Clinical Neuroscience

[Surgery of the Pediatric Spine]

VERES Róbert, LIPÓTH László

JULY 22, 2009

Clinical Neuroscience - 2009;62(07-08)

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Clinical Neuroscience

[Current medical aspects of pantethine (in English language)]

HORVÁTH Zoltán, VÉCSEI László

[Pantethine, the stable disulfide form of pantetheine, is the major precursor of coenzyme A, which plays a central role in the metabolism of lipids and carbohydrates. Coenzyme A is a cofactor in over 70 enzymatic pathways, including fatty acid oxidation, carbohydrate metabolism, pyruvate degradation, amino acid catabolism, haem synthesis, acetylcholine synthesis, phase II detoxification, acetylation, etc. Pantethine has beneficial effects in vascular disease, it able to decrease the hyperlipidaemia, moderate the platelet function and prevent the lipid-peroxidation. Moreover its neuro-endocrinological regulating role, its good influence on cataract and cystinosis are also proved. This molecule is a well-tolerated therapeutic agent; the frequency of its side-effect is very low and mild. Based on these preclinical and clinical data, it could be recommended using this compound as adjuvant therapy.]

Clinical Neuroscience

[Pompe’s disease - Part I - Pathogenesis and clinical features]

ILLÉS Zsolt, TRAUNINGER Anita

[Pompe’s disease is an ultra-orphan disease caused by the deficiency of lysosomal alpha-glucosidase. At present, it is the only inherited muscle disorder, which can be treated by replacement of the enzyme. According to the natural course, early infantile and late childhood-juvenile-adult cases are known. Respiratory insufficiency, cardiomyopathy, and muscle hypotonia are cardinal symptoms/signs in infantile Pompe’s disease, while cardiomyopathy is absent in adult-onset cases. CK levels are always elevated in the sera of infantile patients. Hip-girdle dystrophy and orthopnoe should alert suspicion in adult patients. Diagnosis is established by decreased activity of the enzyme or mutational analysis. Muscle biopsy can be misleading in adult cases due to absence of glycogen in the examined specimen. In this review, we also discuss our experiences obtained by the treatment of three patients.]

Clinical Neuroscience

[Acute disseminated encephalomyelitis in childhood]

LIPTAI Zoltán, ÚJHELYI Enikő, MIHÁLY Ilona, RUDAS Gábor, BARSI Péter

[Background and purpose - Acute disseminated encephalomyelitis is a rare inflammatory demyelinating disorder often preceded by infection or vaccination. The purpose of the study was the systematic analysis of clinical, radiological and microbiological profiles of children treated at Szent László Hospital, and the comparison of findings with literature data. Methods - Demographic, infectological, clinical, radiological, laboratory and virological data of patients treated and followed-up between 1-Jan-1998 and 30-June-2008 were reviewed and analysed. Results - 19 children met diagnostic criteria. Their mean age was 6.8 years. A prodromal illness - mostly febrile viral infection, upper respiratory infection or chickenpox - preceded neurological symptoms in 17 patients. All had polysymptomatic encephalopathy, 2 children had spinal symptoms. The cerebrospinal fluid was abnormal in all but one. A viral etiology was definite in 7 and probable in 8 cases. MRI disclosed white matter changes in 18, cortical and deep gray matter in 16, cerebellar in 6, brain stem in 14 and spinal cord changes in 2 cases. Repeat MRI performed mean 4 months later showed complete resolution in 6 and partial resolution in 11 patients. 13 patients received high-dose methylprednisolone, 2 of whom were also treated with plasma exchange and 1 with immunoglobulin. 9 children required mechanical ventilation. 2 patients died, 10 recovered without and 7 with sequelae. 2 patients developed further demyelinating events: multiple sclerosis and multiphasic disseminated encephalomyelitis, respectively. Conclusion - Clinical, radiological and follow-up results were similar to those published in literature however, triggering viruses were identified in a larger proportion of cases.]

Clinical Neuroscience

[The role of neurolytic obturator nerve block to relieve pain due to cancer and osteoarthritis (in English language)]

EMBEY-ISZTIN Dezső

[Neurolytic obturator nerve block have been performed successfully to relieve pain due to osteolytic metastases of pelvic bone since 1981 in our Pain Clinic. The analgesic effect of one block lasts from three to four months and can be repeated as required. Following the block the patient can go home one hour later. In 2008 we started to perform the neurolytic obturator nerve block to relieve pain due to degenerative osteoarthritis of hip joint. It is a good choice for those patients, who are not enough fit to be operated, or during the waiting time of hip replacement surgery.]

