Clinical Neuroscience

Association of anterior thoracic meningocele and azygos lobe of the lung

DENIZ Ersay Fatih1, SENAYLI Atilla2,3, BICAKCI Ünal2,4

JULY 30, 2016

Clinical Neuroscience - 2016;69(07-08)


Here we report an anterior thoracic meningocele case. Twoyears- old female patient was presented with kyphosis. Azygos lobe of the lung was also demonstrated during radiological studies. Posterolateral thoracotomy incision and extralpeural approach was performed for excision of the anterior meningocele to untether the cord. Although both anomalies are related to faulty embryogenesis and it is well known that faulty embryogenesis may also reveal coexisting abnormalities, we could not speculate a common mechanism for anterior thoracic meningocele and azygos lobe of the lung association.


  1. Faculty of Medicine, Department of Neurosurgery, Gaziosmanpasa University, Tokat, Turkey
  2. Faculty of Medicine, Department of Pediatric Surgery, Gaziosmanpasa University, Tokat, Turkey
  3. Ankara Yildirim Beyazit University, Department of Pediatric Surgery, Ankara, Turkey
  4. Faculty of Medicine, Department of Pediatric Surgery, Ondokuz Mayıs University, Samsun, Turkey



Further articles in this publication

Clinical Neuroscience

Burning mouth syndrome: Evaluation of clinical and laboratory findings


Background and purpose - Burning mouth syndrome is a chronic and persistent painful condition characterized by burning sensation in the oral mucosa. We investigated the etiological factors of patients presented with the history of burning in the mouth who admitted our outpatient clinics over the 8-years period and who had no underlying identifiable local factors. We also tried to determine their demographic and clinical characteristics. Our aim was to investigate the association between burning mouth and psychiatric disorders such as depression and anxiety, chronic diseases like diabetes mellitus (DM) and other laboratory studies in patients complaining of solely burning in the mouth. Methods - The study included patients with the history of burning in mouth who presented in our outpatient clinic between 2005 and 2012. They were evaluated by a neurologist, a psychiatrist, an internist, and a dentist. Complete blood counts, biochemical analysis and cranial magnetic resonance imaging (MRI) were performed for all patients. Results - A total of 26 (22 (84%) females, 4 (15%) males; mean age 55.9 years) patients were enrolled in this study. Five (19.2%) of the patients had depression, 2 (7.7%) had anxiety disorder, 2 (7.7%) had diabetes mellitus, 8 (30%) had B12 vitamin deficiency, 3 (11.5%) had decreased ferritin levels in blood, and 1 (3.8%) had folic acid deficiency. Cranial MRI of all patients were normal. Nine patients (34.6%) had no etiological causes. Conclusion - A multidisciplinary approach in the management of burning mouth and establishment of common criteria for the diagnosis would provide insight into the underlying pathophysiological mechanism.

Clinical Neuroscience

[Meningioma and pregnancy]


[Meningiomas are one of the most frequent primary intracranial tumours, representing one-third of all intracranialneoplasms. The vast majority of meningiomas are histologically benign, but recurrence and progression is quite frequent. They occur usually between the 6th and 7th decade, the female/male ratio is 3:2. Although rare in pregnancy, when occurring, they can cause serious, life-threatening complications due to rapid growth and unfavourable localisation. There are two dominant hypothesis explaining rapid growth in pregnancy: the role of hormonal effects and hemodynamic changes. Several studies tested these theories but none provided unequivocal answer probably because the pathomechanism is complex and multifactorial. We provide an overview of the pathomechanism of meningiomas in pregnancy with emphasis on data obtained by advanced neuropathological, molecular biological, bioinformatic, imaging and epidemiological methods. A better understanding of the processes leading to meningioma development and growth in pregnancy will help us to design personalized therapy and reduce morbidity and mortality.]

