Angiomyofibroblastoma in a male patient ­ a case report on a rare paratesticular lesion and review of the literature / Angiomyofibroblastoma férfiban - egy ritka paratesticularis eltérés és irodalmi áttekintés.

Összefoglaló. A scrotum képalkotó vizsgálóeljárásai közül elsonek választandó az ultrahang, mivel könnyen hozzáférheto, szenzitivitása és specificitása magas. Szerepe kiemelendo mind az intratesticularis eltérések differenciáldiagnózisában, mind pedig a kevésbé ismert paratesticularis eltérések esetében. Az urológiai ambulancián egy 56 éves férfi jelentkezett kivizsgálásra tapintható terime miatt. Ultrahangvizsgálattal paratesticularis elváltozás látszódott, mely a vizsgálat során az inguinalis csatorna irányába többször elmozdult. A laesio dignitása nem volt meghatározható, ezért mutéti eltávolításra került sor. A szövettani vizsgálat a férfiak körében ritkán eloforduló angiomyofibroblastoma diagnózisát véleményezte. A paratesticularis elváltozások ugyan ritkán fordulnak elo, de a gyakoribb entitások és azok ultrahangos sajátosságainak ismerete elengedhetetlen a terápia tervezése szempontjából. Orv Hetil. 2021; 162(34): 1376-1382. Summary. Ultrasonography is the basic imaging technique for the evaluation of testicular structures because it is easily accessible and has high sensitivity and specificity. It plays a significant role in the differential diagnosis of intratesticular changes, in addition, its role should be emphasised in rare paratesticular abnormalities. A 56-year-old male presented in the urology department complaining of a palpable inguinal mass and was referred to ultrasonography for further evaluation. A scrotal ultrasound was performed, and it described a mobile paratesticular mass without any specific characterizations. Therefore the lesion was removed, and the histological analysis established the diagnosis of angiomyofibroblastoma. Paratesticular lesions are rare, but it is essential to know the frequent abnormalities and the corresponding ultrasound findings for planning of treatment. Orv Hetil. 2021; 162(34): 1376-1382.

"Gap in the shield" ­ imaging of the thyroid gland from the multidisciplinary perspective / "Rés a pajzson" - a pajzsmirigy modern képalkotó vizsgálata multidiszciplináris szemszögbol.

Összefoglaló. A pajzsmirigy az elso szervek közé tartozik, melyek megjelenítésében, betegségeinek felfedezésében az ultrahang-diagnosztikának fontos szerepe van. A pajzsmirigybetegségek a lakosság jelentos részét érintik, és a technika fejlodésével egyre több pajzsmirigyeltérés, göb kerül felfedezésre. A pajzsmirigy rosszindulatú folyamatainak nincs egy bizonyos specifikus jele, viszont az ultrahangkép alapján meghatározhatók a malignitásra gyanús eltérések. Erre az elmúlt években több összefoglaló rendszer is született. Jelen összefoglaló tanulmányunknak az a célja, hogy bemutassuk a pajzsmirigy ultrahangdiagnosztikájának fejlodését; összehasonlítsuk az egyes leletezési rendszereket, úgymint TIRADS, EU-TIRADS, K-TIRADS, melyek célja a feltehetoleg rosszindulatú göbök kiszurése, azonosítása a mindennapi rutinmunka során; vizsgáljuk a különbözo rendszerek kapcsolatát a patológia által használt Bethesda-pontrendszerrel. Az ultrahangvizsgálat megfelelo értékelése, a pontrendszerek ismerete segíthet a pajzsmirigygöb differenciáldiagnózisában, a követési frekvencia meghatározásában, csökkentheti az aspirációs citológiák számát, ezzel támogatva a klinikai döntéshozatalt. Orv Hetil. 2021; 162(14): 530-541. Summary. The thyroid gland was one of the first organs, the ultrasound (US) examination of which has played an important role. The thyroid diseases affect a large part of the population, and with the development of imaging technology, more and more thyroid abnormalities, nodules and malignant lesions are being discovered. There are no specific signs of thyroid cancer, but the suspicious signs could be determined by US. In recent years, several systems have been developed. The aim of our review is to demonstrate the development of US diagnostics of the thyroid gland; to compare the different reporting systems, such as TIRADS, EU-TIRADS, K-TIRADS, which should help to identify the questionable lesions in the daily routine work. We examine the relationship between the different US systems and the Bethesda point score used by pathologists. The literature review shows that the US examination supports the clinical decisions, helps to select, who should have a fine-needle biopsy, and allows to determine the frequency of follow-up. The number of unnecessary fine-needle biopsies could be reduced, too. Our paper is part of a bigger research, the ethical license number is 23/2020, University of Szeged. Orv Hetil. 2021; 162(14): 530-541.