Clinical Neuroscience

[Removal of multiple thoracic dumbbell tumours through combined hemi-semi laminectomy and minimal invasive paraspinal approach]

PAPP Zoltán

[One third of the primary spinal tumours are neurinomas. These tumours most often located intraduralyextramedullary, but 15-25 percent of the cases, the tumour extends to the paraspinal region through the neuroforamina. Spinal manifestation of Schwann-cell tumours, usually neurofibromas, can be seen in neurofibromatosis. 31 years old female patient, suffering from NF1 was operated on with a Th 3-6 and Th 10-11 dumbbell tumours. At the first step the tumour mass, located in the spinal canal, was resected through a partial hemilaminectomy. At the second operation the extraspinal tumour was resected through a minimal invasive paraspinal approach. The author present the combined use of minimal invasive spine surgical technics and discuss the benefits and disadvantages of these approaches, compared to the standard surgical procedures.]

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Clinical Neuroscience

Neuroscience highlights: Main cell types underlying memory and spatial navigation

KRABOTH Zoltán, KÁLMÁN Bernadette

Interest in the hippocampal formation and its role in navigation and memory arose in the second part of the 20th century, at least in part due to the curious case of Henry G. Molaison, who underwent brain surgery for intractable epilepsy. The temporal association observed between the removal of his entorhinal cortex along with a significant part of hippocampus and the developing severe memory deficit inspired scientists to focus on these regions. The subsequent discovery of the so-called place cells in the hippocampus launched the description of many other functional cell types and neuronal networks throughout the Papez-circuit that has a key role in memory processes and spatial information coding (speed, head direction, border, grid, object-vector etc). Each of these cell types has its own unique characteristics, and together they form the so-called “Brain GPS”. The aim of this short survey is to highlight for practicing neurologists the types of cells and neuronal networks that represent the anatomical substrates and physiological correlates of pathological entities affecting the limbic system, especially in the temporal lobe. For that purpose, we survey early discoveries along with the most relevant neuroscience observations from the recent literature. By this brief survey, we highlight main cell types in the hippocampal formation, and describe their roles in spatial navigation and memory processes. In recent decades, an array of new and functionally unique neuron types has been recognized in the hippocampal formation, but likely more remain to be discovered. For a better understanding of the heterogeneous presentations of neurological disorders affecting this anatomical region, insights into the constantly evolving neuroscience behind may be helpful. The public health consequences of diseases that affect memory and spatial navigation are high, and grow as the population ages, prompting scientist to focus on further exploring this brain region.

Clinical Neuroscience

Autonomic nervous system may be affected after carpal tunnel syndrome surgery: A possible mechanism for persistence of symptoms after surgery

ONDER Burcu, KELES Yavuz Betul

After carpal tunnel surgery, some patients report complaints such as edema, pain, and numbness. Purpose – The aim of this study was to evaluate autonomic nervous system function in patients with a history of carpal tunnel surgery using sympathetic skin response (SSR). Thirty three patients (55 ±10 years old) with a history of unilateral operation for carpal tunnel syndrome were included in the study. The SSR test was performed for both hands. Both upper extremities median and ulnar nerve conduction results were recorded. A reduced amplitude (p=0.006) and delayed latency (p<0.0001) were detected in the SSR test on the operated side compared to contralateral side. There was no correlation between SSR and carpal tunnel syndrome severity. Although complex regional pain syndrome does not develop in patients after carpal tunnel surgery, some of the complaints may be caused by effects on the autonomic nervous system.

Clinical Neuroscience

Cyanocobalamin and cholecalciferol synergistically improve functional and histopathological nerve healing in experimental rat model

ALBAY Cem, ADANIR Oktay, AKKALP Kahraman Asli, DOGAN Burcu Vasfiye, GULAEC Akif Mehmet, BEYTEMUR Ozan

