Clinical Neuroscience

[Conductive education for children with neurological diseases]

BALOGH Erzsébet, KOZMA Ildikó

JANUARY 20, 2009

Clinical Neuroscience - 2009;62(01-02)

[Conductive education, developed by the 40-s last century, spread out in the world in spite the lack of hard scientific evidences for its benefit. There are sorts of cerebral palsies (athetosis, ataxia) in which conductive education might have the unique role to help. In cerebral palsies of other sorts it is much helpful if the disturbance of body scheme and degree of somatomotor neglect are superior to the palsy. Short term results of conductive education are to see in the better movement coordination whilst the long term outcome is the increased activities of daily living.]

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Clinical Neuroscience

[Genetically determined neuromuscular disorders of some roma families living in Hungary (in English language)]

LÁSZLÓ Aranka, MAYER Péter, KÓBOR Jenõ, RÁCZ Katalin, TÁLOSI Gyula, ENDREFFY Emőke, HERCZEGFALVI Ágnes, HORTOBÁGYI Tibor, TISZLAVICZ László, BEREG Edit, KATONA Márta, SZABÓ János, KARCAGI Veronika

[The authors discuss the clinical and molecular genetic aspects of genetically determined neuromuscular disorders of some Roma families living in Hungary. Among the autosomal recessively inherited spinal muscular atrophic (SMA) group, 8 Caucasian children had the typical 7-8 exonal deletions of the SMA gene, but only 2 patients belonged to the Roma population. There was no difference in the molecular genetic findings among the Caucasian and the Roma SMA patients. All of them had 7- 8 exonal deletions of the SMA gene. We wanted to call attention to the founder mutation of the Roma population in 7 patients suffering from congenital myasthenia (CMS) from 3 Roma families. The 1267G deletion for CMS was detected by molecular genetic method. Clinical onset was pubertal and relatively slow progression of specific and phenotypic features for this founder mutation of acetyl-cholin receptor epsylon gene. In 2 patients (sister and brother) the sarcoglycanopathy 2C type C283Q mutation was proven in one Roma family suffering from limb-girdle muscular dystrophy (LGMD). Two out of the three facioscapular-humeral dystrophy (FSHD) Roma families carried 21.8 kb and 18.5 kb alleles in FSHD A1 gene (D4S139). In one family together with prenatal diagnosis founder mutation in FSHD A1 gene was detected, according to the autosomal dominant (AD) inheritence. In (F2) prenatal diagnosis was carried out, 18.5 kb/18.5 kb homozygosity was proven in the fetus, so the pregnancy was interrupted. In the CMS, LGMD and FSHD Roma patients ancient typical Roma founder mutations were found.]

Clinical Neuroscience

[Pain sensitivity changes in schizophrenic patients and animal models. Part I.]

TUBOLY Gábor, HORVÁTH Gyöngyi

[Diminished pain sensitivity in schizophrenic patients has been reported for more than 50 years, however little is known about the substrate and the basic mechanisms underlying altered pain sensitivity in this disease, therefore, relevant animal models are of decisive importance in the study of psychiatric diseases. The authors report a review consisting of two parts focusing on pain sensitivity changes in patients and in different animal models, which proved the eligibility as schizophrenia models and pain sensitivities have also been determined. The first session discusses the pain sensitivity changes in patients and chronic animal models induced by chronic drug treatments, social isolation or cerebral lesions. The results of human studies suggest that hypoalgesia in schizophrenia might be the endophenotype of this disease, however further studies are warranted to determine the clinical and biological correlation and the social and health consequences of hypoalgesia in schizophrenia. The animal data indicate that the pain sensitivity has changed in most models; however, there are significant controversies between the results, therefore, further studies are needed to find the ideal model.]