Clinical Neuroscience

[The role of zonisamide in the management of pediatric partial epilepsy]

ROSDY Beáta, KOLLÁR Katalin, MÓSER Judit, MELLÁR Mónika

[In our review we discuss the group of approved antiepileptic drugs for children in Hungary. We cite the results of the review conducted by the International League Against Epilepsy on antiepileptic drug efficacy and effectiveness as initial monotherapy for newly diagnosed epileptic seizures and syndromes in pediatric age group. 25% of pediatric epilepsy is therapy resistant, so we further need new drugs, which must be investigated according to the rules of the European Medicine Agency. The ethical dilemmas of childhood drug studies lead to the situation that the new antiepileptic drugs, approved as monotherapy in adult epilepsies, are in the majority just in add-on regimen tested in pediatric patients. As clinicians we appreciate open label extension safety studies. An old-new antiepileptic drug in Europe is zonisamide. Though it was approved for first line monotherapy in pediatric and adult patients with partial and generalised epilepsy in 1989 in Japan, the European Medicine Agency licensed its use as adjunctive therapy in children aged 6 years or older with partial seizures (with or without secondary generalisation) just in 2013. The results of the openlabel extension study appeared in 2014. The mean dose received was 7.5 mg/kg/day. During the open label phase 11% of the patients achieved seizure freedom and it was maintained throughout the study. The drug was generally well tolerated. The most frequently reported treatment-related adverse events were decreased weight (6%), decreased appetite (4%), and headache (2%). No new or unexpected side effects emerged. In conclusion oral zonisamide as adjunctive therapy in pediatric patients aged 6-17 years with partial seizures demonstrated an acceptable safety and tolerability profile and efficacy over a period at least 1 year. So it is a good treatment option in this population.]

Clinical Neuroscience

[Transthyretin familial amyloid polyneuropathy - three Hungarian cases with rare mutations (His88Arg and Phe33Leu)]

CSILLIK Anita, POZSONYI Zoltán, SOÓS Krisztina, BALOGH István, BODÓ Imre, ARÁNYI Zsuzsanna

[Introduction - Transthyretin familial amyloid polyneuropathy is a rare autosomal dominant progressive systemic disesase of adults caused by endoneural amyloid deposition due to point mutations of the transthyretin gene. It is the most severe form among hereditary polyneuropathies, being fatal within 10 years if left untreated. The disease is underdiagnosed, the late onset forms (above the age of 50) being probably more widespread than previously thought. Early diagnosis is essential as the early introduction of causal therapy (tafamidis) slows progression and prolongs survival. Patients - We report here three non-related Hungarian cases of transthyretin familial amyloid polyneuropathy with non- Val30Met mutations (His88Arg in two cases, Phe33Leu in one case). They were all characterized by late-onset, progressive, length-dependent, axonal, sensorimotor polyneuropathy and the simultaneous presentation of severe restrictive cardiomyopathy. In all three cases, clinical and electrophysiological signs of myopathy were also present, suggesting the involvement of skeletal muscles as well. In two cases, high resolution ultrasound of the peripheral nerves was also performed, which showed segmental structural alterations (change or loss of fascicular structure) and some increase of echogenicity of the interfascicular epineurium, without substantial enlargement of the nerves. Conclusion - In Hungary, mainly the rare, non-Val30Met mutation forms of transthyretin familial amyloid polyneuropathy are encountered, as in our cases. As opposed to the Val30Met forms, these mutations are characterized by late onset and simultaneous presentation of severe cardiomyopathy. Our report highlights the importance of considering transthyretin familial amyloid polyneuropathy in the differential diagnosis of late-onset, progressive, axonal polyneuropathies of unknown etiology, particularly if associated with cardiac disease.]

