Hungarian Radiology

[Serious lesion of the skull]

APRIL 20, 2002

Hungarian Radiology - 2002;76(02)

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Hungarian Radiology

[CALENDAR OF RADIOLOGICAL EVENTS, 2002]

Hungarian Radiology

[Conference of the Young Radiologists]

BÁGYI Péter

Hungarian Radiology

[The incidence of Hirschsprung disease with associated congenital anomalies]

WEISENBACH János, KONDOR Ariella, KHEZRI Seddiq, VAJDA Péter

[INTRODUCTION - Several publisactions described the association of Hirschsprung disease with other congenital anomalies. Most studies were in relation with the neurological, ophthalmological and ear-nose-throat disorders. The authors did not find any publication in the Hungarian literature presented other anomalies in patients with Hirschsprung disease. PATIENTS AND METHODS - The data of 43 patients treated for Hirschsprung disease at the Department of Paediatrics of Pécs University was analysed. The most frequent associated anomalies e. g. Down syndrome, omphalocele, anorectal malformation, annular pancreas, torticollis, syndactylia, Meckel diverticulum, upper urinary tract disorder and undescendent testis were studied. The patients who had several congenital anomalies were evaluated separately as well as those having congenital inguinal hernia. The authors compared the number of congenital anomalies occurred in patients with Hirschsprung disease with the incidence of these congenital disorders occurred in non-Hirschsprung patients in the literature. RESULTS - Among 43 patients, 6 children had associated congenital anomalies. 3 patients had only one of the following disorders: undescendent testis, urinary tract duplication and Down syndrome. 3 patients suffered from multiplex congenital anomalies: 1. Down syndrome with annular pancreas and torticollis. 2. Omphalocele, anal atresia, vesicoureteric reflux and syndactylia. 3. Anus atresia, Meckel diverticulum and limb (radius) hypoplasia. In addition 3 patients had other anomalies such as unilateral and bilateral inguinal hernia and umbilical hernia. CONCLUSION - On the basis of our results we can state that the incidence of other associated congenital anomalies are higher in patients with Hirschsprung disease. Single organ developmental anomaly is rare, it usually occurs in association with multiorgan disorders. Most likely this is the explanation why patients with Hirschsprung disease have more associated anomalies.]

Hungarian Radiology

[Atypical diaphragmatic herniation causing bowel obstruction]

ERDŐSI Éva, MÉSZÁROS Anikó, GREXA Erzsébet

[INTRODUCTION - A 21-year-old woman presented with iatrogenic diaphragmatic hernia causing bowel obstruction. CASE REPORT - Plain abdominal X-ray and barium swallow examinations showed a diaphragmatic hernia causing partial gastric obstruction. Surgery confirmed the radiological diagnosis. The hernia was located atypically at the centre of the diaphragm. CONCLUSION - The cause of the hernia was probably related to a pyogenic necrosis due to subphrenic abscess after cholecystectomy performed 2 years earlier, treated by drainage and repeated surgical interventions.]

Hungarian Radiology

[Hungarian radiologists in Sweden]

KARLINGER Kinga

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[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.]

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Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge

TOLVAJ Balázs, HAHN Katalin, NAGY Zsuzsanna, VADVÁRI Árpád, CSOMOR Judit, GELPI Ellen, ILLÉS Zsolt, GARZULY Ferenc

Background and aims – Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports – Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion – Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Clinical Neuroscience

Extraskeletal, intradural, non-metastatic Ewing’s sarcoma. Case report

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Intracranial localization of Ewing’s sarcoma is considerably very rare. Herein, we present clinical and neuroimaging findings regarding a 4-year-old boy with intracranial Ewing’s sarcoma. He was born prematurely, suffered intraventricular haemorrhage, posthaemorrhagic hydrocephalus developed, and a ventriculoperitoneal shunt was inserted in the newborn period. The patient endured re­gular follow ups, no signs of shunt malfunction nor increased intracranial pressure were observed. The last neuroima­ging examination was performed at 8 months of age. Upon reaching the age of 4 years, repeated vomiting and focal seizures began, and symptoms of increased intracranial pressure were detected. A brain MRI depicted a left frontoparietal space-occupying lesion infiltrating the superior sagittal sinus. The patient underwent a craniotomy resulting in the total excision of the tumour. The histological examination of the tissue revealed a small round blue cell tumour. The diagnosis was confirmed by the detection of EWSR1 gene translocation with FISH (fluorescent in situ hybridization). No additional metastases were detected during the staging examinations. The patient was treated in accordance to the EuroEwing 99 protocol. Today, ten years onward, the patient is tumour and seizure free and has a reasonably high quality of life.

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[Zonisamide: one of the first-line antiepileptic drugs in focal epilepsy ]

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[Chronic administration of antiepileptic drugs without history of unprovoked epileptic seizures are not recommended for epilepsy prophylaxis. Conversely, if the patient suffered the first unprovoked seizure, then the presence of epileptiform discharges on the EEG, focal neurological signs, and the presence of epileptogenic lesion on the MRI are risk factors for a second seizure (such as for the development of epilepsy). Without these risk factors, the chance of a second seizure is about 25-30%, while the presence of these risk factors (for example signs of previous stroke, neurotrauma, or encephalitis on the MRI) can predict >70% seizure recurrence. Thus the International League Against Epilepsy (ILAE) re-defined the term ’epilepsy’ which can be diagnosed even after the first seizure, if the risk of seizure recurrence is high. According to this definition, we can start antiepileptic drug therapy after a single unprovoked seizure. There are four antiepileptic drugs which has the highest evidence (level „A”) as first-line initial monotherapy for treating newly diagnosed epilepsy. These are: carbamazepine, phenytoin, levetiracetam, and zonisamide (ZNS). The present review focuses on the ZNS. Beacuse ZNS can be administrated once a day, it is an optimal drug for maintaining patient’s compliance and for those patients who have a high risk for developing a non-compliance (for example teenagers and young adults). Due to the low interaction potential, ZNS treatment is safe and effective in treating epilepsy of elderly people. ZNS is an ideal drug in epilepsy accompanied by obesity, because ZNS has a weight loss effect, especially in obese patients.]

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Isolated hypoglossal nerve palsy due to a jugular foramen schwannoma

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