[Radiological forms of cerebral amyloid angiopathy]. / A cerebralis amyloid angiopathia radiológiai megjelenési formái.

Cerebral amyloid angiopathy (CAA) is most commonly recognized by ß-amyloid deposition in the small and medium sized vessels of the brain. The 71-year-old female presented with a sudden onset of vertigo and headache. By native computer tomography (CT) examination we found cerebral atrophy and the sign of chronic vascular injury. The complaints of the patient worsened, thus magnetic resonance imaging (MRI) was performed. The MRI scan revealed a bleeding transformation of an ischemic lesion in the right occipital region. On the susceptibility weighted scans we could observe old microbleedings, thus we suspected CAA. The patient later re-presented at the clinic with a sudden onset of right sided hemiplegia. We performed a native CT scan which identified cerebral hemorrhage in atypical position confirming our diagnosis. CAA has many radiological presentations. The most important is the lobar cerebral hemorrhage. On the susceptibility weighted MRI scans, we could identify the microbleeds and the superficial siderosis by the deposition of the hemosiderin. The subarachnoid hemorrhage is a sign of bad outcome. In the white matter, we could detect the change of the related inflammation and the leukoaraiosis. Further, two not specific abnormalities can be important as well, such as extended ischemic lesions and perivascular space dilatation. CAA has various appearances on MRI. Repeated vascular events and dementia in old age patients draw attention to its presence. The correct diagnosis can be made with the right interpretation of the patient's complaints in combination with the radiological abnormalities. Orv Hetil. 2018; 159(11): 439-444.

[The role of MRI in measuring the effectivity of disease modifying treatments I]. / Az MRI helye a sclerosis multiplex kezelés hatékonyságának megítélésében I.: mérési markerek.

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.

[The role of MRI in measuring the effectivity of disease modifying treatments II]. / Az MRI helye a sclerosis multiplex kezelés hatékonyságának megítélésében II.: mérési protokollok.

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.

WATERSHED INFARCTION IN HYPEREOSINOPHILIC SYNDROME: A DIAGNOSTIC DILEMMA IN FIP1L1-PDGFR ALPHA-ASSOCIATED MYELOID NEOPLASM.

INTRODUCTION: The FIP1L1-PDGFR alpha-positive, hypereosinophilic syndrome (HES) is a new category of hematological entities. Various clinical symptoms may occur, with no specific characteristics in either the clinical picture or the neuroimaging findings, and this may give rise to a diagnostic dilemma. A report on a long follow-up period (10 years) in a case of HES that presented with neuropsychiatric symptoms appears to be unique. Besides the complexity of the diagnostic process, the successful treatment is discussed. CASE REPORT: The HES was diagnosed in a male patient at the age of 33 years, with involvement of the central nervous system and the myocardium. After the onset of the clinical signs, the MRI indicated bilateral cerebral and cerebellar cortico-subcortical lesions involving the watershed areas, mainly in the parieto-occipital regions. High-dose intravenous steroid (methylprednisolone 500 mg/day) alleviated the neurological symptoms within a few weeks, and the administration of imatinib (200 mg/day) resulted in an impressive regression of the hypereosinophilia and splenomegaly within 6 weeks. During the follow-up, the patient has continued to receive imatinib. The molecular remission has persisted, no new complaints have developed and the condition of the patient has remained stable. CONCLUSION: The timely recognition of the HES and identification of the disease subtype which led to the administration of imatinib may be the key to successful treatment. The long stable follow-up period gives rise to a new dilemma in the treatment of the HES in these special cases: for how long should a patient receive a tyrosine kinase inhibitor, and may the treatment be suspended?

HYPERGLYCAEMIC HEMIBALLISMUS: IMPLICATIONS FROM CONNECTIVITY ANALYSIS FOR COGNITIVE IMPAIRMENTS.

Hyperglycaemia induced movement disorders, such as hemiballism are rare disorders. The syndrome is characterised by the triad of hemiballism, contralateral T1-hyperintense striatal lesion and non-ketotic hyperglycaemia. Here we report a patient with untreated diabetes presenting with acute onset of hemiballism. MRI revealed T1 hyperintensity of the head of the caudate nucleus and the anterior putamen. The patient also had acantocytosis. Based on the detailed examination of the neuroradiological results and earlier findings we will discuss the pathomechanism. Based on previous findings microhemorrhages, extensive mineralisation, gemistocytic astrocytosis might play a role in the development of the imaging signs. The connectivity pattern of the striatal lesion showed extensive connections to the frontal cortex. In coexistence with that the most severe impairment was found on the phonemic verbal fluency task measuring frontal executive functions.

