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Multiple pathologies may underlie corticobasal syndrome (CBS), including Alzheimer's disease (AD). Typical amnestic AD is characterized by early selective hippocampal atrophy. The profile of hippocampal atrophy in AD patients presenting as CBS (CBS-AD), compared to CBS patients of non-AD pathologies (CBS-nAD) and amnestic AD patients, has not been studied.To compare hippocampal subfield atrophy patterns between CBS-AD, CBS-nAD, typical amnestic AD patients, and control subjects.Automated hippocampal subfield volumetry was performed via the hippocampal subfield segmentation pipeline of Freesurfer 6.0 on 3D T1-weighted images. CBS patients were classified as CBS-AD or CBS-nAD based on CSF AD biomarkers by applying the AT(N) classification system. Mean volumes of nine hippocampal subfields, head-body-tail segments, total hippocampus, and entorhinal and parahippocampal gyrus cortical thickness were measured.Eighty-three subjects were included (CBS-AD: n = 14; CBS-nAD: n = 17; amnestic AD: n = 29; controls: n = 23). CBS-AD patients had greater whole hippocampal and hippocampal subfield atrophy compared to CBS-nAD. CBS-AD and amnestic AD patients did not differ in subfield volumes. CBS-nAD did not exhibit hippocampal atrophy compared to controls, with the exception of fimbria. (Cohen's d = 1.27; p = 0.038). Presubiculum (Cohen's d = 1.00; p = 0.002) and hippocampal body (Cohen's d = 0.95; p = 0.001) volumes exhibited the greatest differences between CBS-AD and CBS-nAD. Hippocampal subfield volume provided combined sensitivity and specificity 80% for the discrimination of CBS-AD from CBS-nAD.CBS-AD and amnestic AD patients exhibit comparable, and significantly greater hippocampal atrophy compared to CBS-nAD patients. Hippocampal subfield volumetry in CBS is indicative of an AD underlying pathology.

Pediatric-onset multiple sclerosis (MS, POMS) accounts for 3-5% of all MS cases and is characterized by a highly inflammatory profile, often warranting treatment with high-efficacy agents. Our aim is to present real-world data of a series of 18 Hellenic POMS patients treated with natalizumab (NTZ) either as adolescents or as adults, after high disease activity has efficiently subsided. Clinical and imaging/laboratory data from 18 POMS patients who have received at least one NTZ infusion were selected in this single-center retrospective observational study. Human leukocyte antigen (HLA) genotyping was performed with standard low-resolution sequence-specific oligonucleotide techniques. Eighteen patients with a mean age of disease onset of 15.3 ± 2.4 years were treated with NTZ with a mean of 51.7 ± 46.4 infusions, 6 as adolescents and 12 as adults. 22.2% were treatment naïve. At the end of the observational period, patients of both groups remained relapse-free, with no radiological activity and significantly reduced disability accumulation. No evidence of disease activity (NEDA)-3 status was achieved in 66.7% of all patients, 58.3% in the adult-treated, and 83.3% in the adolescent-treated POMS patients. NTZ was generally well tolerated. Only 5 adverse events were observed, in 3 patients who were carriers of the HLA-DRB1*15 (HLA-DRB1*15/HLA-DRB1*11 and HLA-DRB1*15/HLA-DRB1*13 genotypes), 1 homozygous for the HLA-DRB1*03 allele and 1 heterozygous for HLA-DRB1*04 and HLA-DRB1*16 alleles. NTZ is highly efficacious and mostly safe for POMS patients with high disease activity in all age groups. The role of immunogenetics in personalized patient evaluation and treatment needs to be further investigated.

