SVIBOR - Project code: 3-01-028

MINISTRY OF SCIENCE AND TECHNOLOGY

Strossmayerov trg 4, HR - 10000 ZAGREB
tel.: +385 1 459 44 44, fax: +385 1 459 44 69
E-mail: ured@znanost.hr

SVIBOR

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Project code: 3-01-028


AMYOTROPHIC LATERAL SCLEROSIS


Main researcher: JUŠIĆ, ANICA (19602)


Assistants
Type of research: applied
Duration from: 01/01/91. to 01/01/94.
Papers on project (total): 42
Papers on project quoted in Current Contents: 2
Institution name: Medicinski fakultet, Zagreb (108)
Communication
Phone: 385 (0)
Phone: 385 (0) 01 45 66 777
Summary: Amyotrophic lateral sclerosis is the illness of main interest in the world research. The basic problem is to separate the ALS like syndromes, the ALS variations with other signs of CNS involvement and hereditary forms. The idiopatic form of unknown origin is also subject of this research. The therapy with BPC (gastric juice peptide), Sandimun and Medrol pulse therapy was applied in idiopatic cases with only transient improvement. The research on improvement of quality of life in ALS terminal stages resulted with organisation of the First croatian symposium Hospice / palliative care, The death is part of the life. It will be held on 6th and 7th of May 1994. The carnitin deficiency research, specific cerebrospinal fluid analysis and sympathetic fibres electrophysiological analysis, inspite of its importance did not proceed because of technical handicaps. In the project subgroup the secondary muscle potentials (H and F) were very successfully analysed in healthy adults, infants and children. It is the first study of the kind although there was no possibility to evaluate correctly the results in patients if the issues in normals are unknown. The systematic analysis proved also that motoneurons with different function differ also electrophysiologically - the original finding of high significance. The pattern of H and F features distribution in infants and children is the same as in adults and fairly constant. The contractures in neuromuscular diseases, were analysed from diagnostic - ethiopathogenetic point of view. For the first time in Croatia, orthopedic treatment of contracture, the operation accor. Rideau and antepositon of posterior tibial muscle were performed. In some cases the functional improvement was significant. Differential diagnostic research in childrens neuromuscular diseases proceeded with many positive results.

Keywords: ALS syndroms and variants, Hereditary ALS, ALS therapy, Hospice / palliative care, H and F potentials, contractures, neuromuscular diseases of infants and children

Research goals: The goal of the main part of the project was the separation of motor neurone diseases with known origin from the essential or idiopatic ALS of unknown ethiopathogenesis. The second one was the subject of special attention. One of the project's goals was the investigation of possibilities to improve the quality of the terminal part of the life of ALS patients. The project leader visited St.Christopher's Hospice in London in which hundereds of ALS patients terminated their lives. She got included in their Information center and is receiving all the time, free of charge, the latest news in the field. Due to this collaboration and application of ideas and methods of the teaching and research hospice, the project leader formed the programme for the first croatian symposium organizedby Senat of Croatian Academy of Medical Sciences (the project leader is the Senats secretary) and three other association. The subproject of secondary H and F potentials was especially fruit full. The goal was systematical elaboration of normal H and F potentials features distribution, so thatdeviations in illness could be properly evaluated (although necessary notmet yet in the world literature. It resulted first in two doctor thesis and one magisterium pointing to quite new method of peripheral motoneurons differentiation. The differences recordedin adults were recorded in infants and children too. It will be very interesting to folow up sistematically how these differences correlate with histochemistry and liability to different neuromuscular pathology, especially different stages of motor neuron disease. The contracture problem in neuromuscular disease is oftena great one. The diagnostic criteria and indications for in theframe of this project introduced new contracture operation were successfully elaborated. Some specific feature in childrens neuromuscular diseases were elaborated also (Cardiomyopathies, electrophysiological features, enzymes studies and oth.)

COOPERATION - INSTITUTIONS


  1. Name of institution: Gradska bolnica "Sestre milosrdnice", klinika za rehabilitaciju, Elektromiografski laboratorij
    Type of institution: International organization
    Type of cooperation: Joint project
    City: 10000 - Zagreb, Croatia

  2. Name of institution: Medicinski fakultet, Klinički bolnički centar Rebro, Zavod za kliničku biokemiju
    Type of institution: International organization
    Type of cooperation: Joint project
    City: 10000 - Zagreb, Croatia
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Last update: 10/09/95
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