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

[Selective dorsal rhizotomy in the treatment of spasticity - Hungarian experiences ]

FEKETE Gábor, NOVÁK László, VEKERDY-NAGY Zsuzsanna, BOGNÁR László

[Objective - We summarize our initial experiences with selective dorsal rhizotomy that we introduced in Hungary to manage the spasticity of patients with cerebral palsy. Patients and methods - Thirty spastic patients were enrolled in our study. All of the patients were operated between July 2014 and June 2015. We performed selective dorsal rhizotomy from a single-level approach at the level of conus medullaris in all cases, with electrophysiological control. Results - We could perform the surgery in all cases safely. Adverse events related either to damage of neurological structures, or to surgery did not occur. The planned rehabilitation courses before and after the operations are in progress in all cases. Conclusion - Selective dorsal rhizotomy is an irreversible surgical intervention to treat spasticity. With sufficient experience and electrophysiological control it can be performed safely, and the early results are promising. ]

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

Paraoxonáz-1-aktivitás és fenotípusos megjelenése sclerosis multiplexben

KURTULUS Fatma, YAMAN Aylin, ELLIDAG Yasar Hamit, EREN Esin, GÖMCELI Bicer Yasemin, YILMAZ Necat

Introduction - Human serum paraoxonase (PON1) and arylesterase (ARE) are lipophilic antioxidant enzymes. PON-1 serum activity diverges in individuals and populations, which might be due to polymorphisms in the PON-1 gene. The PON1 activity phenotyping method, based on the ratio of the stimulated PON activity and the ARE activity, could determine the low-activity homozygotes (QQ), intermediate activity heterozygotes (QR), and high-activity homozygotes (RR) regardless of the genotype. The aim of the present study was to determine the PON1 phenotype distribution and enzymatic activity of PON1 and ARE in multiple sclerosis (MS) patients. Materials and methods - Thirty-four relapsing remitting MS (RRMS) patients (22 females and 12 males; median age 42 (range 20-55) years) in the remission phase and thirty-four age-sex matched healthy controls (19 females and 15 males; median age 37 (21-60) years) were included in this study. All patients had clinically definite MS according to McDonald’s criteria. Results - Serum PON1 and ARE enzyme activities, as well as salt-stimulated PON1, were not significantly different between the patient and control groups. Phenotype distributions were as follows: QQ 58.8%, QR 38.2%, and RR 3% in MS patients (n=34); QQ 44.1%, QR 50%, and RR 5.9% in the control group (n=34). QQ (low activity) phenotypic distribution was more common in MS patients than controls, but this difference was not significant (p=0.14). Conclusions - Our results did not reveal meaningful relationships between PON1 activity or PON1 phenotypes and MS. More studies in larger samples and in all phases of the disease are needed in the future.

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

[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.]

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[Successful radiological diagnostics in Bouveret’s syndrome]

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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.

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[About the care of patients with hyperuricaemia and gout]

[This consensus document is intended to provide guidance for the effective and efficient treatment of asymptomatic individuals with high uric acid levels and gout patients.]

Clinical Neuroscience

[What happens to vertiginous population after emission from the Emergency Department?]

MAIHOUB Stefani, MOLNÁR András, CSIKÓS András, KANIZSAI Péter, TAMÁS László, SZIRMAI Ágnes

[Background – Dizziness is one of the most frequent complaints when a patient is searching for medical care and resolution. This can be a problematic presentation in the emergency department, both from a diagnostic and a management standpoint. Purpose – The aim of our study is to clarify what happens to patients after leaving the emergency department. Methods – 879 patients were examined at the Semmel­weis University Emergency Department with vertigo and dizziness. We sent a questionnaire to these patients and we had 308 completed papers back (110 male, 198 female patients, mean age 61.8 ± 12.31 SD), which we further analyzed. Results – Based on the emergency department diagnosis we had the following results: central vestibular lesion (n = 71), dizziness or giddiness (n = 64) and BPPV (n = 51) were among the most frequent diagnosis. Clarification of the final post-examination diagnosis took several days (28.8%), and weeks (24.2%). It was also noticed that 24.02% of this population never received a proper diagnosis. Among the population only 80 patients (25.8%) got proper diagnosis of their complaints, which was supported by qualitative statistical analysis (Cohen Kappa test) result (κ = 0.560). Discussion – The correlation between our emergency department diagnosis and final diagnosis given to patients is low, a phenomenon that is also observable in other countries. Therefore, patient follow-up is an important issue, including the importance of neurotology and possibly neurological examination. Conclusion – Emergency diagnosis of vertigo is a great challenge, but despite of difficulties the targeted and quick case history and exact examination can evaluate the central or peripheral cause of the balance disorder. Therefore, to prevent declination of the quality of life the importance of further investigation is high.]