[Neurovascular compression of the medulla oblongata: a rare cause of secondary hypertension]. / Nyúltveloi neurovascularis kompresszió. A szekunder hypertonia ritka oka.

Compression of the rostral ventrolateral medulla oblongata is one of the rarely identified causes of refractory hypertension. In patients with severe, intractable hypertension caused by neurovascular compression, neurosurgical decompression should be considered. The authors present the history of a 20-year-old man with severe hypertension. After excluding other possible causes of secondary hypertension, the underlying cause of his high blood pressure was identified by the demonstration of neurovascular compression shown by magnetic resonance angiography and an increased sympathetic activity (sinus tachycardia) during the high blood pressure episodes. Due to frequent episodes of hypertensive crises, surgical decompression was recommended, which was performed with the placement of an isograft between the brainstem and the left vertebral artery. In the first six months after the operation, the patient's blood pressure could be kept in the normal range with significantly reduced doses of antihypertensive medication. Repeat magnetic resonance angiography confirmed the cessation of brainstem compression. After six months, increased blood pressure returned periodically, but to a smaller extent and less frequently. Based on the result of magnetic resonance angiography performed 22 months after surgery, re-operation was considered. According to previous literature data long-term success can only be achieved in one third of patients after surgical decompression. In the majority of patients surgery results in a significant decrease of blood pressure, an increased efficiency of antihypertensive therapy as well as a decrease in the frequency of highly increased blood pressure episodes. Thus, a significant improvement of the patient's quality of life can be achieved. The case of this patient is an example of the latter scenario.

Observations of changes of blood pressure before and after neurosurgical decompression in hypertensive patients with different types of neurovascular compression of brain stem.

AIMS: The neurovascular pulsatile compression of the rostral ventrolateral medulla can be divided into different subtypes. The posterior inferior cerebellar artery and/or vertebral artery can compress either the rostral ventrolateral medulla or the cranial nerves IX and X or both and on left, right or both sides. METHODS: It was retrospectively investigated whether the types of neurovascular compression can influence blood pressure values. Data from 13 resistant hypertensive patients after decompression were investigated. RESULTS: Six patients had 2 compressions, two had only medulla compression, four had only nerve compression on the left side and one had 2 compressions on both sides. There was no correlation between the types of compression and the levels of blood pressure, either before or after the decompression. Both, systolic and diastolic blood pressures and pulse pressure also decreased in all cases after the decompression but the change was significant only in the group with 2 compressions on the left side. CONCLUSION: According to our data, in a severe hypertension not responding to conventional antihypertensive therapy, the surgical decompression of the brain stem independently of the types of neurovascular compression could guarantee a decrease of blood pressure and improved sensitivity to antihypertensive medication.

[Vascular compression syndromes of the cranial nerves]. / Az agyidegek vascularis kompressziós szindrómál.

The blood vessels which are running nearby the cranial nerves and the brainstem can be elongated; curves and loops of the vessels may develop mostly due to the degenerative alterations of ageing and these vessels can compress the surrounding neural elements. The authors report a review of vascular compression syndromes based on the literature and their own experience. The typical clinical symptoms of the syndromes subserving the proper diagnosis, the pathomechanism, the significance of imaging especially the magnetic resonance angiography, the experience with the surgical technique of microvascular decompression which is the only causal treatment of the syndromes are discussed. In cases of non-responsible medical treatment the microvascular decompression should be the eligible treatment in certain syndromes (trigeminal and glossopharyngeal neuralgia, hemifacial spasm) for it is a highly effective and low risk method.

Size of the Carotid Body in Patients with Cardiovascular and Respiratory Diseases Measured by Computed Tomography Angiography: A Case-Control Study.

