Clinical Neuroscience

[Diffusion weighted magnetic resonance imaging of metachromatic leukodystrophy - a case report]

PINTÉR Nándor1, KOLLÁR Katalin2, RUDAS Gábor3, BARSI Péter3

MARCH 25, 2009

Clinical Neuroscience - 2009;62(03-04)

[We present the case of a 7 years old boy with the juvenile form of metachromatic leukodystrophy. Beside conventional sequences, a diffusion-weighted magnetic resonance study was performed. On T2-weighted images a diffuse hyperintensity of the cerebral white matter with a typical tigroid pattern was seen as a well-known characteristics of the disease. No difference was detectable on T2-weighted images between the different cerebral white matter regions. Diffusion study showed low apparent diffusion coefficient values in the temporal and orbitofrontal regions and normal values in other cerebral regions compared to the normal appearing cerebellar white matter, wich is partly consistent with the previously reported data. The results of early diffusion studies of the disease are not straightforward and the physical and biochemical backgrounds of the diffusion properties still remain unknown. The more data we collect, the closer we get to the understaning of the diffusion changes in metachromatic leukodystrophy.]


  1. Semmelweis Egyetem, Kardiológiai Központ, Képalkotó Diagnosztikai Részleg, Budapest
  2. Heim Pál Gyermekkórház, Neurológiai Osztály, Budapest
  3. Semmelweis Egyetem, MR Kutatóközpont, Budapest



Further articles in this publication

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.]

Clinical Neuroscience

[Account on the 17. Congress of the Hungarian Society of Neuroradiology and on the position of neuroradiology in Hungary today]


[Account on the 17. Congress of the Hungarian Society of Neuroradiology and on the position of neuroradiology in Hungary today 2009;62(03-04)]

Clinical Neuroscience

[Current issues of neuroimaging diagnostics of multiple sclerosis]


[Magnetic resonance imaging techniques have been routinely used in diagnostics and follow-up of multiple sclerosis and magnetic resonance imaging findings are incorporated into the current diagnostic criteria of the disease. International guidelines were created aiming to define the indication, timing, minimum requirements and interpretation of magnetic resonance examination in multiple sclerosis. In Hungary, there is a lack of widely-accepted standardized protocol, therefore presenting the magnetic resonance diagnostic criteria and the international guidelines may prove useful. It may also point towards consideration of a national guideline.]

Clinical Neuroscience

[Application of diffusion weighted imaging in neuroradiology]


[Diffusion weighted magnetic resonance (MR) imaging is available on all modern MR scanners. It depicts the motion of water molecules in the brain tissue and intracranial foreign tissues and provides information on changes in the proportion of intra- and extracellular space and the characteristics of foreign intracranial tissues and fluids. It is of utmost importance eg. in the diagnostics and differential diagnostics of acute ischaemic brain lesions, the diagnostics of inflammatory brain processes and in the differential diagnostics of intracranial space-occupying lesions. The examination method of short scanning and post-processing time must be completed with the apparent diffusion coefficient (ADC) maps and it is indispensable in the everyday neuroradiological diagnostics. Diffusion tensor imaging and tractography are able to depict the white matter tracts. They require a longer scanning and post-processing time and have several technical problems yet to be solved, but they provide help in their current state e.g. in the surgical planning of intracranial space-occupying lesions.]

Clinical Neuroscience

[State of the art neurointerventional treatment of intracranial vascular anomalies]


[Haemorrhagic stroke is frequently caused by rupture of intracranial vascular anomalies. The role of minimally invasive therapy in the treatment of such lesions has increased dramatically within the past two decades. The purpose of this study is to summarize the pathology and clinical features of these anomalies and to overview the potential applications of neurointerventional techniques in their treatment. Endovascular therapy is the first choice of treatment for most intracranial aneurysms. Both pial and dural arteriovenous malformations are being treated by endovascular techniques, but the combination of different modalities (such as endovascular, direct surgery and radiosurgery) is frequently applied. Capillary malformations require surgical removal and venous anomalies do not allow for any type of invasive treatment. State of the art therapy of intracranial vascular anomalies require institutions equipped with appropriate imaging facilities and having equal access to both conventional neurosurgical and neurointerventional techniques with ample experience and case load.]

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[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

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.

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

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Clinical Neuroscience

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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

[Magnetic resonance imaging in the course of alemtuzumab and teriflunomide therapy]

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