Clinical Neuroscience

[Determining the term of schizencephaly]

KENÉZ József, LEEL-ŐSSY Lóránt

MAY 20, 2007

Clinical Neuroscience - 2007;60(05-06)

[Determining the term of schizencephaly 2007;60(05-06)]

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Clinical Neuroscience

[100 years of riddle… X. Jubilee Alzheimer’s disease congress on the 100th anniversary of disease description]

[100 years of riddle… X. Jubilee Alzheimer’s disease congress on the 100th anniversary of disease description 2007;60(05-06)]

Clinical Neuroscience

[TREATMENT OF SPASTIC UPPER LIMB WITH BOTULINUS TOXIN]

DÉNES Zoltán, FEHÉR Miklós, VÁRKONYI Andrea

[Objective - Examination of the effect of local botulinus toxin treatment on spastic upper limb, on patients with different brain injury. Patients and method - Prospective study in Traumatic Brain Injury Rehabilitation Unit of the National Institute for Medical Rehabilitation in the year 2003 and 2004. Thirteen patients (eight with stroke and five with traumatic brain injury) were treated locally on the spastic upper limb with 100 units botulinus A toxin. Results - Spasticity decreased one or two level on Modified Ashworth Scale, and in nine cases the good result were observed still at the end of 3rd month. No local or other complication was detected. Conclusions - Local treatment with botulinus toxin is an effective and safe method to decrease spasticity on upper limb in patients with different brain injury.]

Clinical Neuroscience

[NOVEL CELL-BIOLOGICAL IDEAS DEDUCIBLE FROM MORPHOLOGICAL OBSERVATIONS ON “DARK” NEURONS REVISITED]

GALLYAS Ferenc

[The origin, nature and fate of ”dark“ (dramatically shrunken and hyperbasophilic) neurons are century-old problems in both human and experimental neuropathology. Until a few years ago, hardly any cell-biological conclusion had been drawn from their histological investigation. On the basis of light and electron microscopic findings in animal experiments performed during the past few years, my research team has put forward novel ideas concerning 1. the nature of ”dark“ neurons (malfunction of an energystoring gel-structure that is ubiquitously present in all intracellular spaces between the ultrastructural elements), 2. the mechanism of their formation (non-programmed initiation of a whole-cell phase-transition in this gel-structure), 3. their capability of recovery (programmed for some physiological purpose), 4. their death mode (neither necrotic nor apoptotic), and 5. their relationship with the apoptotic cell death (the gel structure in question is programmed for the morphological execution of ontogenetic apoptosis). Based on morphological observations, this paper revisits these ideas in order to bring them to the attention of researchers who are in a position to investigate their validity by means of experimental paradigms other than those used here.]

Clinical Neuroscience

[LATE CONTRALATERAL EPILEPTOGENESIS AFTER INCOMPLETE SURGERY IN TEMPORAL LOBE EPILEPSY FOLLOWED ACROSS 18 YEARS]

HALÁSZ Péter, JANSZKY József, KELEMEN Anna, BARSI Péter, RÁSONYI György

[Objectives - To present evidence of changes in seizure semiology suggesting late contralateral epileptogenesis after incomplete surgery in a patient with temporal lobe epilepsy. Methods - The presently 36 year old female patient was followed across 18 years by clinical observation and EEG, and video-EEG monitored before and 18 years after surgery. Results - The patient had complex partial seizures defined by video-EEG which started from the right temporal lobe with an ictal spread to the contralateral (left) temporal lobe. After right amygdalo-hippocampectomy she did not become seizure free. Years after surgery a new type of seizure emerged. Video-EEG monitoring 18 yrs after surgery revealed two seizure types. One started in the right temporal region clinically resembling to the earlier seizures. The new seizure type showed left sided electroclinical pattern. The postoperative MRI detected bilateral hippocampal sclerosis. Side specific memory tasks revealed bilateral hippocampal dysfunctions with subdominant (right) side predominance. Conclusions - The well documented evolution from unilateral to bilateral seizures suggests late contralateral epileptogenesis in which the persisting seizure spread from the primary epileptogenic side and/or the earlier silent contralateral hippocampal sclerosis (HS) may play role. This case show that progressive changes with bilateral involvement may occur during the course of chronic temporal lobe epilepsy.]

Clinical Neuroscience

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[Congress calendar 2007;60(05-06)]

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Clinical Neuroscience

[The connection between the socioeconomic status and stroke in Budapest]

VASTAGH Ildikó, SZŐCS Ildikó, OBERFRANK Ferenc, AJTAY András, BERECZKI Dániel

[The well-known gap bet­ween stroke mortality of Eastern and Western Euro­pean countries may reflect the effect of socioeconomic diffe­rences. Such a gap may be present between neighborhoods of different wealth within one city. We set forth to compare age distribution, incidence, case fatality, mortality, and risk factor profile of stroke patients of the poorest (District 8) and wealthiest (District 12) districts of Budapest. We synthesize the results of our former comparative epidemiological investigations focusing on the association of socioeconomic background and features of stroke in two districts of the capital city of Hungary. The “Budapest District 8–12 project” pointed out the younger age of stroke patients of the poorer district, and established that the prevalence of smoking, alcohol-consumption, and untreated hypertension is also higher in District 8. The “Six Years in Two Districts” project involving 4779 patients with a 10-year follow-up revealed higher incidence, case fatality and mortality of stroke in the less wealthy district. The younger patients of the poorer region show higher risk-factor prevalence, die younger and their fatality grows faster during long-term follow-up. The higher prevalence of risk factors and the higher fatality of the younger age groups in the socioeconomically deprived district reflect the higher vulnerability of the population in District 8. The missing link between poverty and stroke outcome seems to be lifestyle risk-factors and lack of adherence to primary preventive efforts. Public health campaigns on stroke prevention should focus on the young generation of socioeconomi­cally deprived neighborhoods. ]

