Clinical Neuroscience

[ANIMAL MODELS OF HUNTINGTON’S DISEASE]

GÁRDIÁN Gabriella

NOVEMBER 30, 2006

Clinical Neuroscience - 2006;59(11-12)

[Huntington’s disease is an autosomal dominantly inherited progressive neurodegenerative disorder. The main symptoms are choreiform, involuntary movements, personality changes and dementia. Huntington’s disease is a member of a group of diseases caused by CAG repeat expansions. One research aim is to determine the earliest molecular changes associated with Huntington’s disease. There is no possibility for this in humans, but various early changes have been identified in an animal model of Huntington’s disease. They are constructed by excitotoxin causing striatal lesion, or mitochondrial toxins inducing energy impairment, or by generating transgenic mice.]

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Clinical Neuroscience

[THORACIC MENINGOCELE]

FEKETE Tamás Fülöp, VERES Róbert, NYÁRY István

[Herniation of the meninges through a defect of the spinal canal is a spinal meningocele, and is usually located dorsally in the lumbosacral region. Meningoceles are usually part of a complex developmental disorder, or of a systemic disease, or it can be iatrogenic, as well. We report a very rare case of a true anterior thoracic meningocele.]

Clinical Neuroscience

[NEW METHODS IN THE INVESTIGATION OF BRAIN HYPOXIA]

SZILÁGYI Géza, NAGY Zoltán

[The main challenge is the investigation of mechanism for apoptosis research and the drug development. Mitochondria have a key position in the production of reactive oxygen species and in the evolution of apoptosis. More possible pathway will be known with the apoptosis investigation. For development of neuroprotective molecules could give strategies the investigation of apoptosis. Exact knowledge of apoptosis provides possibility to screen new neuroprotective molecules. We elaborate a research assay, which could provide quantitative and qualitative data about the free radical production and the mitochondrial transmembrane potential using confocal microscope. So thus we could screen drug candidate, neuroprotective molecules.]

Clinical Neuroscience

[BIOMECHANICS OF INTRACRANIAL ANEURYSMS]

FARKAS István, NYÁRY István, RAFFAI Gábor, ZILAHY G, MONOS Emil

[Introduction - Viscoelastic parameters of circumferential and meridional strips of ruptured and silent aneurysms were investigated (considered clinical and histological data either) in order to advance the prediction of risk of aneurysm rupture. Method - In our clinical practice, aneurysms managed by microsurgery aneurysm clipping were removed. Meridional and circumferential strips were cut. Strips were investigated by an uniaxial biomechanical instrument: distending force was recorded as the length of the strips was increased in steps. Normal stress-relaxation patterns were detected. The shape of strain curves well overlapped with the Standard Linear Solid Model curve, as had been expected. The viscosity, serial and parallel elastic moduli of the model were then computed. Results - Linear correlation was demonstrated amongst peek distending force detected and aneurysm strip thickness. Steric inhomogenity was detected at the meridional and circumferential strips. Strain-stress behaviour of ruptured and silent aneurysm specimen showed significant difference. Values of strips originated from patients suffered from hypertension as well as strips originated from aneurysms had been histologically found inflamed were higher. Discussion - Results of these observations are going to be used to set three dimensional computer model in cooperation with IT team of Budapest University of Technology and Economics to advance rupture risk prediction.]

Clinical Neuroscience

[Report of the Hungarian Epilepsy League]

SZUPERA Zoltán

Clinical Neuroscience

[CONGRESS CALENDAR]

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Evaluation of neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio in essential tremor

