Lege Artis Medicinae

[TAKAYASU’S ARTERITIS DETECTED IN AN EARLY PHASE]

SZÁNTÓ Antónia, VEISZ Richárd, KRENÁCS László, CSIKI Zoltán, GRIGER Zoltán, ZEHER Margit

SEPTEMBER 15, 2006

Lege Artis Medicinae - 2006;16(08-09)

[INTRODUCTION - Takayasu’s arteritis is an idiopathic inflammatory disease of the great elastic arteries, mainly of the aorta and its primary branches. It primarily affects young women. CASE REPORT - A 20-year-old woman presented with general inflammatory symptoms that had begun 3 to 4 months before. Earlier she underwent a biopsy from a mass on the neck thought to be a lymph node, which on histopathological examination turned out to be a non-necrosing granulomatosis in the wall of a large artery. Based on this finding as well as on the presence of fever, weight loss, carotid murmur, anaemia, elevated platelet count, increased sedimentation rate, the lack of pulse and the findings of imaging examinations, Takayasu’s arteritis was diagnosed. Treatment was started with high-dose steroid monotherapy, which resulted in clinical and laboratory remission after a short time. The imaging findings (neck soft tissue sonogram and MRI) also showed improvement, however, a moderate but definite disease activity was still present, thus steroid treatment was supplemented with azathioprin. CONCLUSION - It is important to follow the disease both clinically and with imaging techniques for an early recognition of the need for changing or adjusting the treatment.]

COMMENTS

0 comments

Further articles in this publication

Lege Artis Medicinae

[A new simple tool for tonometric determination of the pCO2 in the gastrointestinal tract]

BODA Domokos, TÁLOSI Gyula, KASZAKI József

Lege Artis Medicinae

[STATE-OF-THE-ART DIAGNOSIS AND TREATMENT OF PITUITARY ADENOMAS SECRETING GROWTH HORMONE]

CZIRJÁK Sándor

[The history of the treatment of pituitary adenomas that cause acromegaly is as long as that of neurosurgery. While in the first half of the past century the aim of surgery was to save the patient's life, later the radical removal of the tumour was coupled with an effort to decrease complications, morbidity and mortality to the minimum. Today, beside all these, the complete sparing of the remaining hypophyseal substance and restoration of normal pituitary function are also important goals. The achievement of these goals is efficiently served by recent advances in microscopy, the minimally invasive methods of craniotomy, the availability of endoscopy in neurosurgery, three-dimensional computerguided neuronavigation, intraoperative colour Doppler sonography, as well as intraoperative real-time MRI. Recent developments in pharmacological research have created new promising conservative treatment modalities that supplement surgery, including somatostatin analogues and growth hormone receptor agonists. Also as supplementary treatment to surgery, occasionally replacing it, new radiosurgical methods, such as stereotaxic radiation, gamma knife, and heavy particle irradiation have gained grounds in neurosurgical practice.]

Lege Artis Medicinae

[BRONCHUS ASSOCIATED LYMPHOID TISSUE LYMPHOMA]

HERTEL Katalin, ZSIRAY Miklós, SOLTÉSZ Ibolya

[INTRODUCTION - Primary lymphomas rarely occur in the lung. CASE REPORT - The authors present the case of an asymptomatic 61-year-old man. The patient was identified on routine chest X-ray having a streak infiltrate in the upper lobe of the right lung, which did not respond to antibiotics. On histological examination of the bronchoscopic specimen BALT- (bronchus associated lymphoid tissue) lymphoma was presumed. Since staging showed the disease to be localized, lobectomy was performed. The patient is symptom-free 16 months after surgery and there is no relapse. CONCLUSION - Although BALT-lymphomas are of low-grade malignancy in most of the cases, relapses can develop in the original organ or in other extra-nodal locations years later and BALTlymphomas may also transform into large-cell lymphomas of more aggressive behaviour.]

Lege Artis Medicinae

[DIAGNOSIS, DIFFERENTIAL DIAGNOSIS AND TREATMENT OF HYPERPROLACTINAEMIA]