Neurocysticercosis (NCC), the most common central nervous system (CNS) parasitic infection among the immunocompetent population can imitate every clinical feature of brain-diseases accurately, drawing attention away from the real culprit and delaying the proper treatment. There are two types of NCC, the parenchymal and the extraparenchymal form. The extraparenchymal NCC include the ventricular cysticercosis, the subarachnoid cysts including giant cysts or racemose cysticercosis with chronic meningitis, the spinal (intra- or extramedullary) cysticercosis and the ophthalmic cysticercosis. It is estimated that about 30% of epilepsy cases in endemic countries are due to NCC and especially the racemose NCC is more aggressive and associated with higher mortality rates. There is a significant heterogeneity in clinical phenotypes, regarding the racemose NCC, which depends on the parasite load and evolutionary stage in association with its location in CNS and the host’s immune response. Crucial for the management of the racemose NCC is the early recognition of the symptoms and the swift initiation of antiparasitic therapy with anti-inflammatory agents in combination with the shunt-insertion in cases of obstructive hydrocephalus. In view of the former considerations we conducted a narrative literature review on racemose NCC and described the diagnostic challenges of a relevant case that we had evaluated in our Department of Neurology.

Background: Cortical demyelination and meningeal inflammation have been detected neuropathologically in multiple sclerosis (MS) and recently in myelin oligodendrocyte glycoprotein antibody disease (MOGAD). Objectives: To assess in vivo cortical and leptomeningeal involvement in MOGAD. Methods: We prospectively evaluated 11 MOGAD and 12 relapsing-remitting MS (RRMS) patients combining three-dimensional fluid-attenuated inversion recovery (3D-FLAIR) and 3D-T1-weighted (3D-T1w) sequences at 3-Tesla magnetic resonance imaging (MRI). Leptomeningeal contrast enhancement (LMCE) was assessed on 3D-FLAIR post-gadolinium (3D-FLAIRGd). Cerebral cortical lesions (CCLs) were classified as either intracortical–subpial (IC–SP) or leukocortical (LC). Results: CCLs were present in 8/11 MOGAD and 12/12 RRMS patients, with the number of CCLs being significantly lower in MOGAD (median (interquartile range (IQR)) 3 (0.5–4) vs 12 (4.75–19), p = 0.0032). In MOGAD, IC–SP lesions were slightly more prevalent than LC lesions (2 (0–2.5) vs 1 (0–2), p = 0.6579); whereas in RRMS, IC–SP lesions were less prevalent than LC lesions (3.5 (2.75–5.5) vs 9 (2–12.75), p = 0.27). LMCE was observed in 3/11 MOGAD and 1/12 RRMS patients; MOGAD with LMCE showed an increased median number of CCLs compared with MOGAD without LMCE (8 (4–9) vs 2.5 (0.75–3.25), p = 0.34). No correlation was observed between MOGAD MRI findings and (a) MOGAD duration, (b) serum MOG-immunoglobulin G1 titers, and (c) oligoclonal band presence. Conclusion: We described cortical lesion topography and detected for the first time LMCE using 3D-FLAIRGd sequences in MOGAD patients.

Clinical Practice Points • Progressive multifocal leukoencephalopathy (PML) is a rare but fatal demyelinating disease caused by John Cunningham polyomavirus, mainly affecting immunosuppressed patients. It sporadically complicates hematological malignancies and has been reported after monoclonal antibody treatment, predominantly rituximab. In recent years, obinutuzumab, a third-generation type II anti-CD20+ monoclonal antibody, has been widely used for chronic lymphocytic leukemia and follicular lymphoma. • We present the case of a patient with relapsing follicular lymphoma who developed PML after obinutuzumab therapy and is, to our knowledge, the first case to be thoroughly presented in the literature. • Although PML is very rare in everyday clinical practice, this report highlights the importance of suspecting PML in any lymphoma patient undergoing novel treatments and presenting focal neurologic deficits, in an effort to avoid unnecessary invasive procedures and delay in diagnosis.

