Clinical Neuroscience

Cerebral amyloid angiopathy related inflammation: is susceptibility weighted imaging the clue for diagnosis?

CSÉCSEI Péter1, KOMOLY Sámuel1, SZAPÁRY László1, BARSI Péter1

MARCH 30, 2016

Clinical Neuroscience - 2016;69(03-04)

DOI: https://doi.org/10.18071/isz.69.E021

Background - Cerebral amyloid angiopathy-related inflammation (CAA-ri) is characterized by various neurological symptoms such as gradually developing confusion, progressive cognitive decline, seizure or headaches; T2 hyperintensities on magnetic resonance imaging (MRI); and neuropathological evidence of cerebral amyloid angiopathy (CAA) and associated vascular or perivascular inflammation. Although histological confirmation is necessary for accurate diagnosis, in case of typical clinical features and neuroimaging, the diagnosis can be established without biopsy. Case summary - We present the case of a 57-year-old man with a history of hypertension who presented to the emer¬gency department 3-week history of progressive headache and a gradually developing altered mental status. On examination, he was found to have left sided weakness and decreased pscyhomotility. Routine clinical work-up (lab investigations, CT, cerebrospinal fluid analysis) did not show obvious diagnosis, so we performed an MRI. It raised the suspicion of CAA-ri which diagnosis was verified by neuroradiological evaluation. High dose steroid treatment was initiated. The patient rapidly responded to treatment, his focal neurological signs resolved. Control MRI after 1.5 months showed multiple haemorrhagic laesions in the field of previous inflammation which posteriorly supported the previous supposed work-diagnosis. Conclusions - Although histopathology is the gold standard for the diagnosis of cerebral amyloid angiopathy, the typical clinical presentation, good response to steroids and accurate neuroradiological criteria make biopsy unnecessary to diagnose CAA-ri.

AFFILIATIONS

  1. Department of Neurology, University of Pécs, Pécs, Hungary

COMMENTS

0 comments

Further articles in this publication

Clinical Neuroscience

[Antecedents to the commencement and history of the West- Pannonic neurological forum]

GARZULY Ferenc, GRUBITS János, NIKL János

[Introduction - Numerous professional groups and sections for the medical specialities have been organized since 1953 in the West-Transdanubian region of Hungary, but such association of neurologists had not occured. Establishing the West-Pannonic Neurological Forum - The lack of regional collaboration among neurologists was related to several factors, among which the most important factor was the lack of a regional medical university, which could coordinate the professional activities. This severe gap necessitated in 1998 the organization of a professional group, that has become a driver for case-consulting conferences and different postgraduate trainings for the physicians specialized in neurology, neurosurgery and neurorehabilitation in counties of Győr-Moson-Sopron, Vas, Veszprém and Zala. The functioning of the Forum - Meetings are organized twice a year for physicians and paramedical staff (nurses, hospital attendants, physiotherapists) on Thursdays afternoons in different towns of the region, in two sections. The lectures are followed by a buffet, after which everyone can get home before too late. Ocasionally guest-lecturers are invited to present scientific topics from Hungarian universities or abroad. However, the main form of the presentations is defined as case discussion. Conclusions - The numbers of platform and other presentations in the physicians’s section have exceeded half a thousand, while in the paramedical section reached the threehundreds. At the 38. meeting of the Forum in January of this year, the number of participants was more than twohundreds, reflecting that both physicians and their coworkers are greatly interested in this form of interactions.]