Clinical Neuroscience

Gray matter atrophy in presymptomatic Huntington’s patients

KIRÁLY András, KINCSES Zsigmond Tamás, SZABÓ Nikoletta, TÓTH Eszter, CSETE Gergő, FARAGÓ Péter, VÉCSEI László

Background - Huntington’s disease is a progressive disease in which neurodegeneration is on-going from the early presymptomatic phase. Development of sensitive biomarkers in this presymptomatic stage that are able to monitor the disease progression and test the efficacy of putative neuroprotective treatments are essential. Methods - Seven presymptomatic Huntington mutation carriers and ten age-matched healthy controls were recruited. Six of the patients participated in a 24 months longitudinal study having MRI scans 12 and 24 months after the baseline measurements. High resolution T1 weighted images were carried out and voxel based morphometry was used to analyse the data. Apart of group differences, correlation of CAG repeat number with focal cortical thickness and with global gray matter volume was calculated. Results - Focal cortical atrophy was found bilaterally in the superior temporal sulcus and in the left middle frontal gyrus in presymptomatic Huntington patients in whom no sign of cognitive or motor deterioration was detected. Global gray matter atrophy (p<0.048) and decreased total brain volume was found. The number of CAG triplets showed no correlation with the focal gray matter atrophy and total brain volume. Strong correlation between the CAG repeat number and global gray matter volume was found (p<0.016). Conclusion - Cortical atrophy is apparent in the early, presymptomatic stage of the disease. With further validation in large patient sample atrophy measure could be biomarker of disease progression and putatively of neurodegeneration.

All articles in the issue

Related contents

Lege Artis Medicinae

[Nutritional status, realizing sarcopenia and the importance of prehabilitation in surgical departments]

CSIBA Borbála, NAGY Ákos, LUKOVICH Péter, BAROK Bianka

[INTRODUCTION - Malnutrition can significantly influence the surgery’s outcomes. Currently, patients risk grouping is based on the body mass index (BMI), and the preparation for surgery is concerned only as nut­rients administration. PATIENTS AND METHODS - The Nutrition Support Team established in our Department is assessing first the patients’ nutritional status (BMI, MUST), sarcopenia (skinfold measure, handgrip strength) and fitness status (6 min step test, sit to stand test). Risk group patients were suggested nutrients and physiotherapy prior to the surgical operation. In order to follow up our patients we created an online interface and repeated the tests immediately before the operation. RESULTS - 135 cancer patients (76 male and 59 female) were operated. Their average age was 69.6 years. 33 patients had weight loss before the first consultation (average=8.7 kg). Their average BMI was 26.3. 21 patients had gained weight in the last 6 months (average=7.8 kg). Patients with left descending colon, liver - and also pancreatic tumors had overweight BMI values while the rest of pa­tients ill with right colon and stomach neo­plasm had normal weight. Those patients who we enrolled to the online system had better results at the second assessment. CONCLUSIONS - According to our survey, most of the patients had overweight BMI values but had sarcopenia based on anthropometric tests. Therefore, the importance of in time recognized and preoperatively started nutritional therapy must be coupled with the parallel applied physiotherapy. ]

Clinical Oncology

[Up-to-date treatment of head and neck cancers]


[The head and neck squamous cell cancer is often detected at an advanced stage, resulting in dismal prognosis despite the relevant developments of the oncotherapy in the last decades. The introduction of new techniques, new drugs and combinations though improves the survival for certain subgroups of patients, meanwhile the organ-function preservation and side effects reduction approaches lead to improved quality of life. The preventive-supportive care prior and during the therapy (dental care, nutrition, toxicity management) and the complex rehabilitation has paramount importance. MRI and 18FDG PET-CT and the advanced methods of molecular pathology became part of the diagnostic work up. The selection of the therapy based on the tumor characteristics, HPV detection, on the patient’s condition and wishes, as well as on multi-disciplinary team decision based on the available technical options. Early stage tumors can be successfully treated either by surgery alone or by radiation therapy. For locally advanced cases concomittant chemo-radiotherapy stays in the axis of the complex management using advanced radiation technique (IGRT-adaptive- IMRT) with cisplatin (or if contraindicated with cetuximab), which could be complemented by induction chemotherapy and/or surgery. Several new approaches are currently being tested in clinical studies after establishment of cisplatine-cetuximab treatment for recurrent/metastatic tumors. In the future, detection of molecular processes and driver mutations could result in development of more effective targeted anti-tumor agents, and individual molecular tumor profi le guided therapy, including the various forms and combinations with emerging immunotherapy.]