Testicular adrenal rest tumor in the background of congenital adrenal hyperplasia / [Testicularis adrenalis rest tumor a congenitalis adrenalis hyperplasia hátterében]

The prevalence of testicular adrenal rest tumours varies in different forms of congenital adrenal hyperplasia. Patients with 21-hydroxilase deficiency usually have bilateral and palpable testicular nodules. Although adrenal rest tumours are well documented in the literature, the diagnosis and management require a multidisciplinary approach: the cooperative work of endocrinologists, urologists, pathologists and radiologists is essential. In the case of an early diagnosis, appropriately increased corticosteroid treatment may reduce the tumour mass. In advanced stages, tumours can lead to irreversible parenchymal damage causing infertility. The importance of an early and accurate diagnosis cannot be emphasized enough, since the therapy differs significantly from other benign or malignant testicular neoplasia. A case of a testicular adrenal rest tumour is presented along with the multidisciplinary perspectives of the diagnosis and management of these lesions. Orv Hetil. 2020; 161(16): 623­631.

Correlation of neurochemical and imaging markers in migraine: PACAP38 and DTI measures.

OBJECTIVE: To examine whether interictal plasma pituitary adenylate cyclase-activating peptide 38-like immunoreactivity (PACAP38-LI) shows correlation with the microstructural integrity of the white matter in migraine. METHODS: Interictal plasma PACAP38-LI was measured by radioimmunoassay in 26 patients with migraine (24 women) who underwent diffusion tensor imaging afterward using a 1.5-tesla magnetic resonance scanner. Data were analyzed using tract-based spatial statistics included in FMRIB's Software Library. RESULTS: Interictal plasma PACAP38-LI showed significant correlation with mean diffusivity (p < 0.0179) mostly in the bilateral occipital white matter spreading into parietal and temporal white matter. Axial and radial diffusivity showed positive correlation with interictal PACAP38-LI (p < 0.0432 and p < 0.0418, respectively) in the left optic radiation and left posterior corpus callosum. Fractional anisotropy did not correlate significantly with PACAP38-LI. With disease duration as a nuisance regressor in the model, PACAP38-LI correlated with axial and mean diffusivity in the left thalamus (p < 0.01). CONCLUSION: We report a link between PACAP38, a pathobiologically important neurochemical biomarker, and imaging markers of the disease that may bolster further research into the role of PACAP38 in migraine.

Paraneoplastic neuromyelitis optica spectrum disorder: A case report and review of the literature.

Neuromyelitis optica spectrum disorders (NMOSD) are demyelinating, autoimmune diseases affecting the central nervous system. Typically, recurrent optic neuritis and longitudinal extensive transverse myelitis dominates the clinical picture. In most cases NMOSD are associated with autoantibodies targeting the water channel aquaporin-4 (AQP-4). NMOSD usually present in young adults. Clinical findings suggestive of NMOSD in elderly patients should raise the suspicion of a paraneoplastic etiology. To our knowledge, we report the first case of a 66 year-old female patient with paraneoplastic NMOSD that is associated with squamous cell lung carcinoma. Anti-AQP-4 was present in both the serum and cerebrospinal fluid of the patient. However, immunhistological staining of the malignant tissue did not show presence of AQP-4 on the surface of tumour cells.

