold the animal mortality and diminishes the seizure manifestations that occur in the interictal period of the epileptic status.

39-52 1153

This article presents the anamnestic, clinical, electro-encephalographic and neuroimaging findings in 5 patients with epilepsy combined with Wolf-Hirschhorn syndrome (WHS). According to our data and the results of others, this combination has its specific characteristics. These include: a high incidence of epilepsy in patients with WHS (50-100% of cases), an early debut of seizures (mainly in the first year of life), fever-provoked seizures, and a variety of seizure types – focal paroxysms, bilateral tonic-clonic seizures, atypical febrile seizures, atypical absences and epileptic spasms. In addition, there may be frequent epileptic seizures tending toward status epilepticus, a slowing of the major EEG activity, a local EEG slowing (mainly in the posterior and bi-frontal areas), and regional / multiregional epileptiform activity. In more than 50% of cases, the diffuse peakwave activity is observed; the broad spectrum anti-epileptic drugs are highly efficient in 80% of cases. Based on this study, we propose recommendations for the management of patients with epilepsy combined with WHS.

54-60 727

Though brain hemiatrophy is not among the leading causes of symptomatic epilepsy; the problem remains important and is of great practical interest to neurologists and neurosurgeons in Russia. In this article; we present an example of successful surgical treatment of Dyke-Davidoff-Masson syndrome. Here; functional hemispherotomy was performed in a 22 years old female patient; as a result; the occurrence and intensity of epileptic seizures decreased. This clinical case provides hope for this procedure to show even better results if performed in early childhood.


61-71 1259
In recent decades, MRI increased the success rate of the diagnosis and surgical treatment of epilepsy. Approximately 30% of patients with epilepsy suffer from the drug-resistant form of this disease. Focal cortical dysplasia is the most common abnormality of cortical development that is often associated with drug-resistance. Surgery has an important role in the treatment of epilepsy caused by focal cortical dysplasia. Focal cortical dysplasia is classified by its morphological characteristics. By large, focal cortical dysplasia can be diagnosed using the up-to-date neuroimaging tools. The aim of that paper is to review the methods available for the diagnosis of focal cortical dysplasia.
72-82 760
Consequences of traumatic brain injury (TBI) in children and adolescents pose a major medical and social problem, as TBI interferes with neuro ontogenesis. Among these consequences, paroxysmal disorders that manifest in the long-term after TBI represent an important issue. These disorders include posttraumatic headaches, posttraumatic epilepsy and subclinical epileptiform EEG activity. In this review, clinical manifestations, pathophysiology and management of the long-term post-TBI paroxysmal disorders in children and adolescents are discussed.
83-93 1288
About 30% of patients with temporal epilepsy are resistant to antiepileptic drugs; in such cases, surgical treatment is considered to be the best option. Existence of an extra-temporal epileptogenic focus in the insula is a possible cause of seizures after temporal lobe surgery in about 20% of patients. Because of its localization, the anatomical features of the insular lobe, as well as the entire concept of insular epilepsy have not been well documented. Yet, it has been established that the seizures developing from the insular cortex can mimic the paroxysms arising from the temporal lobe. When the insula is involved in the epileptic process, resection of the insular cortex improves the outcome of surgical treatment. Rational and well-based selection of candidates for surgical treatment is of paramount importance for patients with drug-resistant epilepsy. The article presents a review of the up-to-date literature on this matter.

ISSN 2077-8333 (Print)
ISSN 2311-4088 (Online)