Background Parkinsonian symptoms are common adverse effects of antipsychotics. Older adults are particularly vulnerable to drug-induced parkinsonism. Nonetheless, parkinsonian symptoms in seniors treated with antipsychotics cannot be straightforwardly attributed to antipsychotic medication. A comprehensive diagnostic workup is necessary in many cases in order to shed light on the cause of such symptoms in this patient population. Case series Eight cases of hospitalized depressed older adults with parkinsonian symptoms, who were treated for at least one year with antipsychotics, are reported. Based on neurological consultation, structural brain imaging and Ioflupane (I-123) dopamine transporter (DAT) single photon emission computerized tomography (SPECT), Parkinson’s disease was diagnosed in one case, idiopathic tremor in another, vascular parkinsonism in another one, while in another individual parkinsonian symptoms persisted at 12-month post-discharge follow-up even though his/her symptoms were classified as drug-induced on discharge. In four patients, parkinsonian symptoms were definitely drug-induced and no movement disturbances were reported at follow-up. Conclusions Differences in the cause and outcome of parkinsonian symptoms in seniors treated with antipsychotics merit systematic and in-depth study considering the therapeutic and prognostic implications of an accurate detection of the cause of such symptoms. Familiarizing clinical psychiatrists with these differences could pave the way towards approaching seniors with severe, atypical and/or persistent parkinsonian symptoms in a more individualized diagnostic and therapeutic manner, and towards more cautious prescribing of antipsychotics in this age group.

Background and PurposeAutomated brain volume analysis from MRI images, is gaining an important role in computer-aided diagnosis. This study compares the volumes of brain segments that measured by two automated brain analysis software: NeuroQuant and volBrain. The aim is to investigate the quantitative and qualitative reliability of automated brain analysis.MethodsWe compared same brain segment volume measurements, from 45 patients, that calculated with both NeuroQuant and volBrain software. The statistical two-tailed unpaired t-test method used to determine if there is a significant difference on the same segment measurements performed from each software. Additionally, least square method used, to provide any evidence of linear correlation between them. In a case of extreme difference, manual volume measurement of a segment was performed, with JIM8 software.ResultsFrom the comparison of brain segments volumetric measurements made by NeuroQuant and volBrain, the differences for the Intracranial Cavity, Putamen, Thalamus and Amygdala considered extremely significant, the Whole Brain, Cerebellum, Hippocampus considered not significant, White Matter considered quite significant. In most cases strong linear correlation between the two software measurements found.ConclusionsThe unexpected significant differences found in some segments raised questions about the reliability of automated volume analysis as a quantitative tool. Strong linear correlation of the volumes presents that both software finally provides good qualitative information on brain structures, which is an important factor for computed aided diagnosis.

Background Baló’s Concentric Sclerosis (BCS) is a rare heterogeneous demyelinating disease with a variety of phenotypes on Magnetic Resonance Imaging (MRI). Existing literature lacks data especially on the therapeutic approach of the disease which we intended to elucidate by means of suggesting a new possible BCS classification and introducing different therapeutic concepts based on each BCS-subgroup characteristics. Methods We present a retrospective study of eight treated patients with BCS-type lesions, emphasizing on MRI characteristics and differences on therapeutic maneuvers. Results Data analysis showed: at disease onset the BCS-type lesion was tumefactive (size ≥2 cm) in 6 patients, with a mean size of 2.7 cm (± 0.80 SD); a coexistence of MS-like plaques on brain MRI was identified in 7 patients of our cohort. The mean age was 26.3 years (±7.3 SD) at disease onset and the mean follow-up period was 56.8 months (range 9–132 months). According to radiological characteristics and response to therapies, we further categorized them into 3 subgroups: a) Group-1; BCS with or without coexisting nonspecific white matter lesions; poor response to intravenous methylprednisolone (IVMP); treated with high doses of immunosuppressive agents (4 patients), b) Group-2; BCS with typical MS lesions; good response to IVMP; treated with MS-disease modifying therapies (2 patients), c) Group-3; BCS with typical MS lesions; poor response to IVMP; treated with rituximab (2 patients). Conclusions Our study introduces a new insight regarding the categorization of BCS into three subgroups depending on radiological features at onset and during the course of the disease, in combination with the response to different immunotherapies. Immunosuppressive agents such as cyclophosphamide are usually effective in BCS. However, therapeutic alternatives like anti-CD20 monoclonal antibodies or more classical disease-modifying MS therapies can be considered when BCS has also mixed lesions similar to MS. Future studies with a larger sample size are necessary to further establish these findings, thus leading to better treatment algorithms and improved clinical outcomes.