IVÁN Gabriella, GÓTH Miklós

[Hyperprolactinaemia is one of the most common endocrine diseases. Besides hypothyroidism- associated hyperprolactinaemia and that arising as a side effect of certain drugs, the most common cause of hyperprolactinaemia is a pituitary microadenoma (<10 mm in diameter) or macroadenoma (≥10 mm) that produces prolactin (prolactinoma). In addition, several physiological conditions can elevate (mostly temporarily) the serum prolactin level, therefore, setting up the precise diagnosis requires careful evaluation of the patient’s history and the laboratory, clinical, and imaging findings. Moreover, macroprolactinaemia, which is usually not a pituitary tumour-related disease, should also be ruled out. Prolactinomas represent the most common form of functioning pituitary adenomas, accounting for 30-40% of such tumours. The typical clinical symptoms of hyperprolactinaemia may be modulated by the mass effect of macroadenomas. In women the disease typically manifests as menstrual disturbance of various degree, including primary or secondary amenorrhoea, oligomenorrhoea, short luteal phase, infertility and galactorrhoea. In men reduced libido, impotence, infertility, gynaecomastia and, rarely, galactorrhoea are the typical symptoms. Dopamine agonist therapy is the first choice of treatment. Dopamine agonist therapy (bromocriptine and the recently developed quinagolide and cabergoline) successfully lowers the serum prolactine level in nearly 90% of cases, and, importantly, it also reduces the size of the tumour in the majority of cases. This explains why today surgery and radiotherapy are only used in rare special cases of prolactinoma.]

Lege Artis Medicinae

[BREAST CANCER CARE: FROM PREVENTION TO SURVEILLANCE]

KAHÁN Zsuzsanna

[Breast cancer is the most common malignancy in women in developed countries. The development of most breast cancers is related to various hormonal effects, while 10% is associated with inherited gene mutations. Most of the primary prevention methods aim at decreasing the effects of hormones, but education on proper lifestyle is also an important risk-lowering method. The primary treatment of early breast cancer is usually breast-conserving surgery, either with the targeted removal of regional lymph nodes (by sentinel lymph node labelling) or with axillary block-dissection. The aim of postoperative radiotherapy is the eradication of the tumour cells left behind. Beside the locoregional tumour control this also plays a role in the prevention of recurrence or a secondary systemic dissemination. Adjuvant systemic treatments are used for the eradication of disseminated microscopic tumour foci. The use of modern adjuvant treatments may reduce death from the disease by up to 50%. The risks of relapse or death may be estimated based on established prognostic factors. While in low-risk patients it is not worth starting medical treatment, especially in view of the side effects, while in other cases chemo- or hormonal therapy may save the patient's life. The choice of the medical treatment should also depend on the patient's general health, the concomittant diseases and her preferences. The collaboration of the various specialists involved in the care of breast cancer patients can best take place at specialised breast centres that are equipped with the necessary technical basis, knowledge and professional experience.]

All articles in the issue

Related contents

Clinical Neuroscience

Long-term follow-up results of concomitant chemoradiotherapy followed by adjuvant temozolomide therapy for glioblastoma multiforme patients. The importance of MRI information in survival: Single-center experience

LUKÁCS Gábor, TÓTH Zoltán, SIPOS Dávid, CSIMA Melinda, HADJIEV Janaki, BAJZIK Gábor, CSELIK Zsolt, SEMJÉN Dávid, REPA Imre, KOVÁCS Árpád

Introduction - Glioblastoma multiforme (GBM) is the most common malignant primary anomaly of central nervous system. The GBM infiltrates the nearly sturctures from the initial tumor and its metastatic attribution is well known. The aim of our single-centered retrospective study was to introduce the importance of postoperative medical imaging confirmation of total tumor resection for patient with GBM combined concomitant and adjuvant chemoradiotherapy on a 10 year long patient follow up. Methods - From January 2006 to April 2015 we registered 59 patients with newly diagnosed GBM at the University of Kaposvár Health Center Institute of Diagnostic Imaging and Radiation Oncology. The histological diagnosis was confirmed by a proficient neuropathologist (World Health Organisation WHO; grade IV astrocytoma). According to histological status if the ECOG performance status of patients allowed it the mutidisciplinary oncoteam recommended adjuvant chemoradiotherapy all features strictly by Stupp protocol. (60 Gy dose on the gross tumor volume and 2-3 cm margin for the clinical target volume with parallel 75 mg/m2 TMZ. Four weeks after monotherapial phase patients had to recieve 6 cycles of TMZ first cycle with 150 mg/m2 up to 200 mg/m2). The irradiation was carried out by a conformal three dimensional planning system. Results - 59 patients with the median age of 63 (range 17-84) year. Our sample counted 34 male patients and 25 woman patients. 14 patients underwent gross total tumor resection while, 39 patients underwent partial resection and the rest from our sample 6 patients passed through biopsy. Statistical analysis showed a lengthier survival among males than females, with a median survival of 13 months for males and females, the OS of 26.209 for males, meanwhile 15.625 for females. However, the difference is not considerable (log-rank p=0.203). Our study found that the estimated survival of patients at least 50 years old is significantly shorter at a median survival of 12 months (log rank p=0.027) than that of patients below 50 years of age at a median survival of 23 months. The longest estimated median survival was calculated with patients of ECOG '0' condition (16 months). However, no significant difference was found in the estimated survival of patients of different ECOG conditions (log-rank p=0.146). Based on the extent of surgery, complete resection resulted in the longest average survival of 36.4 months, followed by 21.5 months among patients with biopsy, and 15.8 months among patients with partial resection. Different surgical procedures, however, did not result in significant differences in survival (log-rank p=0.059). The overal survival of patients who had complete resection confirmed by MRI compared with the overal survival of patients with residual tumor confirmed by MRI as well we can estimate that there is significant difference between these two groups (p=0,004). Conclusion - Despite complex and intense treatment, recurrence is inevitable and causes relatively rapid death. In our analysis complete resection, as defined from the neurosurgeon’s report and postoperative MRI, resulted in an independently significant improvement in OS. Our results are the evidences that the treatment of patients with glioblastoma multiforme in Hungary is at least on the same level as any other developed European countries.