Clinical Neuroscience

Hungarian experiences with the Beliefs About Attractiveness Scale

CZEGLÉDI Edit, SZABÓ Kornélia

Background and purpose - Sociocultural influences regarding bodily appearance and their psychological consequences play a considerable role in the development and maintenance of body image disturbance and eating disorders. The purpose of the study was to explore the psychometric properties of the Beliefs About Attractiveness Scale-Revised and its correlates among young adults in Hungary. Methods - In our cross-sectional online study, participants were 18-35 years old (N=820, 40% male). Measures: self-reported anthropometric data, Beliefs About Attractiveness Scale-Revised, Eating Disorder Inventory, SCOFF questionnaire, Sociocultural Attitudes Towards Appearance Questionnaire-3, and Rosenberg Self-esteem Scale. Results - The exploratory factor analysis showed that the fit indices of the three-factor solution are acceptable (c2(171)=5124.8, p<0.001, CFI=0.944, TLI=0.918, RMSEA=0.054, SRMR=0.030). Along the original ‘Importance of being thin’ and the ‘Importance of being fit’ factors, a third factor emerged, namely the ’Life fulfilment aspect of attractiveness’ factor. Internal consistency and construct validity of the scales were confirmed. Among those who were at risk of developing an eating disorder, all of the measured beliefs were significantly greater than among those who were not at risk (thin: Z=6.501, p<0.001, Cohen’s d=0.63, fit: t(818)=-4.749, p<0.001, Cohen’s d=0.41, and life fulfilment: t(239)=-5.702, p<0.001, Cohen’s d=0.53). Conclusion - The Hungarian version of the Beliefs About Attractiveness Scale-Revised is a reliable, valid measure and we suggest its introduction into Hungarian research. Relationships between beliefs about attractiveness and self-esteem, body image and eating disorders suggest intervention opportunities in with regards to prevention and treatment of eating disorders.

Clinical Neuroscience

The electrophysiological changes after 1 hz RTMS in ALS patients. A pilot study

MAJOR Zsigmond Zoltán, VACARAS Vitalie, MARIS Emilia, CRISAN Ioana, FLOREA Bogdan, MAJOR Andrea Kinga, MURESANU Fior Dafin

Motor neuron diseases are disabling poor prognostic conditions, with no successful treatment. Repetitive transcranial magnetic stimulation might offer a temporary functional improvement. Objective - We intended to evaluate the extent of the functional improvement using electrophysiological and clinical tests. Methods - Patients with motor neuron disease (amyotrophic lateral sclerosis) were included. Muscle strength and respiratory function assessment represents the clinical approach, and central motor conduction time, motor unit number estimation, blink reflex and H-reflex stands for electrophysiology. Two tests were performed using the whole battery prior and after low frequency repetitive transcranial magnetic stimulation, using 1 Hz stimulation frequency for five consecutive days, 20 minutes daily, at 80% of the individual resting motor threshold. Results - Central motor conduction time, muscle strength and pulmonary function showed no statistically significant differences, but a tendency towards improvement. Motor unit number estimation, blink reflex and H-reflex showed a significantly better outcome after the five day repetitive transcranial magnetic stimulation treatment. Conclusion - Low frequency repetitive transcranial magnetic stimulation influences beneficially electrophysiological parameters in amyotrophic lateral sclerosis, but with little clinical impact; further studies are needed to validate the extent of the effect.

Clinical Neuroscience

[The therapeutic use of transcranial magnetic stimulation in major depression]

NÉMETH Viola Luca, CSIFCSÁK Gábor, KINCSES Zsigmond Tamás, JANKA Zoltán, MUST Anita

[The antidepressive effect of repetitive transcranial magnetic stimulation (rTMS) has been investigated for almost 20 years now. Several studies have been published aiming to identify the exact and reliable parameters leading to the desired therapeutic effect. However, the related literature shows great variability. The current overview aims to provide a comprehensive overview of factors associated with the therapeutic effect of rTMS in major depression. High frequency stimulation of the left dorsolateral prefrontal cortex (DLPFC) for 3-6 weeks leads to mood improvement comparable to the effect of antidepressive medications in 35-40% of patients. Pharmacotherapy resistant patients treated with rTMS reach remission for 3 months on average. Low frequency stimulation of the right DLPFC appears to be similarly effective, though much less investigated so far. In addition to the exact delineation of the stimulation area, treatment outcome is also related to stimulation intensity as well as the number of sessions and impulses. Considering the safety and tolerability aspects of rTMS, it might be a significant therapeutic support for therapy resistant patients. Above this, patients diagnosed with major depression might benefit from the additional positive influence of rTMS improving the effect of antidepressive medication. Based on converging research evidence, the Food and Drug Administration (FDA) agency approved the use of rTMS as a treatment option for therapy resistant major depression in 2008. So far, in Hungary rTMS is primarily considered as a promising tool in research settings only. Hopefully, patients suffering from major depression will increasingly benefit from the positive therapeutic effect of this intervention.]

