Clinical Neuroscience

[Closure of nasocranial fistulas with “bath-plug” technique and multilayer reconstruction]

PISKI Zalán, BÜKI András, NEPP Nelli, BURIÁN András, RÉVÉSZ Péter, GERLINGER Imre

MAY 30, 2016

Clinical Neuroscience - 2016;69(05-06)

DOI: https://doi.org/10.18071/isz.69.0211

[Background and purpose - In case of dehiscenses developing on the anterior scull base, complete closure resulting in the cessation of the communication between the nasal cavity and the intracranial space is mandatory as soon as possible, in order to prevent serious complications. With the development of the endoscopic techniques, the endonasal management for the reconstruction has become available in recent decades. Methods - We aim to present the reconstruction techniques applied in our department in the cases of two patients recently operated at our institute. The choice of methods primarily depends on the size and the localization of the defect. Dehiscenses under 5 mm of diameter can be closed with the so called “bath-plug” technique, while bigger defects, where the required closure of the plug is not possible, can be solved with multilayer reconstruction. We use autogenous fascia, fat and muco-periosteum in both cases. Results - Our patient, who underwent the aforementioned “bath-plug” procedure, could be discharged after a few days of uneventful postoperative period. During a tenmonth follow- up period new fistula formation was not observed. In the case of a patient who underwent multilayer reconstruction, meningitis occurred postoperatively, which was resolved after antibiotic therapy. During a 17- month follow- up period recurrent liquorrhoea did not occur. Conclusion - With suitable technical background and appropriate endoscopic skills the surgeries of the anterior skull base cerebrospinal fluid fistulas can be performed efficiently and with low complication rate. These are minimally invasive procedures accompanied by less surgical trauma, morbidity and shorter hospitalization, hence these techniques are considered to be cost-effective and well- tolerated for the patients.]

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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.]

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[Successful radiological diagnostics in Bouveret’s syndrome]

KISS Katalin, FARKAS Szabolcs, LUKOVICH Péter, MAGYAR Péter, MESTER Ádám, MAKÓ Ernő

[INTRODUCTION - Bouveret’s syndrome I is a rare clinical entity, a special form of gallstone ileus. Based on a case study the authors describe the clinical presentation, the complications and diagnostic work up of the Bouveret’s syndrome I. CASE REPORT - A 75-year-old female patient with repeated vomiting and haematemesis was examined. Known gallstones and obstructive jaundice was noted in the case history. Urgent gastroscopy was performed at admission, which proved haemorrhagic esophagitis as the cause of the haematemesis. A gallstone was found by endoscopy distal to the pyloric region obstructing the bowel lumen. Radiological examinations proved the presence of the stone exactly at the localization that was given. Surgery confirmed the diagnosis. CONCLUSION - Bouveret’s syndrome I should be considered in patients with repeated and long lasting vomiting and bile stone in the case history. Conventional X-ray may be sufficient to establish the diagnosis, however further imaging studies are needed to clarify exact anatomical situation and potential complications of the disease.]

Clinical Neuroscience

Cases of inborn errors of metabolism diagnosed in children with autism

CAKAR Emel Nafiye, YILMAZBAS Pınar

Autism spectrum disorder is a neurodevelopmental disorder with a heterogeneous presentation, the etiology of which is not clearly elucidated. In recent years, comorbidity has become more evident with the increase in the frequency of autism and diagnostic possibilities of inborn errors of metabolism. One hundred and seventy-nine patients with diagnosis of autism spectrum disorder who presented to the Pediatric Metabolism outpatient clinic between 01/September/2018-29/February/2020 constituted the study population. The personal information, routine and specific metabolic tests of the patients were analyzed retrospectively. Out of the 3261 patients who presented to our outpatient clinic, 179 (5.48%) were diagnosed with autism spectrum disorder and were included in the study. As a result of specific metabolic examinations performed, 6 (3.3%) patients were diagnosed with inborn errors of metabolism. Two of our patients were diagnosed with classical phenylketonuria, two with classical homocystinuria, one with mucopolysaccharidosis type 3D (Sanfilippo syndrome) and one with 3-methylchrotonyl Co-A carboxylase deficiency. Inborn errors of metabolism may rarely present with autism spectrum disorder symptoms. Careful evaluation of the history, physical examination and additional findings in patients diagnosed with autism spectrum disorder will guide the clinician in the decision-making process and chose the appropriate specific metabolic investigation. An underlying inborn errors of metabolism may be a treatable cause of autism.

