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and 2.2% for the occipital. Thus, in children with symptomatic epilepsy on a background of developmental abnormalities of the brain there was a sharp decline of the FA for frontal and occipital lobes and a huge increase of the MD for the frontal lobes. According to other authors state integrity of the corpus callosum provides stability of cognitive and emotional status of the children. Thus, the presence of these changes of FA and MD to some extent indicates the "interest" of the various regions of the brain in promoting epileptic seizures, and is being as predictor of the developing of the disease.
Conclusion
We revealed that in children with symptomatic epilepsy, regardless of its etiology, the presence of discrete regions of the abnormal changes of the white matter of the brain was discovered, while the pathological changes of white matter were localized not only in the epileptogenic, but also in the opposite hemisphere. The decreased anisotropy in these areas of heterotopic grey matter is likely to be caused by the comparisons of anisotropy in the abnormally located grey matter in the patients with anisotropy in white matter in control subjects. More interestingly, anisotropy and diffusivity changes were found also outside the anomaly regions in normal-appearing
tissue. These findings suggest that brain anomaly is often more extensive than the visible lesion, with widespread subtle malformation.
The regions with abnormal white matter FA and MD values not only matched to zones of limbic circle, but also to areas of intra- and interhemispheric connections uniting the frontal, temporal, parietal and occipital lobes of the brain. Determining of specific clusters of lesions of the brain tracts in symptomatic epilepsy in children, regardless of etiology, is important to predict the prognosis of the disease and the likely possibility of cognitive impairment. Thus, this study showed that symptomatic epilepsy in children is characterized by a variety of changes in the white matter of the brain tracts that lead to the dissociation of certain regions of the brain, which subsequently causes further breaking connections between cortical and subcortical regions and interrupting transfer of information. MRI diffusion allows to extend the representation of micro structural changes in integrity of gray and white matter of brain in symptomatic epilepsy and clarify the structural and metabolic etiologic subtype of the disease according to the recommendations of the International League Against Epilepsy.
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Aminova Dildora, Tashkent Institute for Paediatric Medicine, Neurology department. Tashkent, Uzbekistan. E-mail: gulzor5@rambler.ru
Clinical and neurophysiological manifestations of epileptic encephalopathy in children
Abstract: Summarizing the data of clinical and EEG correlations in epileptic encephalopathy, can reasonably link the permanent presence of psychiatric and neurological manifestations of epileptic activity in functionally important regions of the brain.
Clinical and neurophysiological manifestations of epileptic encephalopathy in children
We studied the clinical and neuroimaging manifestations of epileptic encephalopathy. Patients between the ages of 5 and 14 years was conducted clinical neurological examination, studied the state of intelligence in children using techniques Raven with a view to making diagnostic judgments about the level of formation of certain mental operations. An analysis of their EEG studies showed that the centrotemporal adhesions can cause severe speech, cognitive, mental, behavioural disorders in children without seizures. Thus, summarizing the clinical and electroencephalographs data correlations in epileptic encephalopathies and non paroxysmal epileptic disorders can reasonably link the permanent presence of psychiatric and neurological manifestations of epileptic activity in functionally important regions of the brain.