Clinical Neuroscience

[Neuroprotection in Parkinson’s disease and other neurodegenerative disorders: preclinical and clinical findings]

RÁKÓCZI Károly, KLIVÉNYI Péter, VÉCSEI László

[The authors summarized the evidence supporting neuroprotection based on the data available in the literature. In vivo and in vitro studies have indicated that many compounds can decrease neurodegeneration, excitotoxicity, oxidative stress, protein aggregation, disturbance of Ca2+ homeostasis and compensate the energy impairment. Selegiline, rasagiline, dopamine agonists and other molecules (ubiquinone, kynurenic acid, tocopherol, creatine, glatiramer acetate) exert neuroprotective effects in preclinical studies. Much less clinical data are available regarding neuroprotection in different neurological disorders. In this review, such preclinical and clinical evidences are summarized.]

Clinical Neuroscience

[Measurement of mental fatigability by task related spectral EEG. A pilot study (in English language)]

RAJNA Péter, HIDASI Zoltán, PÁL Iván, CSIBRI Éva, VERES Judit, SZUROMI Bálint

[Background - Task related EEG spectra are promising markers of mental activity. But the cooperation of the patients necessary for the registration limits its application in the neuro-psychiatry. Methods - EEG difference spectra on counting (EDSC) - was developed to detect the effect of a short calculation task on the spectral EEG. The originality of the task situation is a continuous mental work in a very short period of time, while the level of task difficulty is adapted to the patient’s actual mental capacity. While the rest pre-task and the post task EEG sections were compared, the results show the mental “EEG fatigability” caused by the short intensive cognitive activity. The first preliminary results have been demonstrated by a comparative study of two healthy and three patient (probable Alzheimer disease, post-stroke state without mental deficit and mixed type of dementia) groups. Results - Similarly to the findings of other authors, in addition to the differences of the alpha band seen on the temporo-parieto-occipital regions, the frontal localization and the beta band seem to be prominent, too. Demented patients had stronger EEG reactions than post-stroke patients without mental deficits and healthy elder persons had more extensive changes than the younger ones. Conclusions - The test can be considered as indirect marker showing the different mental fatigability in diverse pathological conditions and during the aging process. Effect of therapeutic processes can also be followed based on “key-lock principle”. Standardization of the test is essential for the introduction of EDSC to the every-day routine of clinical neuropsychiatry.]

Clinical Neuroscience

[Application of robot hand technique in the course of microneurosurgical operations]

CSÓKAY András, VALÁLIK István, JOBBÁGY Ákos

[Introduction - The aim of this study was to determine whether the new robot hand technique can help to avoid the complication in the course of high precise microneurosurgical operations. Methods - The physical efficacy was measured by tremorometry. The comperative study of the incidence of complications measured the clinical efficacy. Results - The tremors of the operating hand and the number of complications have decreased effectively. Conclusion - The precise level of robots could be available by novel robot hand technique. By this technique the microsurgical work has become more effective.]

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Clinical Neuroscience

[Is the implementation of Vojta therapy associated with faster gross motor development in children with cerebral palsy? ]

SANZ-MENGIBAR Jose Manuel , MENENDEZ-PARDIÑAS Monica , SANTONJA-MEDINA Fernando

[Vojta therapy has been reported as clinically beneficial for strength, movement and gross motor activities in individual cases and is being included within the second of three levels of evidence in interventions for cerebral palsy. The goal of this study is to understand the effect of Vojta therapy on the gross motor function. Our clinical trial followed a one group, pre-post design to quantify rates of changes in GMFM-88 after a two-months period undergoing Vojta therapy. A total of 16 patients were recruited. Post-intervention acceleration rates of GMFM-88-items acquisition (0.005; p<0.001) and Locomotor Stages (1.063; p<0.0001) increased significatively following Vojta the­rapy intervention. In this study, Vojta therapy has shown to accelerate the acquisition of GMFM-88-items and Loco­motor Stages in children with cerebral palsy younger than 18 months. Because functional training was not utilised, and other non-Vojta therapy intervention did not influence the outcome, Vojta therapy seems to activate the postural control required to achieve uncompleted GMFM-88-items. ]

