Clinical Neuroscience

[Experiences of hypothalamic hamartoma surgeries]

NOVÁK László1, KISS Máté Tamás1, KLEKNER Álmos1, NAGY Andrea2, FEDORCSÁK Imre3, BOGNÁR László1

MAY 30, 2016

Clinical Neuroscience - 2016;69(05-06)

DOI: https://doi.org/10.18071/isz.69.E032

[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.]

AFFILIATIONS

  1. Debreceni Egyetem, Klinikai Központ, Idegsebészeti Klinika, Debrecen
  2. Országos Klinikai Idegtudományi Intézet, Budapest
  3. Debreceni Egyetem, Klinikai Központ, Gyermekgyógyászati Intézet, Debrecen

COMMENTS

0 comments

Further articles in this publication

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.]

Clinical Neuroscience

[Genetically determined diseases associated with pathological brain iron accumulation and neurodegeneration]

ÁCS Péter, MOLNÁR Mária Judit, KLIVÉNYI Péter, KÁLMÁN Bernadette

[The rare, genetically determined group of diseases characterized by pathological accumulation of iron in the central nervous system and progressive, typically movement disorder’s symptoms are called NBIA (neurodegeneration with brain iron accumulation). By the rapid development of molecular genetics, it has become apparent that different mutations in numerous genes can lead to pathological cerebral iron accumulation. Simultaneously, it has also been recognized that the age of onset, the symptoms and the prognosis of NBIA disorders are much more diverse than it was previously perceived. To our knowledge, a review article on the most recent clinical data of NBIA has not been published in Hungarian. In the first part of this publication, we survey the general clinical characteristics and the diagnostic algorithm of NBIA diseases and address some considerations for differential diagnostics. In the second part of this review, the particular NBIA disorders are presented in details. The purpose of this article is to provide a clinical overview that may be useful for neurologists, pediatricians and any other medical practitioners interested in this field.]

Clinical Neuroscience

The relationship between serum cholesterol and verbal memory may be influenced by body mass index (BMI) in young healthy women

DARNAI Gergely, PLÓZER Enikõ, ALTBACKER Anna, PERLAKI Gábor, ORSI Gergely, KŐSZEGI Tamás, NAGY Szilvia Anett, LUCZA Tivadar, KOVÁCS Norbert, JANSZKY József, CLEMENS Zsófia

The objective of this study was to examine the relationship between verbal memory and total cholesterol (TC) levels related to body mass index (BMI) in healthy young women. Verbal memory was assessed using the Rey Auditory-Verbal Learning Test (RAVLT) while total serum cholesterol was measured by enzymatic colorimetric test. In order to analyze the potential significance of BMI subjects were divided into three groups according to their calculated BMI percentile values. No significant correlation was found when assessing the group as a whole. However a remarkable pattern of correlation emerged when assessing the BMI groups separately: a close-to-significant positive correlation was found for total learning score and TC in the low BMI group, no correlation emerged in the medium BMI group while a strong inverse correlation was found in the high BMI group. These findings indicate that the relationship between verbal memory and serum TC level is also influenced by BMI.

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.

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.]

All articles in the issue

Related contents

Clinical Neuroscience

[Simultaneous occurrence of cavernous angioma and developental venous anomaly]

KUNCZ Ádám, VÖRÖS Erika, BODOSI Mihály

[The significance of the simultaneous occurrence of cavernous angioma and developmental venous anomaly, the diagnostic possibilities and the surgical treatment are discussed based upon three cases. Angiography and magnetic resonance imaging together are necessary to get an accurate diagnosis. According to the literature and to the fact that it is the cavernous angioma that mainly causes the symptoms, the cavernosus angioma should be resected, while the developmental venous anomaly should be spared.]