Clinical Neuroscience

Late simultaneous carcinomatous meningitis, temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting with mono-symptomatic vertigo – a clinico-pathological case reporT

JARABIN András János, KLIVÉNYI Péter, TISZLAVICZ László, MOLNÁR Anna Fiona, GION Katalin, FÖLDESI Imre, KISS Geza Jozsef, ROVÓ László, BELLA Zsolt

Although vertigo is one of the most common complaints, intracranial malignant tumors rarely cause sudden asymmetry between the tone of the vestibular peripheries masquerading as a peripheral-like disorder. Here we report a case of simultaneous temporal bone infiltrating macro-metastasis and disseminated multi-organ micro-metastases presenting as acute unilateral vestibular syndrome, due to the reawakening of a primary gastric signet ring cell carcinoma. Purpose – Our objective was to identify those pathophysiological steps that may explain the complex process of tumor reawakening, dissemination. The possible causes of vestibular asymmetry were also traced. A 56-year-old male patient’s interdisciplinary medical data had been retrospectively analyzed. Original clinical and pathological results have been collected and thoroughly reevaluated, then new histological staining and immunohistochemistry methods have been added to the diagnostic pool. During the autopsy the cerebrum and cerebellum was edematous. The apex of the left petrous bone was infiltrated and destructed by a tumor mass of 2x2 cm in size. Histological reexamination of the original gastric resection specimen slides revealed focal submucosal tumorous infiltration with a vascular invasion. By immunohistochemistry mainly single infiltrating tumor cells were observed with Cytokeratin 7 and Vimentin positivity and partial loss of E-cadherin staining. The subsequent histological examination of necropsy tissue specimens confirmed the disseminated, multi-organ microscopic tumorous invasion. Discussion – It has been recently reported that the expression of Vimentin and the loss of E-cadherin is significantly associated with advanced stage, lymph node metastasis, vascular and neural invasion and undifferentiated type with p<0.05 significance. As our patient was middle aged and had no immune-deficiency, the promoting factor of the reawakening of the primary GC malignant disease after a 9-year-long period of dormancy remained undiscovered. The organ-specific tropism explained by the “seed and soil” theory was unexpected, due to rare occurrence of gastric cancer to metastasize in the meninges given that only a minority of these cells would be capable of crossing the blood brain barrier. Patients with past malignancies and new onset of neurological symptoms should alert the physician to central nervous system involvement, and the appropriate, targeted diagnostic and therapeutic work-up should be established immediately. Targeted staining with specific antibodies is recommended. Recent studies on cell lines indicate that metformin strongly inhibits epithelial-mesenchymal transition of gastric cancer cells. Therefore, further studies need to be performed on cases positive for epithelial-mesenchymal transition.

Clinical Neuroscience

Alexithymia is associated with cognitive impairment in patients with Parkinson’s disease

SENGUL Yildizhan, KOCAK Müge, CORAKCI Zeynep, SENGUL Serdar Hakan, USTUN Ismet

Cognitive dysfunction (CD) is a common non-motor symptom of Parkinson’s disease (PD). Alexithy­mia is a still poorly understood neuropsychiatric feature of PD. Cognitive impairment (especially visuospatial dysfunction and executive dysfunction) and alexithymia share com­mon pathology of neuroanatomical structures. We hypo­thesized that there must be a correlation between CD and alexithymia levels considering this relationship of neuroanatomy. Objective – The aim of this study was to evaluate the association between alexithymia and neurocognitive function in patients with PD. Thirty-five patients with PD were included in this study. The Toronto Alexithymia Scale–20 (TAS-20), Geriatric Depression Inventory (GDI) and a detailed neuropsychological evaluation were performed. Higher TAS-20 scores were negatively correlated with Wechsler Adult Intelligence Scale (WAIS) similarities test score (r =-0.71, p value 0.02), clock drawing test (CDT) scores (r=-0.72, p=0.02) and verbal fluency (VF) (r=-0.77, p<0.01). Difficulty identifying feelings subscale score was negatively correlated with CDT scores (r=-0.74, p=0.02), VF scores (r=-0.66, p=0.04), visual memory immediate recall (r=-0.74, p=0.01). VF scores were also correlated with difficulty describing feelings (DDF) scores (r=-0.66, p=0.04). There was a reverse relationship bet­ween WAIS similarities and DDF scores (r=-0.70, p=0.02), and externally oriented-thinking (r=-0.77,p<0.01). Executive function Z score was correlated with the mean TAS-20 score (r=-62, p=0.03) and DDF subscale score (r=-0.70, p=0.01) Alexithymia was found to be associated with poorer performance on visuospatial and executive function test results. We also found that alexithymia was significantly correlated with depressive symptoms. Presence of alexithymia should therefore warn the clinicians for co-existing CD.