Clinical Neuroscience

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AUGUST 20, 2002

Clinical Neuroscience - 2002;55(07-08)

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Clinical Neuroscience

[The molecular genetic control of bony developmental malformations affecting the craniocervical junction and the cervical spine]

DÁVID Károly, KASÓ Gábor, THOROGOOD Peter V, STEVENS John M, CROCKARD H Alan

[In this review a new interpretation of the origin of bony developmental malformations affecting the craniocervical junction and the cervical spine is presented based on recent advances in the understanding of embryonic development of the spine and its molecular genetic control. Radiographs, CT and MRI scans or CT myelograms of patients with Klippel-Feil syndrome were used for demonstration. Detailed clinical and radiologial analysis of these patients was published earlier [David KM, Stevens JM, Thorogood P, Crockard HA. The dysmorphic cervical spine in Klippel-Feil syndrome: interpretations from developmental biology. Neurosurg Focus 1999;6(6):1.]. Homeotic transformation due to mutations or disturbed expression of Hox genes is a possible mechanism responsible for C1 assimilation. Notochordal defects and/or signalling problems, that result in reduced or impaired Pax-1 gene expression, may underlie vertebral fusions. This, together with asymmetrical distribution of paraxial mesoderm cells and a possible lack of communication across the embryonic mid-line, could cause the asymmetrical fusion patterns. The wide and flattened shape of the fused vertebral bodies, their resemblance to the embryonic cartilaginous vertebrae and the process of progressive bony fusion with age suggest that the fusions occur before or, at the latest, during chondrification of vertebrae. The authors suggest that the aforementioned mechanisms are likely to be, at least in part, responsible for the origin of the bony developmental malformations affecting the craniocervical junction and the cervical spine.]

Clinical Neuroscience

[Application of kinematic parameters for the assessment of impairments due to central motoneuron damage]

FAZEKAS Gábor, FEHÉR Miklós, KOCSIS László, STEFANIK Györgyi, BOROS Zsuzsanna, JURÁK Mihály

[Evidence based medicine requires objective methods for the assessment of status of the patients. The method described by the authors makes it possible to assess motoric impairment of patients in an objective way. It is based on three-dimensional motion analysis. Authors present the case history of two patients with spastic hemiparesis due to central nervous system damage. Changes in motoric impairment were followed by three-dimensional motion analysis. This method can be adapted for the assessment of motor impairment arising from other reasons as well.]

Clinical Neuroscience

[The evolution of psychoneuroimmunology]

SOMOGYI István, SZEKERES György, SZENDI István

Clinical Neuroscience

[Percutaneous procedure for treatment of diseased vertebral bodies with different etiology: vertebroplasty]

KASÓ Gábor, STEFANITS János, KÖVÉR Ferenc, HUDVÁGNER Sándor, DÓCZI Tamás

[Percutaneous vertebroplasty is a radiologically guided invasive technique consisting of the injection of surgical cement into the diseased vertebral body. The procedure results in immediate pain relief and strengthening of the bone due to the polymerization process of the filling material hardening the vertebral body and preventing further collapse. This method is suitable for the treatment of osteoporotic vertebral fractures and of osteolytic vertebral body metastases without neurological signs, in multiple appearance as well. Authors present technical details of the procedure performed by bi-directional fluoroscopy and combined CT-fluoroscopy control as well as short-term experience obtained by treatment of 17 patients.]

Clinical Neuroscience

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Clinical Neuroscience

Cholinesterase inhibitors and memantine for the treatment of Alzheimer and non-Alzheimer dementias

BALÁZS Nóra , BERECZKI Dániel, KOVÁCS Tibor

In aging societies, the morbidity and mortality of dementia is increasing at a significant rate, thereby imposing burden on healthcare, economy and the society as well. Patients’ and caregivers’ quality of life and life expectancy are greatly determined by the early diagnosis and the initiation of available symptomatic treatments. Cholinesterase inhibitors and memantine have been the cornerstones of Alzheimer’s therapy for approximately two decades and over the years, more and more experience has been gained on their use in non-Alzheimer’s dementias too. The aim of our work was to provide a comprehensive summary about the use of cholinesterase inhibitors and memantine for the treatment of Alzheimer’s and non-Alzheimers’s dementias.