TAK Zeynal Abidin Ali, SENGUL Yildizhan

Introduction - Although essential tremor (ET) is the most common cause of tremor, the pathology and underlying mechanisms have not fully understood yet. In addition to kinetic tremor, patients may present several types of tremor, gait ataxia, hearing deficits and eye movement abnormalities. Non-motor symptoms and signs have also added to definition of ET. There is significant evidence indicating the neurodegenerative nature of the disease. New studies indicate that inflammation may have a place in the etiology. The neutrophil-to lymphocyte ratio (NLR) and the platelet-to lymphocyte ratio (PLR) have recently begun to be used as a marker of systemic inflammation. Our study aims at finding a clue for systemic inflammation in ET. Methods - 67 patients with ET and 40 healthy controls were recruited for the study. The total white blood cells (WBC), absolute neutrophil count, lymphocyte count and platelet count were retrieved. The NLR was calculated by dividing the neutrophil count by the lymphocyte count and the PLR was calculated by dividing the platelet count by the lymphocyte count. Results - Patient and control groups were similar in terms of age and gender. The mean age of patient group was 25.29 ± 8.24 years and that of control group was 26.77 ± 6.73 years. The NLRs were 1.85 ± 0.58 in the patient group and 1.96 ± 0.53 in the control group. For the patient group and the control group the PLRs were 103.52 ±32.80 and 91.26 ± 31.57 respectively. There were no statistically significant differences between the participants for both NLR and PLR. Conclusion - The pathophysiological mechanism for essential tremor (ET) remains unclear. However, there is an increasing amount of research being conducted on the subject. Discussions about ET’s definition as a neurodegenerative disease are ongoing. Although previous studies showed that neuroinflammation could be a part of etiology of disease, this study has failed to demonstrate systemic inflammation in ET.

Clinical Neuroscience

[Diffusion MRI measured white matter microstructure as a biomarker of neurodegeneration in preclinical Huntington’s disease]

KINCSES Tamás Zsigmond, SZABÓ Nikoletta, TÓTH Eszter, ZÁDORI Dénes, FARAGÓ Péter, NÉMETH Dezsõ, JANACSEK Karolina, BABOS Magor, KLIVÉNYI Péter, VÉCSEI László

[Background - Huntington’s disease is a progressive neurodegenerative disease, genetically determined by CAG trinucleotide expansions in the IT15 gene. The onset of the symptoms is related to the number of CAG triplets. Because the patients are asymptomatic in the early phase of the disease, in vivo biomarkers are needed to follow up the neurodegeneration and to test putative neuroprotective approaches. One such promising biomarker is the diffusion MRI measured microstructural alteration of the white matter. Methods - Seven presymtomatic, mutation carriers and ten age-matched healthy controls were included in the study. Diffusion parameters were compared between groups and correlated with measures describing neurodegeneration. In order to reduce the possible misregistration bias due to atrophy the analysis was restricted to the core of each fibre bundles as defined by maximal fractional anisotropy (Tract- Based Spatial Statistics). Results - Decreased fractional anisotropy, along with increased mean, parallel and perpendicular diffusivity was found in white matter tracts, mainly in the corpus callosum. An inverse correlation was detected between the fractional anisotropy and neurodegeneration score (derived from the number of CAG triplets and the patient age) from the areas of the left precentral gyrus, frontal lobe, corpus callosum and the capsula extrema. Altered diffusion parameters are promising biomarkers of the neurodegeneration in Huntington’s disease.]

Clinical Neuroscience

Gray matter atrophy in presymptomatic Huntington’s patients

KIRÁLY András, KINCSES Zsigmond Tamás, SZABÓ Nikoletta, TÓTH Eszter, CSETE Gergő, FARAGÓ Péter, VÉCSEI László

Background - Huntington’s disease is a progressive disease in which neurodegeneration is on-going from the early presymptomatic phase. Development of sensitive biomarkers in this presymptomatic stage that are able to monitor the disease progression and test the efficacy of putative neuroprotective treatments are essential. Methods - Seven presymptomatic Huntington mutation carriers and ten age-matched healthy controls were recruited. Six of the patients participated in a 24 months longitudinal study having MRI scans 12 and 24 months after the baseline measurements. High resolution T1 weighted images were carried out and voxel based morphometry was used to analyse the data. Apart of group differences, correlation of CAG repeat number with focal cortical thickness and with global gray matter volume was calculated. Results - Focal cortical atrophy was found bilaterally in the superior temporal sulcus and in the left middle frontal gyrus in presymptomatic Huntington patients in whom no sign of cognitive or motor deterioration was detected. Global gray matter atrophy (p<0.048) and decreased total brain volume was found. The number of CAG triplets showed no correlation with the focal gray matter atrophy and total brain volume. Strong correlation between the CAG repeat number and global gray matter volume was found (p<0.016). Conclusion - Cortical atrophy is apparent in the early, presymptomatic stage of the disease. With further validation in large patient sample atrophy measure could be biomarker of disease progression and putatively of neurodegeneration.