Lege Artis Medicinae

[STATE-OF-THE-ART DIAGNOSIS AND TREATMENT OF PITUITARY ADENOMAS SECRETING GROWTH HORMONE]

CZIRJÁK Sándor

[The history of the treatment of pituitary adenomas that cause acromegaly is as long as that of neurosurgery. While in the first half of the past century the aim of surgery was to save the patient's life, later the radical removal of the tumour was coupled with an effort to decrease complications, morbidity and mortality to the minimum. Today, beside all these, the complete sparing of the remaining hypophyseal substance and restoration of normal pituitary function are also important goals. The achievement of these goals is efficiently served by recent advances in microscopy, the minimally invasive methods of craniotomy, the availability of endoscopy in neurosurgery, three-dimensional computerguided neuronavigation, intraoperative colour Doppler sonography, as well as intraoperative real-time MRI. Recent developments in pharmacological research have created new promising conservative treatment modalities that supplement surgery, including somatostatin analogues and growth hormone receptor agonists. Also as supplementary treatment to surgery, occasionally replacing it, new radiosurgical methods, such as stereotaxic radiation, gamma knife, and heavy particle irradiation have gained grounds in neurosurgical practice.]

Clinical Neuroscience

[TEMPORAL LOBE EPILEPSY - STATE OF ART REVIEW]

HALÁSZ Péter, FOGARASI András

[The temporal lobe epilepsy (TLE) is the most important kind of partial epilepsy both from practical and research point of view, where studies brought many new results in the last years. This article is a state of art review with a special emphasis on medial temporal lobe epilepsy (MTLE). We show the clinical symptoms, EEG and neuroimaging signs, the psychiatric co-morbidities and psyhosocial consequences. Etiological factors, among them hippocampal sclerosis and hippocampal reorganisation is assessed in a more detailed form. The possibilities of pharmacological and surgical treatment are also shown, together with the brief outline of the Hungarian situation. TLE is presented as a model for the development of partial epilepsies.]

Hungarian Radiology

[Supracondylar process syndrome]

PÓTA Zsuzsanna, HUSZANYIK István, KAZAI Sándor, RÓDE László

[INTRODUCTION - Supracondylar process is a rare bone over-growth of atavistic origin. The entity shows several similarities with the tunnel syndromes. CASE REPORT - The case reports originate from the diagnosis and the treatment of two female patients. Both of them presented with elbow region pain, weakness in finger flexion and numbness. The clinical picture, the decrease in the speed of nerve conduction in the region of the supracondylar process and radiological evidence of the process over-growth made the proper diagnosis possible in both cases. Due to the failure of conservative therapy both patients underwent surgery. Careful surgical removal of the process and neurolysis resulted in cessation of symptoms. CONCLUSION - Use of atypical radiographic positioning and flouroscopy might be required to achieve a diagnosis. In patients who do not respond to physiotherapy and local steroid injections, surgical removal of the supracondylar process is indicated which prevents further arterial and nerve degeneration.]

Lege Artis Medicinae

[The role of sentinel lymph node biopsy in surgical oncology]

PÉLEY Gábor, KÖVES István, SINKOVICS István, FARKAS Emil, DUBECZ Sándor, KOVÁCS Tibor, KERESZTES Sándor, VÁMOSI-NAGY István

[Regional lymphadenectomy (or lymph node dissection) is the integral part of the surgical treatment for most solid malignant tumors. The purpose of this procedure is twofold: firstly, to achieve locoregional control of the disease and secondly, the staging of the disease. The subsequent complication and morbidity rate of regional lymphadenectomy can be significant: the tumor can disseminate after surgery in a number of patients conventionally staged as node negative (pN0). In the 90’s, a new and minimally invasive surgical regional staging method, the sentinel lymph node biopsy has been developed and introduced into clinical practice. Based on the theory of the sentinel lymph node concept, the lymphatic drainage of the tumor is mapped, and the first tumor-draining lymph node, the sentinel lymph node is localized and removed. The detailed histological examination of this lymph node determines the regional tumor status more sensitively than routinely used staging methods and is more cost effective. This technique may allow the use of selective surgical or radiation treatment of tumors draining to superficial lymph node basins. The history and theoretical background of sentinel lymph node biopsy are also reviewed with the feasibility, technique and role of this new method in different tumor locations discussed.]