Introduction - Peripheral nerve injury (PNI) is a frequent problem among young adults. Hopefully, regeneration can occur in PNI unlike central nervous system. If nerve cut is complete, gold standard treatment is surgery, but incomplete cuts have been tried to be treated by medicines. The aim of the study was to evaluate and compare clinical and histopathological outcomes of independent treatment of each of Vitamin B12 (B12) and Vitamin D3 (D3) and their combination on sciatic nerve injury in an experimental rat model. Materials and methods - Experimental animal study was performed after the approval of BEH Ethics Committee No. 2015/10. 32 rats were grouped into four (n=8) according to treatment procedures, such as Group 1 (controls with no treatment), Group 2 (intraperitoneal 1 mg/kg/day B12), Group 3 (oral 3500 IU/kg/week D3), Group 4 (intraperitoneal 1 mg/kg/day B12+ oral 3500 IU/kg/week D3). Sciatic Functional Index (SFI) and histopathological analysis were performed. Results - SFIs of Group 2, 3, 4 were statistically significantly higher than controls. Group 2 and 3 were statistically not different, however Group 4 was statistically significantly higher than others according to SFI. Axonal degeneration (AD) in all treatment groups were statistically significantly lower than in Group 1. AD in Group 4 was significantly lower than in Group 2 and 3; there was no significant difference between Group 2 and 3. There was no significant difference between Group 1,2 and 3 in Axonolysis (A). But A of Group 4 was significantly very much lower than all others. Oedema- inflammation (OE-I) in all treatment groups were significantly lower than in Group 1; there was no significant difference between Group 2 and group 4. OE-I in Group 2 and 4 were significantly lower than in Group 3. There were no significant differences between Group 1, 2 and 3 in damage level scores; score of Group 4 was significantly lower than of Group 1. Conclusions - B12 and D3 were found effective with no statistically significant difference. But combined use of B12 and D3 improve nerve healing synergistically. We recommend combined use of B12 and D3 after PNI as soon as possible.

Clinical Neuroscience

[MR imaging of acute disseminated encephalomyelitis and multiple sclerosis in children. A review (in English language)]

PATAY Zoltán

[Inflammatory diseases of the central nervous system (CNS) are relatively rare in children, but their relevance to public health is considerable due to frequent and significant long term morbidity and even mortality. As in adults, acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) and their variants are the most common entities in this group of pathologies in the pediatric patient population. Recent efforts have focused on establishing standardized diagnostic criteria schemes to facilitate the diagnosis and differential diagnosis of these diseases, however especially with multiple sclerosis those have not been fully validated yet for disease occurring in children. In recent decades the role of MRI has been constantly increasing in the diagnostic work-up of suspected inflammatory diseases of the CNS as well as in the follow-up of patients with confirmed disease. Currently, MRI is the first-line diagnostic imaging modality in ADEM and MS and is fully integrated in the most widely used diagnostic criteria schemes, but it has a key role in clinical therapeutic research trials as well. This paper provides an update on the current concepts and strategies of MRI in inflammatory diseases of the CNS, as well as a review of the imaging semiology of the various disease entities and variants with emphasis on clinical and imaging particularities relevant to the pediatric patient population.]

Lege Artis Medicinae

[OVARIAN CHORIOCARCINOMA - A MOLECULAR PATHOLOGIC APPROACH TO DIFFERENTIAL DIAGNOSIS]

ERÉNYI Éva, SZIGETVÁRI Iván, ANDRIKOVICS Hajnalka, TORDAI Attila, URBÁN Márta, SIMON Károly

[INTRODUCTION - Gestational and non-gestational choriocarcinoma, the two types of choriocarcinoma with distinct aetiologies, cannot be differentiated by conventional histological or immunohistochemical methods. In certain cases the verification of the origin of the tumour by molecular biological techniques is essential for prognostic and therapeutic reasons. CASE REPORT - A 22-year-old woman presenting with acute abdominal pain was examined and operated on. During surgery a tumour was found in the right ovary. “Pure” choriocarcinoma was histologically diagnosed and gestational origin was supposed based on the distinct clinical features and histological picture. This was subsequently confirmed by molecular genetic testing. Parallel VNTR (variable number of tandem repeats) analysis by PCR of DNA from the tumour tissue and the patient's peripheral blood was performed. The pattern showed the presence of an allele presumed to be of paternal origin. (Blood sample from the father was not available.) One regimen of Vepesid, Cisplatina and Bleomycin was applied, and more than three years after the diagnosis the patient is free of disease. CONCLUSION - Multidisciplinary cooperation led to an accurate diagnosis and successful treatment. Non-radical surgery with the preservation of fertility followed by proper early chemotherapy provided excellent results.]