Atypical forms of demyelinating diseases with tumor-like lesions and aggressive course represent a diagnostic and therapeutic challenge for neurologists. Herein, we describe a 50-year-old woman presenting with subacute onset of left hemiparesis, memory difficulties and headache. Brain MRI revealed a tumefactive right frontal-parietal lesion with perilesional edema, mass effect and homogenous post-contrast enhancement, along with other small atypical lesions in the white-matter. Brain biopsy of cerebral lesion ruled out lymphoma or any other neoplastic process and patient placed on corticosteroids with complete clinical/radiological remission. Two years after disease initiation, there was disease exacerbation with reappearance of the tumor-like mass. The patient initially responded to high doses of corticosteroids but soon became resistant. Plasma-exchange sessions were not able to limit disease burden. Resistance to therapeutic efforts led to a second biopsy that showed perivascular demyelination, predominantly consisting of macrophages, with a small number of T and B lymphocytes, and the presence of reactive astrocytes, typical of Creutzfeldt-Peters cells. The patient received high doses of cyclophosphamide with substantial clinical/radiological response but relapsed after 7-intensive cycles. She received 4-weekly doses of rituximab with disease exacerbation and brainstem involvement. She eventually died with complicated pneumonia. We present a very rare case of recurrent tumefactive demyelinating lesions, with atypical tumor-like characteristics, with initial response to corticosteroids and cyclophosphamide, but subsequent development of drug-resistance and unexpected exacerbation upon rituximab administration. Our clinical case raises therapeutic dilemmas and points to the need for immediate and appropriate immunosuppression in difficult to treat tumefactive CNS lesions with Marburg-like features.

Considering the number of multiple sclerosis (MS) patients referred for clinical spinal cord imaging, the optimization of imaging protocols plays a crucial role. We aimed to evaluate the use of proton density (PD) turbo spin-echo (TSE) with spectral attenuated inversion recovery (SPAIR) fat suppression and compare it with the currently recommended T2-TSE-SPAIR in sagittal plane in cervical spinal cord imaging. In this study 35 MS patients with clinically suspected or known spinal cord lesions were scanned on a 3.0T magnetic resonance imaging (MRI) system. In addition to the routine protocol, PD-TSE-SPAIR sequences were obtained to quantitatively and qualitatively evaluate lesion detectability and image quality compared to T2-TSE-SPAIR sequences. Quantitative analysis was based on measurements of lesion-to-cord contrast ratio (LCCR), lesion contrast-to-noise ratio (LCNR) and lesion dimensions and the qualitative analysis on ranking with a predetermined score scale. The presence of lesions in these sequences was verified in axial T2 multi-echo gradient echo images. In quantitative analysis, the lesions on PD-TSE-SPAIR had statistically significantly higher contrast (p < 0.05), according to the statistical test of LCCR, LCNR calculated contrast and measured lesion dimensions. Qualitative analyses were congruent with quantitative results; the median rank of PD-TSE-SPAIR was significantly higher than T2-TSE-SPAIR (p < 0.05). Of the 34 detected lesions 9 (26%) were not visualized in T2-TSE-SPAIR sequence. Considering its superiority in contrast ratios and lesion dimensions when compared to T2-TSE-SPAIR in both qualitative and quantitative analyses, we therefore recommend PD-TSE-SPAIR as a pivotal sequence to evaluate demyelinating spinal cord lesions at 3T.

Various MRI markers have been applied to support the diagnosis of progressive supranuclear palsy (PSP), such as midbrain diameter and surface, superior cerebellar peduncle (SCP) width, midbrain to pons (m/p) diameter and surface ratio and the Magnetic Resonance Parkinsonism Index (MRPI). These markers provide excellent diagnostic accuracy in discriminating Richardson's syndrome from other causes of Parkinsonism. Idiopathic normal pressure hydrocephalus (iNPH) may mimic Richardson's syndrome, particularly in cases of subtle opthalmokinetic abnormalities. The aim of this study was to compare these MRI markers in PSP and iNPH and examine their diagnostic accuracy.Forty-three patients with probable PSP, 17 patients with iNPH, and 29 controls were included. Midbrain diameter and surface, SCP width, m/p diameter and surface ratio and the MRPI were recorded. The "hummingbird sign," "morning glory sign" and "mickey mouse sign" were also evaluated. Analysis of covariance, chi-squared test, and ROC curve analysis were used as appropriate.All MRI measurements differed significantly among the three study groups. Comparison of PSP and iNPH patients produced the following significant differences: midbrain diameter (P .0001), m/p diameter ratio (P .0001), SCP width (P = .050), and MRPI (P = .049). None of these markers produced combined high (80%) specificity and sensitivity. Qualitative MRI signs were specific, but lacked sensitivity.Midbrain morphology in iNPH may resemble that of PSP. Established MRI markers of midbrain and SCP atrophy cannot confidently differentiate PSP from iNPH. MRI markers do not provide combined high sensitivity and specificity for the differential diagnosis of PSP from iNPH.