Clinical Neuroscience

[Computed tomographic examination of cranial lesions, a paleoradiological approach]

ZÁDORI Péter, BAJZIK Gábor, BÍRÓ Gergely, LELOVICS Zsuzsanna, BALASSA Tímea, BERNERT Zsolt, ÉVINGER Sándor, HAJDU Tamás, MARCSIK Antónia, MOLNÁR Erika, ŐSZ Brigitta, PÁLFI György, WOLFF Katalin, REPA Imre

[Background and purpose - Introducing the multidisciplinary paleoradiology research at the Institute of Diagnostic Imaging and Radiation Oncology of the Kaposvár University, highlighting the cases with potential central nervous system involvement - from the scanning methods to the 3D printing - in order to draw attention to the historical background and clinical aspects of certain pathological conditions. Methods - The authors developed the examination protocols for three different CT scanners. Among the examined archaeological remains cranial lesions were identified in 26 cases, from which 4 cases with potential central nervous system involvement are demonstrated. The scanning parameters and the advantages of secondary image reconstructions (multiplanar reconstruction, maximum intensity projection, three-dimensional volume rendering technique) are presented with the cases. Results - The authors demonstrate a case with destructive skull lesions due to syphilis from the 15th century AD, a condition rarely seen or even unknown nowadays in the modern world. With the CT images of the skull base fracture from the Iron Age, signs of healing could be verified. Using the CT images a non-invasive approach is presented in the case of the craniofacial osteosarcoma in order to visualize the local status and the direct intracranial propagation. Advantages of the 3D VRT reconstructions are shown in the case of unilateral coronal suture synostosis. Conclusion - Paleoradiological CT examinations serve as a non-invasive, non-destructive tool for studying archaeological remains and artifacts. The special applications provided by the imaging modality contribute to the conventional paleopathological investigations. Keywords: paleoradiology, computed tomography, 3D reconstruction, skull lesions, paleopathology]

All articles in the issue

Related contents

Clinical Neuroscience

A case with reversible neurotoxicity induced by metronidazole

EREN Fulya, ALDAN Ali Mehmet, DOGAN Burcu Vasfiye, GUL Gunay, SELCUK Hatem Hakan, SOYSAL Aysun

Background - Metronidazole is a synthetic antibiotic, which has been commonly used for protozoal and anaerobic infections. It rarely causes dose - and duration - unrelated reversible neurotoxicity. It can induce hyperintense T2/FLAIR MRI lesions in several areas of the brain. Although the clinical status is catastrophic, it is completely reversible after discontinuation of the medicine. Case report - 36-year-old female patient who had recent brain abscess history was under treatment of metronidazole for 40 days. She admitted to Emergency Department with newly onset myalgia, nausea, vomiting, blurred vision and cerebellar signs. She had nystagmus in all directions of gaze, ataxia and incompetence in tandem walk. Bilateral hyperintense lesions in splenium of corpus callosum, mesencephalon and dentate nuclei were detected in T2/FLAIR MRI. Although lumbar puncture analysis was normal, her lesions were thought to be related to activation of the brain abscess and metronidazole was started to be given by intravenous way instead of oral. As lesions got bigger and clinical status got worse, metronidazole was stopped. After discontinuation of metronidazole, we detected a dramatic improvement in patient’s clinical status and MRI lesions reduced. Conclusion - Although metronidazole induced neurotoxicity is a very rare complication of the treatment, clinicians should be aware of this entity because its adverse effects are completely reversible after discontinuation of the treatment.

Clinical Neuroscience

Insights into the structure and function of the hippocampal formation: Relevance to Parkinson’s disease

GYÖRFI Orsolya, NAGY Helga, BOKOR Magdolna, KÉRI Szabolcs

The link between the hippocampus and declarative memory dysfunctions following the removal of the medial temporal lobe opened unexplored fields in neuroscience. In the first part of our review, we summarized current theoretical frameworks discussing the role of hippocampus in learning and memory. Several theories are highlighted suggesting that the hippocampus is responsible for assembling stimulus elements into a unitary representation that later can be utilized to simulate future events. The hippocampal formation has been implicated in a growing number of disorders, from neurodegenerative diseases to atypical cognitive ageing and depression. Recent neuroimaging studies provided new opportunities to study in detail the hippocampal formation’s role in higher levels of the nervous system. We will present data regarding the regional specialization of the hippocampus in experimental models developed for healthy and neurological conditions with a special focus on Parkinson’s disease. Combined evidence from neuroimaging studies suggested that hippocampal volume is reduced in non-demented, newly diagnosed patients with Parkinson’s disease, which is associated with impaired memory performance. These findings proposed that, beyond the well-known striatal dopamine loss, impaired hippocampal synaptic plasticity may contribute to cognitive and affective impairments in early Parkinson’s disease.