Clinical Neuroscience

Cerebral cavernous malformation type 1 with retinal blood vessel tortuosity and KRIT1 gene mutation

KALMÁR Tibor, MARÓTI Zoltán, VADVÁRI Árpád, HALMOSI Ágnes, KÁLOVITS Ferenc, KÁLMÁN Bernadette

Cerebral cavernous malformations (CCMs) represent a relatively rare and heterogeneous clinical entity with mutations identified in three genes. Both sporadic and familial forms have been reported. We present a young female patient with episodic paresthesia and headaches, but without acute neurological deficits. Her mother had a hemorrhaged cavernoma surgically removed 21 years ago. Cranial magnetic resonance imaging revealed multiple cavernous malformations in the size of a few millimeters and the ophthalmologic exam detected retinal blood vessel tortuosity in the proband. Targeted exome sequencing analysis identified a nonsense mutation in exon 16 of the KRIT1 gene, which resulted in a premature stop codon and a truncated protein underlying the abnormal development of cerebral and retinal blood vessels. This mutation with pathogenic significance has been reported before. Our case points to the importance of a thorough clinical and molecular work up despite the uncertain neurological complaints, since life style recommendations, imaging monitoring and genetic counseling may have major significance in the long term health of the patient.

Clinical Neuroscience

[The role of MRI in measuring the effectivity of disease modifying treatments II]

KINCSES Zsigmond Tamás, TÓTH Eszter, FRICSKA-NAGY Zsanett, FÜVESI Judit, RAJDA Cecília, BENCSIK Krisztina, VÖRÖS Erika, CSOMOR Angéla, PALKÓ András, VÉCSEI László

[The paraclinical examinations, principally the MRI have an increasing significance in the diagnosis of multiple sclerosis. However, MRI markers also have a prominent role in monitoring of the disease-course and activity, and also in the planning of possible therapeutic changes. In accordance with previously published international guidelines, in this article we propose a protocol for the monitoring the treatment efficacy in multiple sclerosis. This could be the basis of a consensus based guideline to be implemented in Hungary.]

Clinical Neuroscience

[Childhood sporadic type of hemiplegic migraine with arteria cerebri media hypoperfusion]

NAGY Csaba, BAJZIK Gábor, SKOBRÁK Andrea, CSORBA Eszter, LAJTAI Anikó, BALOGH Gábor, NAGY Ferenc, VAJDA Zsolt

[Hemiplegic migraine is a rare subtype of migraine that is associated with reversible motor weakness in the aura phase. This is an uncommon form of migraine usually starting in childhood. The purpose of this case report is to highlight the differential diagnostic difficulty of the first attack. We describe a case, where the fluctuating unilateral motor weakness and aphasia suggested that the patient had ischaemic stroke. Nevertheless the brain MRI and MR angiography, the measured 5-hydroxyindole acetic acid (5-HIAA) concentration changes and the spontaneously improving clinical status proved the diagnosis of hemiplegic migraine. The MRI and MR angiography was very beneficial in establishing the correct diagnosis in this case. To distinguish between the familiar and sporadic type of hemiplegic migraine further genetic tests can be carried out.]

Clinical Neuroscience

[The role of MRI in measuring the effectivity of disease modifying treatments I]

TÓTH Eszter, BENCSIK Krisztina, VÖRÖS Erika, FRICSKA-NAGY Zsanett, FÜVESI Judit, RAJDA Cecília, CSOMOR Angéla, PALKÓ András, VÉCSEI László, KINCSES Zsigmond Tamás

[MRI has a significant role in the diagnosis of multiple sclerosis. The newer and newer treatment options of the disease make it necessary to monitor the effectiveness of the therapy. Besides the clinical signs (clinical relapses and progression), the different MRI parameters can also reflect the disease activity. In our current article we summarize those MRI markers, which best predict the long-term disability, based on the international standards.]