OBJECTIVES: Carotid bodies (CBs) play an important role in regulating sympathetic nervous system activity. Thus, they are likely to be enlarged in patients with certain cardiovascular and respiratory diseases. The aim of this case-control study was to verify this hypothesis using computed tomography angiography (CTA). METHODS: We retrospectively analysed 141 CTAs including 16 controls, 96 patients with only hypertension (HT), 12 with HT and previous acute myocardial infarction (AMI), 9 with HT and heart failure (HF), and 8 with HT and chronic obstructive pulmonary disease (COPD). We assessed the data using analysis of variance, with p < 0.05 indicating significance. RESULTS: CB average areas in the controls were 2.31 mm2 (right side (RS)) vs. 2.34 mm2 (left side (LS)). CB size was significantly enlarged in patients with HT: 3.07 mm2 (RS) (p=0.019) vs. 2.91 mm2 (LS) (p=0.002). If AMI (RS: 3.5 mm2; LS: 3.44 mm2) or HF (RS: 4.01 mm2; LS: 4.55 mm2) was associated with HT, the CB size was even more enlarged. COPD did not affect CB size (RS: 2.40 mm2; LS: 2.29 mm2). CONCLUSIONS: Our data showed that certain diseases with increased activity of the sympathetic nervous system were associated with significantly enlarged CBs.

The correlation between the cardiovascular instability and the size of the developed ischaemic lesions in patients who underwent carotid stenting.

AIMS: In this study we investigated the relationship between cardiovascular instability and the size of the developed ischaemic lesions during carotid stent implantation by diffusion-weighted (DWI) magnetic resonance (MR) examination. MATERIAL AND METHODS: We retrospectively analyzed anaesthesia reports and follow-up MR examinations after stent implantation of 192 patients in a 3-year period. Nineteen aspects of cardiovascular status were analyzed. We registered the duration of the intervention, low and high blood pressure (BP) values during anaesthesia and heart rates. The fluctuations of BP and heart rate and the time of their compensations after the stent expansion were also recorded. Values were compared with the number and the size of ischaemic lesions on DWI scans. We used Spearman and Kendall rank correlations and Welch's tests for statistical analysis. Values of p ≤ 0.05 were considered as statistically significant. RESULTS: Decreased heart rate significantly correlated with the number (p = 0.0123) and size (p = 0.00323) of ischaemic lesions during stent expansion. Other cardiovascular parameters did not show any significant correlations. CONCLUSIONS: Our results indicate that only heart rate attenuation affects the size of ischaemic lesions; thus the prevention of bradycardia is highly important.

The use of covered stents for the endovascular treatment of extracranial internal carotid artery stenosis: a prospective study with a 5-year follow-up.

OBJECTIVES: To evaluate the safety and feasibility of the use of covered stents for the treatment of extracranial carotid artery stenosis caused by highly embologenic plaques, and to study the long-term outcome of patients receiving such covered stents. METHODS: Between 2002 and 2007, 46 patients (63% symptomatic, 78.3% male, 67 +/- 8.6 years old) with internal carotid artery stenosis caused by embologenic plaques or restenosis were treated with self-expanding covered stents (Symbiot, Boston Scientific). Pre-dilatation or protecting devices were not used. Post-dilatation was applied in every patient. Each patient was followed long-term. The outcome measures were the occurrence of neurological events, and the development of in-stent restenosis, as detected by clinical examination and duplex ultrasound. RESULTS: The technical success rate of stenting was 100%. There were no neurological complications in the peri-procedural period. The mean follow-up period was 34.3 +/- 27.7 months (the rate of patients lost to follow-up was 15.2%) during which no stroke or stroke-related deaths occurred. Restenosis was detected in 3 patients (6.5%). CONCLUSION: Covered stents provide efficient peri- and post-procedural protection against neurological complications due to embolisation from high-risk plaques during carotid artery stenting. Restenosis of covered stents appears to be infrequent during long-term follow-up.

[Non-obstructive hydrocephalus internus with a rare pathogenesis--mucormycosis]. / Nonobstruktív hydrocephalust okozó ritka kórkip-mucormycosis.

The case of a 9-year-old boy is presented in this article who developed a rare fungal infection of central nervous system. The histopathologic examination has revealed mucormycosis. The diagnosis wasn't confirmed microbiologically as the culture and PCR were negativ. After the iv administered Amphotericin B lipid complex the MR images of the brain have improved. The mucormycosis classically develops in immunodeficient patients and presents an acute, fulminant, mostly lethal infection. This case is very unusual, because the chronic, isolated CNS mucormycosis has slowly developed in immuncompetent patient and only one symptom was the long existing headache. The aim of this paper is reporting the case history and to find out the possible way of infection.