Clinical Neuroscience

[Consensus statement of the Hungarian Clinical Neurogenic Society about the therapy of adult SMA patients]

BOCZÁN Judit, KLIVÉNYI Péter, KÁLMÁN Bernadette, SZÉLL Márta, KARCAGI Veronika, ZÁDORI Dénes, MOLNÁR Mária Judit

[Background – Spinal muscular atrophy (SMA) is an autosomal recessive, progressive neuromuscular disorder resulting in a loss of lower motoneurons. Recently, new disease-modifying treatments (two drugs for splicing modification of SMN2 and one for SMN1 gene replacement) have become available. Purpose – The new drugs change the progression of SMA with neonatal and childhood onset. Increasing amount of data are available about the effects of these drugs in adult patients with SMA. In this article, we summarize the available data of new SMA therapies in adult patients. Methods – Members of the Executive Committee of the Hungarian Clinical Neurogenetic Society surveyed the literature for palliative treatments, randomized controlled trials, and retrospective and prospective studies using disease modifying therapies in adult patients with SMA. Patients – We evaluated the outcomes of studies focused on treatments of adult patients mainly with SMA II and III. In this paper, we present our consensus statement in nine points covering palliative care, technical, medical and safety considerations, patient selection, and long-term monitoring of adult patients with SMA. This consensus statement aims to support the most efficient management of adult patients with SMA, and provides information about treatment efficacy and safety to be considered during personalized therapy. It also highlights open questions needed to be answered in future. Using this recommendation in clinical practice can result in optimization of therapy.]

Clinical Neuroscience

[The importance of patient reported outcome measures in Pompe disease]

MOLNÁR Mária Judit, MOLNÁR Viktor, LÁSZLÓ Izabella, SZEGEDI Márta, VÁRHEGYI Vera, GROSZ Zoltán

[In recent decades it has become increasingly important to involve patients in their diagnostic and treatment process to improve treatment outcomes and optimize compliance. By their involvement, patients can become active participants in therapeutic developments and their observations can be utilized in determining the unmet needs and priorities in clinical research. This is especially true in rare diseases such as Pompe disease. Pompe disease is a genetically determined lysosomal storage disease featuring severe limb-girdle and axial muscle weakness accompanied with respiratory insufficiency, in which enzyme replacement therapy (ERT) now has been available for 15 years. In our present study, patient reported outcome measures (PROMs) for individuals affected with Pompe disease were developed which included questionnaires assessing general quality of life (EuroQoL, EQ-5D, SF36), daily activities and motor performance (Fatigue Severity Score, R-PAct-Scale, Rotterdam and Bartel disability scale). Data were collected for three subsequent years. The PROM questionnaires were a good complement to the physician-recorded condition assessment, and on certain aspects only PROMs provided information (e.g. fatigue in excess of patients’ objective muscle weakness; deteriorating social activities despite stagnant physical abilities; significant individual differences in certain domains). The psychological effects of disease burden were also reflected in PROMs. In addition to medical examination and certain endpoints monitored by physicians, patient perspectives need to be taken into account when assessing the effectiveness of new, innovative treatments. With involvement of patients, information can be obtained that might remain uncovered during regular medical visits, although it is essential in determining the directions and priorities of clinical research. For all orphan medicines we emphasize to include patients in a compulsory manner to obtain general and disease-specific multidimensional outcome measures and use them as a quality indicator to monitor treatment effectiveness.]

Clinical Neuroscience

[Family planning in multiple sclerosis: conception, pregnancy, breastfeeding]

RÓZSA Csilla

[Family planning is an exceptionally important question in multiple sclerosis, as women of childbearing age are the ones most often affected. Although it is proven that pregnancy does not worsen the long-term prognosis of relapsing-remitting multiple sclerosis, many patients are still doubtful about having children. This question is further complicated by the fact that patients – and often even doctors – are not sufficiently informed about how the ever-increasing number of available disease-modifying treatments affect pregnancies. Breastfeeding is an even less clear topic. Patients usually look to their neurologists first for answers concerning these matters. It falls to the neurologist to rationally evaluate the risks and benefits of contraception, pregnancy, assisted reproduction, childbirth, breastfeeding and disease modifying treatments, to inform patients about these, and then together come to a decision about the best possible therapeutic approach, taking the patients’ individual family plans into consideration. Here we present a review of relevant literature adhering to international guidelines on the topics of conception, pregnancy and breastfeeding, with a special focus on the applicability of approved disease modifying treatments during pregnancy and breastfeeding. The goal of this article is to provide clinicians involved in the care of MS patients with up-to-date information that they can utilize in their day-to-day clinical practice. ]

Clinical Neuroscience

Chronic form of Pisa syndrome after prolonged exposure to low-dose amisulpride treatment

ERDEM Şimşek Nazan, ÖZKAYNAK Sibel Sehur

Pisa syndrome is a movement problem defined by tonic, sustained lateral flexion with a slight posterior rotation of the trunk. It seems to be a side effect of antipsychotic medicine in most cases. The clinical duration of Pisa syndrome can be acute, chronic, or recurrent. As far as we know, no reports are available in the literature on the chronic form of Pisa syndrome caused by low-dose amisulpride. A case of refractory tardive dystonia form of Pisa syndrome during treatment with stable low-dose amisulpride is presented in this report. Long-term, low-dosage amisulpride therapy may induce tardive dystonia even in patients with no other risk factors for dystonia.