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 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.

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.

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.

[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 treatment of pseudoaneurysms with flow diverters after malignant otitis externa.

Background We report a case of bilateral malignant otitis externa complicated with bilateral petrous internal carotid artery pseudoaneurysms and their successful treatment with a flow diverter. Case report A 68-year-old woman with serious complications of type II diabetes mellitus had malignant otitis externa on the right side. She was treated with combined antibiotic therapy and underwent mastoidectomy for mastoiditis. She presented at our hospital with acute hemorrhage from the right external auditory canal. The emergency computed tomography (CT) angiography revealed a multiobulated pseudoaneurysm at the petrous segment of the right internal carotid artery. The pseudoaneurysm was treated with a 5 × 40-mm Surpass flow diverter. Three months later, she developed a malignant external otitis on the left side. As the infection progressed, a left-sided mastoiditis, a brain abscess, and a pseudoaneurysm at the petrous segment of the left internal carotid artery developed. The pseudoaneurysm caused bleeding from the left ear, and was treated with a 5 × 50-mm Surpass flow diverter. No recurrent bleeding was observed. Four months later, a follow-up angiography showed complete occlusion of the pseudoaneurysm on the left side, but a residual aneurysm could be detected on the right side. One year after the first intervention, the follow-up CT and magnetic resonance angiography revealed the complete occlusion of the aneurysms bilaterally. Conclusion The use of a flow diverter appears to be an efficient and safe method to occlude carotid pseudoaneurysms even in an inflammatory milieu.

Aortic arch and common carotid artery plaques with soft components pose a substantial risk of cerebral embolization during carotid stenting.

OBJECTIVES: A higher rate of embolization is considered a disadvantage of carotid stenting (CAS), when compared with carotid endarterectomy. Plaques in the aortic arch (AA) and the common carotid artery (CCA) may be additional sources of embolization to stented internal carotid plaques during CAS. In this study, we aimed to investigate the relationship between these plaques and intracerebral embolization. METHODS: We analyzed the occurrence and composition of plaques in the AA and CCA by computed tomography angiography (CTA) in 101 consecutive cases of CAS. Cases of peri-procedural embolization were detected on diffusion-weighted imaging as lesions demonstrating diffusion restriction. We applied the χ(2) and Fisher's exact tests, as well as logistic regression models. RESULTS: The occurrence of plaques in the AA and CCA was significantly related to the appearance of new diffusion-weighted imaging lesions (p = 0.013 and p = 0.004, respectively). Patients with soft plaques in the AA or CCA had a significantly higher risk of embolization than those without plaques (p = 0.012 and p = 0.006, respectively). In contrast, homogeneously calcified plaques did not pose significantly higher risks. CONCLUSIONS: Soft plaques in the AA and CCA result in a substantial risk of embolization during CAS. Use of a CTA examination of the AA and the CCA in patients with carotid stenosis may help to select lower-risk patients for CAS.

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.

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.

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.

White matter microstructural alterations in migraine: a diffusion-weighted MRI study.

Migraine is a common and disabling neurological disease. The pathomechanism that underlies the disorder is not entirely understood, and reliable biomarkers are missing. In the current analysis we looked for microstructural alterations of the brain white matter in migraine patients by means of diffusion-weighted magnetic resonance imaging. The measurements were carried out with a novel approach based on fine-tuned nonlinear registration and nonparametric permutation test in an alignment-invariant tract representation (Tract-Based Spatial Statistics). We found reduced fractional anisotropy in the right frontal white matter cluster of migraine patients. In the same region we also found increased mean diffusivity and increased radial diffusivity. The probabilistic tractography showed connection of this cluster to other parts of the pain network (orbitofrontal cortex, insula, thalamus, dorsal midbrain). We speculate that these findings reflect maladaptive plastic changes or white matter disintegration.

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.

[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.

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.