IgG4-related disease (IgG4-RD) is a disorder with various clinical manifestations. Central nervous system (CNS) involvement is well recognized, with hypertrophic pachymeningitis and hypophysitis being the most common manifestations. Spinal cord involvement is an extremely rare manifestation. We present the first case of an IgG4-RD patient with spinal cord parenchymal disease and concurrent hypophysitis. We review also the current literature about CNS parenchymal involvement in the context of IgG4-RD. A young female presented with clinical symptoms of myelitis. Cervical spinal cord magnetic resonance imaging (MRI) displayed features of longitudinally extensive transverse myelitis (LETM). Brain MRI showed a small number of high-intensity lesions in the deep white matter and enlargement of hypophysis with homogeneous gadolinium enhancement (asymptomatic hypophysitis). Diagnostic workup revealed elevated IgG4 serum levels (146 mg/dL). Our patient fulfilled the organ-specific diagnostic criteria of IgG4-hypophysitis. Treatment with intravenous glucocorticoids led to rapid clinical response, and to the substantial resolution of imaging findings. Azathioprine was used as a maintenance treatment. One relapse occurred 2 years after the initial diagnosis and patient was re-treated with glucocorticoids. Three years after relapse, patient is in remission with azathioprine. We present the first case of myelitis with radiological features of LETM associated with increased IgG4 serum levels and the simultaneous presence of asymptomatic IgG4-related hypophysitis.

Gerstmann's syndrome is a syndrome caused by lesions of the dominant parietal lobe. It has historically been a subject of dispute as it is often accompanied by other neuropsychological deficits such as aphasia. Novel neuropsychological tests and neuroimaging techniques have lately changed the perspective allowing to decode a conjunction of symptoms like Gerstmann's. In our case, we present Diffusion Tensor Imaging Tractography findings of a patient who acutely exhibited Gerstmann's tetrad. Using advanced structural neuroimaging we tested a hypothesis that up until recently could only be challenged post mortem. Our study offers much needed patient neuroimaging data helping to establish Gerstmann's as a disconnection syndrome. More reports of Gerstmann's syndrome patients including high resolution tractography and fMRI data are needed.

Background The comorbidity between Multiple Sclerosis (MS) and Human Immunodeficiency Virus (HIV) infection is particularly rare. Only a few cases of comorbidity of Clinically Definite(CD)-MS and HIV have been documented worldwide, while the potential beneficial role of antiretroviral therapy regarding MS activity has long been an area of debate. Case report We present a 36-year old male, bearing a diagnosis of CD-MS for twelve years. He had been treated for ten years with interferon-beta-1b, when he voluntarily discontinued therapy, claiming clinical stability. One year later he was diagnosed positive for HIV and he started and continued only on efavirenz/emricitabine/tenofovir-disoproxil fumarate (ATRIPLA®), remaining relapse-free until today. Conclusion This fact, in combination with the unique pharmaceutical composition of the drug, which contains a component similar to a newly-approved agent for MS, dimethyl fumarate, prompted us to review the literature regarding this rare comorbidity and to suggest that the role of the antiretroviral therapy should be further explored in MS.

