Clinical Neuroscience

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

PÉNTEK Márta1,2, HERCZEGFALVI Ágnes3, MOLNÁR Mária Judit4, SZŐNYI László Pál5, KOSZTOLÁNYI György6, PFLIEGLER György7, MELEGH Béla6, BONCZ Imre8, BRODSZKY Valentin1, BAJI Petra1, SZEGEDI Márta9, POGÁNY Gábor10, GULÁCSI László1

MAY 30, 2016

Clinical Neuroscience - 2016;69(05-06)

DOI: https://doi.org/10.18071/isz.69.0183

[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.]

AFFILIATIONS

  1. Budapesti Corvinus Egyetem, Egészségügyi Közgazdaságtan Tanszék, Budapest
  2. Pest Megyei Flór Ferenc Kórház, Reumatológiai és Fizioterápiás Osztály, Kistarcsa 3Semmelweis Egyetem, Általános Orvostudományi Kar, II. Sz. Gyermekgyógyászati Klinika, Budapest
  3. Semmelweis Egyetem, Általános Orvostudományi Kar, II. Sz. Gyermekgyógyászati Klinika, Budapest
  4. Semmelweis Egyetem, Genomikai Medicina és Ritka Betegségek Intézete, Budapest
  5. Semmelweis Egyetem, Általános Orvostudományi Kar, I. Sz. Gyermekgyógyászati Klinika, Budapest
  6. Pécsi Tudományegyetem Klinikai Központ, Általános Orvostudományi Kar, Orvosi Genetikai Intézet, Pécs
  7. Debreceni Egyetem Klinikai Központ, Belgyógyászati Intézet, Ritka Betegségek Tanszék, Debrecen
  8. Pécsi Tudományegyetem, Egészségtudományi Kar, Egészségbiztosítási Intézet, Pécs
  9. Emberi Erôforrások Minisztériuma Egészségpolitikai Fôosztálya, Budapest
  10. Ritka és Veleszületett Rendellenességgel élôk Országos Szövetsége, Budapest

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Clinical Neuroscience

[The changes in quality of life after instrumented surgical fusion of degenerative spondylolisthesis]

SÁRÓ Enikő, MISIK Ferenc, BANCZEROWSKI Péter

[Objective - There is no internationally accepted guideline for treatment of spondylolisthesis in the literature, otherwise this degenerative disease has great social and economical impact. There is no hungarian study examining the efficacy of instrumented fusion procedure in surgical treatment of spondylolisthesis. In current study we examined the effectiveness of fusion technique focusing on the impact of quality of life. Methods - Between 1st January, 2011 and 30th June, 2012 we examined a group of patients - who were operated on by instrumented fusion technique because of spondylolisthesis -, in the National Institute of Clinical Neurosciences, using the Oswestry Low Back Disability Questionnaire. All patients were treated after ineffective conservative treatment. The question was wheather how has changed the patients’ quality of life after the operation. Paired-sample t-test was used in this study. Results - Eighty-eight of the 97 examined patients reported different levels of impovement in the postoperative period, two patient’s condition has not changed in spite of the surgery, seven patient’s condition showed progression in average one year after the surgery. The pain improved most significantly (55.5%) (p<0.0001). Using the 16 point borderline according to the Questionnaire (over moderate disability), significant improvement was detected in 50 patients (51.5%). Succesful surgical result - according to the quality of life - was seen in 77.41% of male and in 50.98% of female patients. According to the different age groups, 72.72% of the younger (before retirement), and 53.06% of the retired patients belonged to this group. At least 15 point improvement was detected 35.05% of the patients, the overall improvement was 10.5 point. Discussion - Our results proved effectiveness of instrumented surgical fusion procedure in the treatment of degenerative spondylolisthesis. According to our results the younger male population with significant symptoms is the group, where improvement in quality of life is more pronaunced after the surgical procedure. Conclusion - The instrumented fusion surgical technique provides successful clinical and surgical outcome in patients with degenerative spondylolisthesis. It could improve the quality of life. Althought multicentre follow-up studies are needed to determine the exact indication and optimal therapy.]

