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Zalaegerszeg, Hungary

Jacobi H.,University of Bonn | Rakowicz M.,Institute of Psychiatry and Neurology | Rola R.,Institute of Psychiatry and Neurology | Fancellu R.,Instituto Neurologico Carlo Besta | And 20 more authors.
Cerebellum | Year: 2013

Although ataxia is by definition the prominent symptom of ataxia disorders, there are various neurological signs that may accompany ataxia in affected patients. Reliable and quantitative assessment of these signs is important because they contribute to disability, but may also interfere with ataxia. Therefore we devised the Inventory of Non-Ataxia Signs (INAS), a list of neurological signs that allows determining the presence and severity of non-ataxia signs in a standardized way. INAS underwent a rigorous validation procedure that involved a trial of 140 patients with spinocerebellar ataxia (SCA) for testing of inter-rater reliability and another trial of 28 SCA patients to assess short-term intra-rater reliability. In addition, data of the ongoing EUROSCA natural history study were used to determine the reproducibility, responsiveness and validity of INAS. Inter-rater reliability and short-term test-retest reliability was high, both for the total count and for most of the items. However, measures of responsiveness, such as the smallest detectable change and the clinically important change were not satisfactory. In addition, INAS did not differentiate between subjects that were subjectively stable and those that worsened in the 2-year observation period. In summary, INAS and INAS count showed good reproducibility, but unsatisfactory responsiveness. The present analysis and published data from the EUROSCA natural history study suggest that INAS is a valid measure of extracerebellar involvement in progressive ataxia disorders. As such, it is useful as a supplement to the measures of ataxia, but not as a primary outcome measure in future interventional trials. © 2012 Springer Science+Business Media New York. Source


Toth Z.,County Hospital of Zala | Czoma V.,County Hospital of Zala
Applied Immunohistochemistry and Molecular Morphology | Year: 2011

The investigators adapted a method to process 150 bone marrow trephine biopsies per year at a general hospital providing pathological diagnosis for a subcenter hematological ward. In brief, specimens were fixed in 10% formaldehyde and embedded in epoxy resin at 56°C without decalcification. The resin was extracted with sodium-etoxide from the sections. Following rehydration, immunohistochemistry was carried out similar to the paraffin method with the usual antigen retrieval and Envision-DAB development. High resolution cytomorphology and immunomorphology was possible without semithin technique. The turnaround time was 3 days: 1 day each for fixation, for resin infiltration/polymerization and for cutting/staining. The antibody panel included 40 to 50 primary antibodies. The final diagnoses were aided by smear morphology, flow cytometry, and lymphoid organ pathology, as required by the special circumstances of the cases. Copyright © 2010 by Lippincott Williams & Wilkins. Source

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