Hungarian Radiology

[Radiological diagnostics of the pancreas neoplasms - Onco Update 2005]

FORRAI Gábor, BODOKY György

[Authors reviewed the recent results of pancreas tumour radiological diagnostics and the place of the imaging and interventional methods. Systematical review of the most recent articles were summarized (July 2003-December 2004) in the following subjects: the etiology and clinico-pathology, general diagnostic and therapeutical questions of early pancreatic neoplasms, abdominal ultrasound, computed tomography, multidetector computed tomography, magnetic resonance imaging, MR-cholangiopancreatography, endoscopic retrograde cholangiopancreatography, endoscopic ultrasound, intraductal ultrasound, endoscopic ultrasound-guided cytology, percutaneous biopsy, positron emission tomography, positron emission tomography - computed tomography, special pancreatic tumours. Experiences about the pancreas diagnostic methods are accumulating year-to-year rapidly. Therefore the current examination algorithm is changing continuously. New diagnostic and therapeutic modalities are entering in the daily practice. These are the reasons why the up-to-date knowledge of the literature is mandatory.]

Clinical Neuroscience

[Frontotemporal dementia - Part III - Clinical diagnosis and treatment]

GALARIOTIS Vasilis, BÓDI Nikoletta, JANKA Zoltán, KÁLMÁN János

[The authors report a comprehensive publication consisting of three parts going into the details of history, prevalence, clinical forms, differential diagnosis, genetics, molecular pathomechanism, and pathology, clinical diagnosis and treatment of frontotemporal dementia (FTD). The third part of the present review focuses on the clinical diagnosis and treatment of FTD. The diagnosis of FTD is problematic even today. Mental status, psychometric testing as well as imaging studies such as PET and SPECT, and laboratory examinations may be helpful in the diagnosis. Unfortunately, bedside tests are generally insensitive to mild dysexecutive deficits. Most of FTDs do not have characteristic laboratory abnormalities or brain atrophy sufficient to set up the diagnosis; these only allow to rule out other disorders and assume the diagnosis of FTD. An effective treatment for FTD is still to be established. The improvement of serotonin metabolism has been proposed as a biological treatment. Recent studies suggest that bromocriptine may improve selective frontal symptoms, but this and the efficacy of other dopaminergic drugs need further evaluation. Drugs that prevent the expression or accumulation of tau seem to be the most promising causal approach. In aphasia behavioral therapy may be attempted. In addition, caregiver management is essential, because as with those of Alzheimer’s dementia patients, they also carry a significant psychosocial stress.]

Hypertension and nephrology

[Hungarian virus research and NASA – nephrology aspects]

RADÓ János

[After the occurrence of varicella viruses remain in a latent condition in the ganglions, but could be reactivated from here causing the disease of herpes zoster. In the years of 1960, we described a herpes zoster „house epidemic” where only the steroid treated patients were infected. Varicella zoster virus was identified by virological methods. Also in a steroid treated patient fatal meningoencephalitis was caused by the generalized herpes zoster. The VZ infection was obviously potentiated by the steroid. Our publications about the interaction between the VZ virus and steroid treatment was echoed – among others – by an editorial of four leading medical journals. Investigators of a NASA medical group also cited our articles. They found during and after spaceflight that in the astronauts symptomless reactivation of the VZ virus, EBV and CMV occurred which was contributed to the stress induced hypercortisolemia. Today we see more worries in the prognosis and outlook in certain cases of the herpes zoster than before. One reason of that is the high number of newly recognized complications. Recently also several new pathway of pathomechanisms has been explored, which led to serious risks. In addition, it turned out that in certain disorders as the artheritis temporalis, where today antivirus antibiotic is the first choice drug, instead of steroid administered alone in the past, inducing further progression in the basic disease and sometimes fatal complications when given too long. Nephrological patients are at special risk in the presence of chronic renal disease, high age and associated diabetes mellitus. The risk may even increase after an otherwise successful renal transplantation in response to the administration of steroids and other compounds. Fortunately in the meantime a vaccine was developed against the VZ virus, studied in large populations and found to be very effective. It probably will be a benediction to the old people with chronic renal disease, after transplantation as well as in others suffering from high risk diseases.]