Clinical Neuroscience

[Experiences of hypothalamic hamartoma surgeries]

NOVÁK László, KISS Máté Tamás, KLEKNER Álmos, NAGY Andrea, FEDORCSÁK Imre, BOGNÁR László

[Background and purpose - Hypothalamic hamartomas are focal, benign congenital malformations that frequently associated with gelastic seizures. Behavioural disturbances, cognitive decline and the appearance of precocious puberty can also be observed. The most effective way to relieve the symptoms is the surgical disconnection between the hamartoma and the hypothalamus. In our study, we retrospectively analyzed the surgical indications and effectiveness of each approach. Methods - Between 1996 and 2014 we operated on 10 hypothalamic hamartomas. Endoscopic assisted resection was performed in three patients. Six patients underwent direct microsurgical resection in various approaches and one patient was treated with Gamma Knife. Results - We achieved significant decrease in the number of seizures in every patient presenting with various seizure types. The surgical resection was effective in the arresting of the puberty praecox as well. However the surgery of these lesions at their special location holds the danger of the appearance of new endocrinological symptoms. According to our observations the operation on hamartoma less effectively ameliorates the psychiatric symptoms than the others. Conclusion - The surgical treatment is effective in the reduction of the initial symptoms and we had no mortality. According to our analysis therapeutic success is anticipated but we couldn’t archive total symptomatic relief in every case. The first approach to these lesions should be the surgery which type must be tailored to each patient.]

Clinical Neuroscience

[New opportunities in neuro-rehabilitation: Robot mediated therapy in conditons post central nervous system impairments]

FAZEKAS Gábor, TAVASZI Ibolya, TÓTH András

[Decreasing the often-seen multiple disabilities as a consequence of central nervous system impairments requires broadening of the tools of rehabilitation. A promising opportunity for this purpose is the application of physiotherapy robots. The development of such devices goes back a quarter of century. Nowadays several robots are commercially available both for supporting upper and lower limb therapy. The aim is never to replace the therapists, but rather to support and supplement their work. It is worthwhile applying these devices for goal-oriented exercises in high repetition, which one physically fatiguing for the therapist or for the correction of functional movement by various strategies. Robot mediated therapy is also useful for motivation of the patient and making the rehabilitation programme more versatile. Robots can be used for assessment of the neuromotor status as well. Several clinical studies have been executed in this field, all over the world. Meta-analyses based on randomized, controlled trials show that supplementing the traditional physiotherapy with a robot-mediated component presents advantage for the patients. Further studies are necessary to clarify which modality and intensity of the exercises, in which group of patients, in which stage lead to the expected outcome.]

Clinical Neuroscience

[Analysis of mental arithmetic task by the “minimum spanning tree” method]

BOHA Roland, TÓTH Brigitta, KARDOS Zsófia, FILE Bálint, GAÁL Zsófia Anna, MOLNÁR Márk

[Goals - In the present study basic arithmetic induced rearrangements in functional connections of the brain were investigated by using graph theoretical analysis what becomes increasingly important both in theoretical neuroscience and also in clinical investigations. Research questions - During mental arithmetic operations (working) memory plays an important role, but there are only a few studies in which an attempt was made to separate this effect from the process of arithmetic operations themselves. The goal of our study was to separate the neural networks involved in cognitive functions. Methods - As an attempt to clarify this issue the graph-theoretical “minimal spanning tree” method was used for the analysis of EEG recorded during task performance. The effects of passive viewing, number recognition and mental arithmetic on PLI based minimal spanning trees (MST) were investigated on the EEG in young adults (adding task: 17 subjects; passive viewing and number recognition: 16 subjects) in the θ (4-8 Hz) frequency band. Results - Occipital task relevant synchronization was found by using the different methods, probably related to the effect of visual stimulation. With respect to diameter, eccentricity and fraction of leafs different task-related changes were found. Discussion - It was shown that the task related changes of various graph indices are capable to identify networks behind the various relevant dominant functions. Thus the “minimal spanning tree” method is suitable for the analysis of the reorganization of the brain with respect to cognitive functions.]

Clinical Neuroscience

Comparison TachoComb with SurgiWrap, Surgicel and Lyodura in epidural fibrosis: An experimental rat model

HUSEYIN Karasu, IŞIL Güzel

Objectives - We designed this study to evaluate the effect of TachoComb and to compare it with SurgiWrap, Surgicel, and Lyodura in an experimental rat model for epidural fibrosis (EF). Methods - This prospective and experimental rat model study was performed at Dicle University, School of Medicine, Department of Neurosurgery, in Diyarbakır, Turkey, between January 2005 and June 2005, using 50 Sprague Dawley rats (30 female and 20 male) with a mean weight of 290 g. The rats were divided into five groups: TachoComb, Surgicel, SurgiWrap, Lyodura, and control. Results - Eight weeks after laminectomy, the rats were killed and EF was evaluated. EF was statistically different between the groups. The lowest EF score was in the TachoComb group (F=6.915, p<0.001). Conclusions - According to this study, TachoComb was found to be a more effective agent in decreasing EF than Surgicel, SurgiWrap, or Lyodura.

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