[Magnetic resonance imaging in the course of alemtuzumab and teriflunomide therapy]. / Mágnesesrezonancia-képalkotás alemtuzumabés teriflunomidkezelés során.

Our work aimed to review the published results of magnetic resonance imaging (MRI) obtained in the course of alemtuzumab and teriflunomide therapy in multiplex sclerosis. In multiplex sclerosis MRI sensitively detects subclinical pathological processes, which do not manifest clinically in the early course of the disease, however have substantial significance from the viewpoint of the long-term disease prognosis. MRI has an increasingly important role in the early monitoring of the therapeutic efficacy. In the last 15 years several clinical trials have been conducted with alemtuzumab and teriflunomide in multiple sclerosis providing evidence about the favourable clinical effect of these drugs. MRI images were acquired in these trials as well, and the results published recently in the scientific literature. These MRI results denote the suppression of the disease activity and the neurodegenerative processes, which may imply a favourable effect on the long-term prognosis of the disease.

Release of PACAP-38 in episodic cluster headache patients - an exploratory study.

BACKGROUND: Activation of the trigeminal-autonomic reflex, involving the trigeminal ganglion, the superior salivatory nucleus and the sphenopalatine ganglion (SPG) is crucial in the pathophysiology of cluster headache (CH). Since pituitary adenylate cyclase-activating polypeptide-38 (PACAP-38) is present both in the SPG and the trigeminal ganglion (TG) and its role in migraine has been described, our aim was to determine the plasma PACAP-38 levels in different phases of episodic CH (ECH). Peripheral cubital fossa blood samples were taken during the ictal and inter-bout periods of male ECH patients and from age-matched healthy controls (n = 9). Plasma PACAP-38-like immunoreactivity (LI) was measured with specific and sensitive radioimmunoassay. FINDINGS: Significantly lower plasma PACAP-38-LI was detected in the inter-bout period of ECH patients than in healthy controls. However, PACAP-38 was significantly elevated in the plasma during CH attacks as compared to the inter-bout phase in the same subjects (n = 5). CONCLUSIONS: This exploratory study suggests that PACAP-38 may be released during the attacks of ECH. Further patients and long-term follow-up are necessary to reveal its function.

Grey matter atrophy in patients suffering from multiple sclerosis.

White matter lesions are defining characteristics of multiple sclerosis (MS), whereas grey matter involvement is a less recognised attribute. Recent investigations using dedicated imaging approaches have made it possible to depict cortical lesions. Additionally, grey matter atrophy may be estimated using various methods. Several studies have suggested that grey matter atrophy closely correlates to clinical disability. In this review we have collected information on grey matter atrophy in MS and the effect of disease modifying therapies upon brain atrophy.

The role of probabilistic tractography in the surgical treatment of thalamic gliomas.

BACKGROUND: Thalamic gliomas represent a great challenge for neurosurgeons because of the high surgical risk of damaging the surrounding anatomy. Preoperative planning may considerably help the surgeon find the most ideal operative trajectory, avoiding thalamic nuclei and important white matter pathways adjacent to the tumor tissue. Thalamic segmentation is a promising imaging tool based on diffusion tensor magnetic resonance imaging. It provides the possibility to predict the relationship of the tumor to thalamic nuclei. OBJECTIVE: To propose a new tool in thalamic glioma surgery that may help to differentiate between normal thalamus and tumor tissue, making preoperative planning possible and facilitating the choice of the optimal surgical approach and trajectory for neuronavigation-assisted surgery. METHODS: Four patients with thalamic gliomas preoperatively underwent conventional and diffusion-weighted magnetic resonance imaging conducted on 1.5 T. Subsequently, probabilistic tractography and thalamic segmentation were performed with the FSL Software as preoperative planning. We also present a case when thalamic segmentation was applied retrospectively using preoperative images. All patients went through neuronavigation-assisted surgery (1 partial, 4 subtotal resections). RESULTS: Surgery performed based on the output of thalamic segmentation caused no deterioration in the neurological symptoms of our patients. Indeed, we noticed improvement in the neurological condition in 3 cases; furthermore, in 2 patients, a concern-free state was achieved. CONCLUSION: We suggest that thalamic segmentation may be applied successfully and routinely in the surgical treatment of thalamic gliomas.