Keywords: Children, epileptic encephalopathy, elektroentsefalo graphics -performance
Introduction. Epilepsy and convulsive paroxysms are the divisions, integrating incoming information stored in a
common pathology of the nervous system in children. As research studies revealed that the frequency of seizures in the general population is 17-20 per 1,000 people, while epilepsy rate are 5-10 per 1000 person [1, 416]. According to the literature the epileptic encephalopathy consists depending on the form of 5 to 40% of behavioural, psychiatric and neuropsychological disorders and to 3-10% of all epileptic disorders [4, 1-15]. The new classification of epileptic syndromes of the International League Against Epilepsy introduced new terminology "epileptic encephalopathy" [2, 358]. It includes chronic disorders in which the severe cerebral dysfunction caused by epileptic discharges, manifested in the electroencephalogram (EEG) as a epileptic activity [3, 37]. The syndromes and well known forms of "catastrophic epileptic encephalopathy" are grouped to this column, in which frequent and severe seizures combined with a delay or regression of neuropsychiatric symptoms (Otahara syndromes, West, Lennox — Gastaut, Dravet et al.). It also includes epileptic aphasia, the Landau — Kleffner and epilepsy with permanent complexes spike — waves during slow-wave sleep, clinically severe speech disorders and other cognitive functions, sometimes without epileptic seizures [5, 250-264]. When non paroxysmal epileptic disorders associated with the involvement of nonspecific median structures of the brain, manifested in the EEG, usually discharges bilaterally synchronous epileptic activity which is a violation of the functions of determining the level of functional activity of the brain, the focus of attention and the general regulation of behaviour, control activities [6, 5-20]. This explains the modal nonspecific clinical manifestations and determines the characteristics of EEG, such as bilateral simultaneous and widespread of the brain. Accordingly, the bilaterally synchronous epileptic form discharges (BSER) exhibit obsessive-compulsive disorder, autism, schizophrenic disorders, attention deficit hyperactivity disorder, depersonalization — derealization, depressive syndrome, behaviour disorders and sleep. Similar mental and behavioural disorders associated with BSER repeatedly described in the literature [7, 54-64]. Focal cortical epileptic discharges contribute to violations of the characteristic functional localization. The prefrontal cortex is the center of attention motivated, goalsetting, social communication, planning, realization and control of emotions and related aspects of behavior: the mental and physical contact, aggression, avoidance [4, 1-15]. It is the executive element, processing information from lying behind
memory, and on this basis provides the behaviour associated with the socialization [7, 54-64].
Hence the complex observed in epileptic dysfunction of the frontal lobes of mental disorders. Specific mental disorders in non paroxysmal fronto-equity Epilepsy is infantile autism and other common disorder, schizophrenic form disorder.
The aim of our study was to investigate the clinical and neuroimaging manifestations of epileptic encephalopathy in children.
Materials and methods. The study was conducted in the neurological department of the clinic of the Tashkent Paedi-atric Medical Institute among 69 children aged 3 to 14 years with a diagnosis of epileptic encephalopathy, ofwhom 22 children with no reports of seizures debut of epileptic seizures observed in a wide age range from 1 month to 10 years (mean 6,3 ± 4,9).
Patients between the ages of 5 and 14 years was underwent clinical neurological examination, also their state of intelligence using techniques Raven are studied with a view to making diagnostic judgments about the level of formation of certain mental operations. The mental development level is studied by using some tsks depending on the success of the decision of tasks of certain series (A, B, C, D, E). Bayley Mental Scale test is used to evaluate the mental development of Children aged 3-5 years.
In this study, we used clinical, neurological and instrumental methods of investigation (EEG).
Results of the study.
The patients were admitted to the hospital with complaints about the back log in the psycho-speech development (38.5%), enuresis (46.2%), logo neurosis (15.4%), mental and behavioural disorders (41.0%).
An objective examination of subclinical symptoms revealed following symptoms such as reflex gemisindroma without pathological signs, weak signs of oral automatism, a symptom chvostek, increased deep reflexes, muscular hypotonia.
Neurological status was characterized in 55.6% of patients with severe epileptic encephalopathy intellectual deficit, autism, gross psychomotor retardation. The 44.4% of patients had hyperactive behavior, the backlog in training at the school relative to their peers, pugnacity, disinhibition, cognitive impairment. Clinical and neurological examination was performed in 55.6% and showed scattered focal symptoms with
severe intellectual deficit. The 18.8% of studied children with epileptic encephalopathy diagnosed with Lennox-Gastaut syndrome, Landau-Kleffner syndrome — 1.4% and 7.8% of West syndrome.
Lennox-Gastaut (SLG) was characterized by polymorphism of seizures, cognitive impairment, specific changes in EEG and resistance to therapy. It was characterized by the following types of attacks: paroxysms of falls, tonic seizures, atypical absence seizures, and sudden falls. In 4 children with this pathology it is observed a brief shutdown of consciousness. The attacks include sudden flexion of the neck and torso, leg extension, reduction of facial muscles, the rotational movement of the eyeballs, apnoea, flushing of the face. All children with this syndrome were observed in psychomotor development, which is compounded with the onset of the disease. EEG pattern showed diffuse slow spike-wave activity and bilateral synchronous with a frequency of 1 Hz 2.5, with an emphasis on the frontal and temporal lobes.