Clinical Neuroscience

Role of positioning between trunk and pelvis in locomotor function of ambulant children with and without cerebral palsy

SANZ-MENGIBAR Manuel Jose, SANTONJA-MEDINA Fernando

Purpose - To understand if children with and without cerebral palsy share the same lumbar postural control threshold on the sagittal plane for the transition between each walking locomotor stage. Method - Observational analysis of sagittal trunk-pelvis kinematics of 97 children with cerebral palsy and 73 with typical development, according to their locomotor stage. Results - Among children with typical development, all average and minimum measurements of the sagittal lumbar curve during the gait events were correlated with age and the locomotor stages of development. Among children with cerebral palsy, there were significant correlations between all average and minimum values of the sagittal lumbar curve and locomotor stages of development but not age. Conclusions - We conclude that, for the same locomotor level, there are no common postural patterns between children with typical development and those with spastic bilateral cerebral palsy for the position between trunk and pelvis in the sagittal plane. Maximal lordosis reduction between trunk and pelvis may change with age or even training, but does not make a positive effect on the locomotor level, while basal and maintenance capacities could explain locomotor function. Trials that failed to assess quality of movement may now have a better understanding of how different interventions improve posture towards the next functional level.

Clinical Neuroscience

[Epidemiology, cost and economic impact of cerebral palsy in Hungary]

FEJES Melinda, VARGA Beatrix, HOLLÓDY Katalin

[Objective - The purpose of our communication was to determine the total cost of cerebral paretic patients in Hungary between 0 and 18 years and to assess their impact on the national budget. Methods - Based on the data of Borsod county we calculated the CP characteristics. The cost of CP was determined by routine care of individuals. Lost Parental Income and Tax were calculated on the basis of average earnings. The ratio of GDP, Health and Social Budget and Health Budget to CP is based on CP annual average cost and frequency. We have developed a repeatable computational model. Results - Of the risk groups, premature birth (30.97%), low birth weight (29.64%), perinatal asphyxia (19.47%) were the most common. Source is unknown of 37.61% of the cases. CP prevalence was 2.1‰. The two-sided (59.7%) and the one-sided (19.0%) spastic pareses dominated. The most serious form is the two-sided spastic paresis (42.5% GMFCS 3-5 degrees). Epilepsy was 22.0%, incontinence was 27%, mental involvement was 46%. Care for one child up to 18 years of age costs an average of 73 million HUF (€ 251,724). The lost family income was 27.36 million HUF (€ 94,345), and lost tax and health care contributions were 14.46 million HUF (€ 49,862). Additionally, 0.525% of the GDP, 0.88% of the full health and social budget and 1.83% of direct medical costs were spent for CP families. Conclusion - The cost of CP disease is significant. Costs can be reduced by improving primary prevention. From the perspective of the family and government, it is better to care for families so they can take care of their disabled children.]

Lege Artis Medicinae

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PAPP Zoltán

[The author provides an overview of the definition and aethiopathogenesis of fetal hypoxia and asphyxia, including possible forms of post asphyxial damages of mature fetuses as well as the incidence and neurological consequences of cerebralpalsy. Among from the screening methods of fetal hypoxia the special importance of colour-Doppler and pulse-Doppler technique (flowmetry), cardiotocography (CTG), non-stress test, biophysical profile examinations, amnioscopy and fetal pulsoxymetry are emphasized. In the prevention of fetal asphyxia the significance of preconceptional and prenatal care, the importance of maternal diseases, pathological conditions of the fetus and the umbilical cord, as well as the influence of factors during labour and delivery are also emphasized. The practice of the authors is supported by the recommendation of British obstetricians, when making a fetal scalp pH examination in case of pathological CTG findings, or performing a prophylactic cesarean section if the pH examination is not available. Since the number of postasphyxial damage in mature newborn infants is still high in Hungary, it is concluded, that an increase is required in the rate of cesarean sections when fetal asphyxia is present. ]