Alterations in PACAP-38-like immunoreactivity in the plasma during ictal and interictal periods of migraine patients.

BACKGROUND: Recent studies on migraineurs and our own animal experiments have revealed that pituitary adenylate cyclase-activating polypeptide-38 (PACAP-38) has an important role in activation of the trigeminovascular system. The aim of this study was to determine the PACAP-38-like immunoreactivity (LI) in the plasma of healthy subjects, and parallel with the calcitonin gene-related peptide (CGRP)-LI in migraine patients in the ictal and interictal periods. METHODS: A total of 87 migraineurs and 40 healthy control volunteers were enrolled in the examination. Blood samples were collected from the cubital veins in both periods in 21 patients, and in either the ictal or the interictal period in the remaining 66 patients, and were analysed by radioimmunoassay. RESULTS: A significantly lower PACAP-38-LI was measured in the interictal plasma of the migraineurs as compared with the healthy control group ( P < 0.011). In contrast, elevated peptide levels were detected in the ictal period relative to the attack-free period in the 21 migraineurs ( P PACAP-38 < 0.001; P CGRP < 0.035) and PACAP-38-LI in the overall population of migraineurs ( P < 0.009). A negative correlation was observed between the interictal PACAP-38-LI and the disease duration. CONCLUSION: This is the first study that has provided evidence of a clear association between migraine phases (ictal and interictal) and plasma PACAP-38-LI alterations.

Target identification for stereotactic thalamotomy using diffusion tractography.

BACKGROUND: Stereotactic targets for thalamotomy are usually derived from population-based coordinates. Individual anatomy is used only to scale the coordinates based on the location of some internal guide points. While on conventional MR imaging the thalamic nuclei are indistinguishable, recently it has become possible to identify individual thalamic nuclei using different connectivity profiles, as defined by MR diffusion tractography. METHODOLOGY AND PRINCIPAL FINDINGS: Here we investigated the inter-individual variation of the location of target nuclei for thalamotomy: the putative ventralis oralis posterior (Vop) and the ventral intermedius (Vim) nucleus as defined by probabilistic tractography. We showed that the mean inter-individual distance of the peak Vop location is 7.33 mm and 7.42 mm for Vim. The mean overlap between individual Vop nuclei was 40.2% and it was 31.8% for Vim nuclei. As a proof of concept, we also present a patient who underwent Vop thalamotomy for untreatable tremor caused by traumatic brain injury and another patient who underwent Vim thalamotomy for essential tremor. The probabilistic tractography indicated that the successful tremor control was achieved with lesions in the Vop and Vim respectively. CONCLUSIONS: Our data call attention to the need for a better appreciation of the individual anatomy when planning stereotactic functional neurosurgery.

Novel therapy in Parkinson's disease: adenosine A(2A) receptor antagonists.

INTRODUCTION: Parkinson's disease (PD) is a progressive neurodegenerative disorder. To date, most of the currently available therapies in PD target the dopaminergic system and none of these therapeutic approaches have been proven to modify the course of the disease. To various extents, these drugs can also cause motor and non-motor complications. A novel target, the adenosine A(2A) receptor (AA2AR), was recently identified, blockade of which may alleviate Parkinsonian symptoms, reduce motor fluctuations and potentially afford neuroprotection. AREAS COVERED: This review is based on a PubMed search covering the relationship of the adenosine receptors and PD. The role of the AA2AR is reviewed and the results of preclinical investigations of antagonists are assessed. A synopsis of current drug development is provided, with a special focus on the pharmacokinetics and relevant clinical trials. EXPERT OPINION: The localization of the AA2AR in the central nervous system, the ultra structural localization and the molecular mechanism of its action reveal the potential importance of the AA2AR in movement disorders. The theoretical background and experimental data indicate that AA2AR antagonists may have a potential therapeutic effect in Parkinson's disease. More importantly, the putative neuroprotective effect needs further investigation.