99-mTc-HMPAO single photon emission computed tomography examinations in genetically determined neurometabolic disorders.

UNLABELLED: The aim of our study was to determine regional cerebral blood flow (rCBF) abnormalities in different types of enzymopathies. PATIENTS AND METHODS: Among the patients with genetically determined enzymopathies 3 patients had aminoacidopathies, and 11 had different types of encephalopathies, from which 10 had mitochondrial encephalomyopathy (MEMP), and 1 patient had hyperuricaemic encephalopathy. Besides the mentioned 14 patients, 1 had ceroid lipofuscinosis and another patient had tuberous sclerosis. The further distribution of the MEMP patients' group was the following--5 patients had MEMP with lactic acidosis, 5 had Leigh's disease (subacute necrotizing encephalopathy), from which 1 had cytochrome-c-oxidase deficiency (COX). Additionally in all patients were performed cerebral MRI and SPECT examination 10 min. after intravenous administration of 20 Mbq/kg 99 mTc-HMPAO. RESULTS: Fourteen out of 16 SPECT findings were pathologic, showing decreased focal frontal/temporal/temporoparietal cerebral blood perfusion. Aminoacidopathic group--all the 3 patients revealed pathologic signs from the aminoacidopathic patients' group. Among them the ornithine transcarbamylase (OTC) heterozygous female patient with left-sided hemiparesis caused by hyperammonemic stroke at 10 month-age, showed right sided temporoparietal, occipital and left frontal hypoperfusion, nearly 6 years after the cerebral vascular attack. This finding might be resulted because of diaschisis. Mitochondrial encephalo-myopathic (MEMP) group--all the four patients with MEMP and lactic acidosis showed focal hypoperfusion in the temporal region, while the perfusion was normal in the COX deficient patient and in 2 Leigh's disease (subacute necrotizing encephalopathy) patients. In the remaining 1 Leigh's patient frontotemporal hypoperfusion was found. In all patients there were non specific structural abnormalities detected by MRI: cortical and subcortical atrophy, and scattered demyelination foci. In the case of ceroid lipofuscinosis the MRI showed cerebral atrophy and cerebellar hypoplasia, and the SPECT showed right frontal and occipital hypoperfusion, bilateral parietal physiological riping process. The patient with tuberous sclerosis showed bilateral temporo-occipital hypoperfusion. CONCLUSION: (1) SPECT images demonstrated hypoperfusion rCBF changes in 14 out of all 16 patients. (2) Regional cerebral/cerebellar hypoperfusion was detected by SPECT in mitochondrial encephalomyopathies, with lactate acidosis and aminoacidopathies giving high informative value about the cerebral perfusion.

Myelination disturbance in a patient with hyperuricemia and hyperserotoninemia combined with 18q deletion syndrome.

We previously reported a male patient with an 18q21.3 deletion, hyperuricemia and typical symptoms of the Lesch-Nyhan syndrome who lacked hypoxanthine-guanine-phosphoribosyl-transferase (HGPRT) deficiency. The patient developed progressive peripheral neuropathy in additon to his profound mental retardation and self-injurious behavior. At the age of 23 years MR imaging revealed globally delayed myelination with relative sparing of the corpus callosum and frontal lobes. They were focal hyperintensities suggestive of gliosis. Multimodality evoked potentials found evidence of impaired central and peripheral conduction. Single photon emission computed tomographic (SPECT) imaging demonstrated left frontal hyperperfusion and under it a temporoparietal hypoperfusion.

Epstein-Barr virus encephalitis mimicking clinical and electroencephalographic characteristics of herpes simplex encephalitis.

A rare case of EBV encephalitis initially diagnosed as Herpes simplex infection is presented to highlight the importance of EBV specific intrathecal ELISA and liquor PCR based differential diagnosis when Herpes simplex encephalitis specific clinical symptoms, neuroimaging signs and electroencephalographic features are present. The case report also suggests that acyclovir treatment might be beneficial for the long term outcome in adult EBV encephalitis patients.

Neurovascular pulsatile compression and neurosurgical decompression of the rostral ventrolateral medulla in medically resistant hypertensive patients.