Journal of Nursing Theory and Practice

[Burnout and depression among healthcare nursing staff]

IRINYI Tamás, NÉMETH Anikó

[The aim was to assess the levels of burnout and depressive symptoms among nursing staff members. We designed an online questionnaire for the present cross-sectional study, which was filled out by 10 285 participants between 01-27-2022 and 02-14-2022. The mean score of burnout showed a decrease compared to the score measured in 2021; however, the percent of nursing staff members suffering from burnout is still high (64.4%), from which 42% indicates severe burnout which would requires treatment. Concerning depressive symptoms, 57.8% of participants did not indicate the presence of depression, the rest presented different levels of depressive symptoms (6.8% showed severe depressive symptoms). Currently patients’ chance of recovery is under the expected level of what our healthcare system could provide, which is partially due to the affected mental state of healthcare nursing staff.]

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

[Rehabilitation results after severe traumatic brain injury ]

DÉNES Zoltán, MASÁT Orsolya

[To assess the rehabilitation outcome after severe traumatic brain injury. Retrospective evaluation of the rehabilitation process and prospective follow-up five years after discharge. Patients – Patients treated in 2013 at the Traumatic Brain Injury Unit, National Institute for Medical Rehabilitation were included in the study (n = 232). Ninety-nine of 232 patients were treated with severe traumatic brain injury. Data were available for 66/99 patients (67%). Fifty patients (13 women and 37 men) were successfully contacted for follow-up (51%), three patients deceased. The mean age of the patients was 42 years (range: 22-72). The majority of them (36/50) was injured in traffic accidents. The mean duration of coma and post-traumatic amnesia were 19 (1-90) and 45 days (5-150), respectively. Patients were admitted for rehabilitation on the 44th (11-111) day after the injury and were rehabilitated for 95 days (10-335). Thirty-eight patients became independent at daily living activity during the rehabilitation period, and none during the follow-up. Two patients needed moderate and one a little help for the daily life. After successful rehabilitation 4 patients continued their higher education, 24 patients worked (six in sheltered, six in the original, 12 in other workplaces). Twenty-two patients did not have permanent jobs, two of whom were retired. The majority of the patients were successfully reintegrated into society. More than half of the patients returned to work or continued their studies. These successes were greatly facilitated by the 40 years of experience and the multidisciplinary team working in the National Institute for Medical Rehabilitation. ]

Clinical Neuroscience

[Disease burden of Duchenne muscular dystrophy patients and their caregivers]

PÉNTEK Márta, HERCZEGFALVI Ágnes, MOLNÁR Mária Judit, SZŐNYI László Pál, KOSZTOLÁNYI György, PFLIEGLER György, MELEGH Béla, BONCZ Imre, BRODSZKY Valentin, BAJI Petra, SZEGEDI Márta, POGÁNY Gábor, GULÁCSI László

[Background and purpose - Data on the disease burden of Duchenne Muscular Dystrophy are scarce in Hungary. The aim of this study was to assess patients’ and their caregivers’ health related quality of life and healthcare utilisations. Methods - A cross sectional survey was performed as part of the European BURQOL-RD project. The EQ-5D-5L and Barthel Index questionnaires were applied, health care utilisations and patients’ informal carers were surveyed. Results - One symptomatic female carer, 50 children (boys 94%) and six adult patients (five males) participated in the study, the latter two subgroups were included in the analysis. The average age was 9.7 (SD=4.6) and 24.3 (SD=9.8) years, respectively. Median age at time of diagnosis was three years. The average EQ-5D score among children and adults was 0.198 (SD=0.417) and 0.244 (SD=0.322), respectively, the Barthel Index was 57.6 (SD=29.9) and 53.0 (SD=36.5). Score of satisfaction with healthcare (10-point Likert-scale) was mean 5.3 (SD=2.1) and 5.3 (SD=2.9). 15 children were hospitalised in the past 12 months for mean 12.9 (SD=24.5) days. Two patients received help from professional carer. 25 children (mean age 11.1, SD=4.4 years) were helped/supervisied by principal informal carer (parent) for mean 90.1 (SD=44.4) hours/week and further family members helped in 21 cases. Correlation between EQ-5D and Barthel Index was strong and significant (0.731; p<0.01) as well as with informal care time (-0.770; p<0.01), but correlation with satisfaction with health care was not significant (EQ-5D: 0.241; Barthel Index: 0.219; informal care: -0.142). Conclusion - Duchenne muscular dystrophy leads to a significant deterioration in the quality of life of patients. Parents play outstanding role in the care of affected children. This study is the first in the Central and Eastern European region that provides quality of life data in this rare disease for further health economic studies.]