In children with the syndrome ofVesta during the seizures there was a rapid slope of the body forward with bent head. All children seizures occurred when the child is asleep or before waking. The frequency of attacks reached tens and hundreds of times a day. All children showed severe impairment of psychomotor development. West syndrome was characterized by generalized high amplitude slow wave or low amplitude rhythms beta EEG band. In one case, the focal epileptic activity was recorded. Interictal EEG changes as hypsarrhythmia, which were characterized by continuous high-amplitude slow-arrhythmic activity with numerous spikes or sharp waves without substantial synchronization between the hemispheres and the various departments within the same hemisphere of the brain.
In our study, Landau-Kleffner syndrome was observed in only1 child aged 4.5 years. This child on the background of the previously normal development has lost the ability to understand speech addressed to him and talk. It was characterized by a heavy defeat receptive (impressive, touch) aspects of speech. Hearing and nonverbal intelligence activities is not affected. The EEG showed paroxysmal changes bilaterally and in the temporal cortex.
In 22 children (31.9%) there was not any seizures in history. The mental status revealed anxiety, speech disorder, mutism, profound mental retardation disorders of speech development, at some stage, even featured a diagnosis of schizophrenia. These children were assigned to the II type of epileptic encephalopathy.
EEG pattern characterized by frontal foci of epileptic form activity. In 16 patients with behavioural disorders there were three severe mental development disorders such as autism, 1 patient — hallucinatory-catatonic disorders, in 2 — childhood schizophrenia. Thus, when the frontal epileptic foci dominant form of pathology behavioural, social and psychotic
circle. Violations of socialization prevalent in patients with left-sided foci. Especially characterized by a high frequency of frontal bilaterally synchronous discharges (56.7%) and in addition (15%) secondary bilateral synchronization in the frontal during lateralized focus, which speaks of the role of the involvement of the medial frontal limbic structures (cingulate and orbitofrontal cortex). Studies in children with behavioural and mental disorders without epileptic seizures in 31.8% of cases have been identified temporal epileptic foci. The clinical picture in the temporal bits divided into two main array: violation of speech functions in 3 patients with left-sided focus on the EEG; general, non-specific with respect to local development neuropsychological function disorders, personality, emotional and affective neurotic disorders in 4 patients with right tricks. Lateralization revealed in the analysis of disorders of speech and language functions: violation of semantic and pragmatic aspects of speech and language memory were observed more frequently in the left hemisphere focus, and any perception of sound pronunciation and intonation aspects of speech prevailed in the right-brain tricks. Thus, when the temporal foci clinical picture corresponds exactly to the functional specialization of the region of localization of epileptic disorders. An analysis of their own data EEG studies show that the centrotemporal adhesions can cause severe speech, cognitive, mental, behavioral disorders in children without seizures, which means that «rolandic focus is not so benign as once thought.» Thus, the described structural changes that can be saved and at the termination of epileptic activity, are responsible for the persistence of clinical disorders.
Conclusion.
Based on our observations, we can conclude that the main EEG phenomenon, along with high-amplitude activity was a gross violation of rhythm. High-amplitude activity was slow in nature, often combined with epileptic form that allowed to classify this as epileptic dysrhythmia. Such a flagrant violation of bioelectric rhythms indicate serious disorder that features selected structures are, as a rule, with the partial forms of epilepsy pacemakers epileptic activity. This fact determines the absence of seizures in patients with the above-mentioned violations of the psyche, which is particularly important, as is now the concept of the genetic mechanism of behavioural disorders and developmental disorders in such a group of patients, as well as dysfunction of the brain transmitter systems (dopaminergic and others.) In summarizing the clinical and electroencephalographic data correlations in epileptic encephalopathy and non paroxysmal epileptic disorders can reasonably link the permanent presence of psychiatric and neurological manifestations of epileptic activity in functionally important regions of the brain. The mechanisms of non paroxysmal epileptic disorders are common with mechanisms of epileptogenesis.
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1. Zenkov L. R. Clinical epileptology (manual for physicians). M. medical information publishing. 2002. - 416 p.
2. Zenkov L. R. Clinical electroencephalography (with elements epileptology). M. MEDpress. 2002. - 358 p.