BACKGROUND/AIMS: In cases of severe primary hypertension not responding to conventional medical therapy, neurovascular pulsatile compression of the rostral ventrolateral medulla on the left side may be considered as an etiological factor in the hypertension. Through neurosurgical decompression, the blood pressure can be reduced in these cases, and the conventional medication can also become more effective. METHODS: The authors retrospectively analysed the changes in the blood pressure and therapy of patients with or without neurosurgical decompression over a 2-year period. The 2-year data were available for 9 operated and 7 non-operated patients with neurovascular compression. The data of control examinations performed 1, 3, 6, 12 and 24 months after the intervention (or after MR-angiography in the non-operated cases) were analysed. RESULTS: After the decompression, both the systolic and diastolic blood pressure decreased significantly and permanently in all cases, and there was an improved response to the medication. In the non-operated group, the blood pressure did not change significantly during the 2 years. CONCLUSION: In severe hypertension that does not respond to conventional therapy, neurosurgical decompression of the brain stem on the left side can guarantee a long-lasting blood pressure reduction and a better response to antihypertensive medication.

Miyazaki Syndrome due to Ventriculoperitoneal Shunt Treatment.

BACKGROUND: The signs and pathomechanism of Miyazaki syndrome is presented through the case of a young female patient. CASE DESCRIPTION: The 33-year-old patient had undergone placement of a ventriculoperitoneal shunt with a pressure-adjustable valve for communicating hydrocephalus years before presenting to our department with the complaints of constant headache and unsteady gait. On the basis of the clinical picture and her history, plain and contrast-enhanced cranial and whole spine magnetic resonance imaging and magnetic resonance angiography examinations were performed, with the scans revealing signs indicative of cerebrospinal fluid hypotension typical of Miyazaki syndrome. CONCLUSION: The article discusses the available literature suggesting the underlying cause in such cases to be the dysfunction of the Starling resistor mechanism due to an improperly adjusted ventriculoperitoneal shunt, which results in excessive cerebrospinal fluid loss accompanied by consequent cerebral venous overflow with vertebral venous engorgement and compressive cervical myelopathy.

Pediatric multiple sclerosis and fulminant disease course: Features and approaches to treatment - A case report and review of the literature.

Multiple sclerosis (MS) is the autoimmune, neurodegenerative disease of the central nervous system (CNS). Typically, it affects the young adult population, however, up to 10% of the cases, it can develop in childhood. Atypical manifestations, such as the tumefactive variant (tMS) or acute disseminated encephalomyelitis (ADEM), especially coupled with fulminant disease course, are even more rare and pose a considerable differential diagnostic and therapeutic challenge. Recently, the therapeutic strategy on the use of disease modifying therapies (DMTs) in MS has shifted to the direction of a more individualized approach, that takes the personal differences heavily into account, in particular regard to the activity and prognosis of the disease. Despite this change has only been applied to adults yet, it is plausible to predict, that it will soon be applied to pediatric patients as well, particularly, as several randomized studies are under way concerning DMTs in pediatric populations. To our best knowledge, we are the first to report a successful natalizumab treatment of pediatric fulminant tMS, in case of a 13.5 years old girl. We feel that this report demonstrates the need of early and adequate treatment in such an aggressive case, because it can reverse the course of a possibly fatal disease.

Possible role of the basal ganglia in the generation of the N30 potential of the median nerve somatosensory evoked potentials.

BACKGROUND AND PURPOSE: The origin and afferentation of the frontal N30 component of the median nerve somatosensory evoked potentials (SEPs) have not yet been fully elucidated. The aim of this study was to assess the possible selective impairment of the N30 component in patients with lacunar infarcts of the basal ganglia as compared to patients with lacunar infarctions sparing the basal ganglia and to a group of healthy subjects. METHODS: Median nerve SEPs were measured in ten patients with lacunar infarctions of the brain (but no cortical atrophy or leukoaraiosis) and 13 healthy volunteers. Four patients had lacunar infarctions affecting the basal ganglia and 6 patients had lesions affecting other structures. RESULTS: In two patients with lesions affecting the head of the caudate nucleus, there was no identifiable N30 component on the affected side. In one patient with bilateral lesions of the globus pallidus, the amplitude of the N30 component was significantly reduced. In one patient with lesion of the tail of the caudate nucleus, the N30 component was unaffected. The amplitude of the N30 component was also reduced in two patients with frontal subcortical white matter lesions. In all the other subjects, we recorded normal N30 components on both sides. CONCLUSION: Our results further support the importance of the basal ganglia, especially the head of the caudate nucleus in the generation of the N30 component of the median nerve SEPs.