Lege Artis Medicinae

[Treatment of pneumothorax in cystic fibrosis]


[INTRODUCTION - More and more children affected by cystic fibrosis reach adulthood. The frequency of complications, such as pneumothorax, increases parallel with the long disease course. PATIENTS AND METHODS - The treatment of 17 manifestations of pneumothorax in 10 young adult cystic fibrosis patients in our institute was analysed retrospectively. RESULTS - Depending on the extension of pneumothorax and the clinical state of the patients the first choice of the treatment was observation alone in 6 cases, insertion of pleuracan in 3 cases and tube thoracostomy in 8 cases. Due to ineffective suction, thoracotomy was necessary in 6 patients, bilaterally in 3 cases. CONCLUSION - The outcome of conservative treatment even in "small, circumscribed pneumothorax" cases of symptom-free patients is uncertain and risky. Insertion of a pleuracan is recommended only in urgent, life-threatening cases, but definitive result should not be expected. If possible, chest drainage should be chosen as the first procedure. After 5-7 days of ineffective chest suction, thoracotomy is mandatory. Recurrence of the pneumothorax is direct indication for surgery. Antibiotic treatment is suggested for the duration of drainage, as well as for the surgical procedure.]

Clinical Oncology

[Treatment of childhood tumors of mesenchymal origin]

CSÓKA Monika

[Mesenchymal cells can be differentiated into skeletal muscle, smooth muscle, adipose tissue, fi brous tissue, bone and cartilage. Tumors can be originated from these tissues as benign tumors - fibroma, lipoma, osteoma, chondroma, haemangioma, myoma, etc. or as malignant tumors - in childhood, most commonly rhabdomyosarcomas, osteosarcoma, Ewing sarcoma, less often fi brosarcoma, liposarcoma or other rare types. Clinically, the outcome of these tumors have improved signifi cantly in the last decade due to the use of multi-modality treatment (chemotherapy, surgery, irradiation, in some cases targeted therapy). The better treatment results are based on early diagnosis and adequate management according to international treatment protocols in pediatric oncology centers.]

Clinical Neuroscience

[Symptomatic subependymomas of the ventricles. Review of twenty consecutive cases]


[Background and purpose - Intraventricular subependymomas are rare benign tumors, which are often misdiagnosed as ependymomas. To review the clinicopathological features of subependymomas. Patient selection and methods - Retrospective clinical analysis of intraventricular subependymomas and systematic review of histological slides operated on at our center between 1985 and 2005. Results - Twenty subependymomas presented at the median age of 50 years (range 19-77). Two (10%) were found in the third, three (15%) in the forth, and 15 in the lateral ventricles. There was male preponderance (12 vs. 8). Ataxia (n=13) and papilledema (n=7) were the most common clinical presentations. Fifteen patients underwent gross total resection, and five had subtotal resection. None of the cases showed mitotic figures, vascular endothelial proliferation or necrosis. Cell proliferation marker MIB-1 activity (percentage of positive staining tumor cells) ranged from 0 to 1.4% (mean 0.3). Two cases were treated with preoperative radiation therapy (50 Gy) before the CT era, three other patients received postoperative radiation therapy for tumors originally diagnosed histologically as low grade ependymomas. Three patients (15%) died of surgical complication between one and three months postoperatively, and three patients died of unrelated causes in eight, 26 and 110 months. Fifteen patients were alive without evidence of tumor recurrence at a median follow-up time of 10 years. Conclusion - Subependymomas are low-grade lesions and patients do well without adjuvant radiotherapy. Small samples from more cellular areas may be confused with low grade ependymomas, and unnecessary radiotherapy may follow. Recurrences, rapid growth rates should warrant histological review, as hypocellular areas of ependymomas may also be a source of confusion.]