BACKGROUND AND PURPOSE: Differential diagnosis of multiple system atrophy, progressive supranuclear palsy, and corticobasal degeneration from Parkinson disease on clinical grounds is often difficult. MR imaging biomarkers could assist in a more accurate diagnosis. We examined the utility of MR imaging surface measurements (MR imaging planimetry) in the differential diagnosis of patients with parkinsonism. MATERIALS AND METHODS: Fifty-two patients with Parkinson-plus (progressive supranuclear palsy, n = 24; corticobasal degeneration, n = 9; multiple system atrophy, n = 19), 18 patients with Parkinson disease, and 15 healthy controls were included. Corpus callosum, midbrain, and pons surfaces; relevant indices; and the Magnetic Resonance Parkinsonism Index were calculated. Corpus callosum subsection analysis was performed, and the corpus callosum posteroanterior gradient was introduced. RESULTS: A Magnetic Resonance Parkinsonism Index value of >12.6 discriminated progressive supranuclear palsy from other causes of parkinsonism with a 91% sensitivity and 95% specificity. No planimetry measurement could accurately discriminate those with multiple system atrophy with parkinsonism from patients with Parkinson disease. A corpus callosum posteroanterior gradient value of ≤191 was highly specific (97%) and moderately sensitive (75%) for the diagnosis of corticobasal degeneration versus all other groups. A midbrain-to-corpus callosum posteroanterior gradient ratio of ≤0.45 was highly indicative of progressive supranuclear palsy over corticobasal degeneration (sensitivity 86%, specificity 88%). CONCLUSIONS: MR imaging planimetry measurements are potent imaging markers of progressive supranuclear palsy and promising markers of corticobasal degeneration but do not seem to assist in the diagnosis of multiple system atrophy with parkinsonism.

The phenotypic heterogeneity in amyotrophic lateral sclerosis (ALS) implies that patients show structural changes within but also beyond the motor cortex and corticospinal tract and furthermore outside the frontal lobes, even if frank dementia is not detected. The aim of the present study was to investigate both gray matter (GM) and white matter (WM) changes in non-demented amyotrophic lateral sclerosis (ALS) patients with or without cognitive impairment (ALS-motor and ALS-plus, respectively). Nineteen ALS-motor, 31 ALS-plus and 25 healthy controls (HC) underwent 3D-T1-weighted and 30-directional diffusion-weighted imaging on a 3 T MRI scanner. Voxel-based morphometry and tract-based spatial-statistics analysis were performed to examine GM volume (GMV) changes and WM differences in fractional anisotropy (FA), axial and radial diffusivity (AD, RD, respectively). Compared to HC, ALS-motor patients showed decreased GMV in frontal and cerebellar areas and increased GMV in right supplementary motor area, while ALS-plus patients showed diffuse GMV reduction in primary motor cortex bilaterally, frontotemporal areas, cerebellum and basal ganglia. ALS-motor patients had increased GMV in left precuneus compared to ALS-plus patients. We also found decreased FA and increased RD in the corticospinal tract bilaterally, the corpus callosum and extra-motor tracts in ALS-motor patients, and decreased FA and increased AD and RD in motor and several WM tracts in ALS-plus patients, compared to HC. Multimodal neuroimaging confirms motor and extra-motor GM and WM abnormalities in non-demented cognitively-impaired ALS patients (ALS-plus) and identifies early extra-motor brain pathology in ALS patients without cognitive impairment (ALS-motor).

Introduction. Aggressive surgical resection constitutes the optimal treatment for intracranial gliomas. However, the proximity of a tumor to eloquent areas requires exact knowledge of its anatomic relationships to functional cortex. The purpose of our study was to evaluate fMRI’s accuracy by comparing it to intraoperative cortical stimulation (DCS) mapping.Material and Methods. Eighty-seven patients, with presumed glioma diagnosis, underwent preoperative fMRI and intraoperative DCS for cortical mapping during tumor resection. Findings of fMRI and DCS were considered concordant if the identified cortical centers were less than 5 mm apart. Pre and postoperative Karnofsky Performance Scale and Spitzer scores were recorded. A postoperative MRI was obtained for assessing the extent of resection.Results. The areas of interest were identified by fMRI and DCS in all participants. The concordance between fMRI and DCS was 91.9% regarding sensory-motor cortex, 100% for visual cortex, and 85.4% for language. Data analysis showed that patients with better functional condition demonstrated higher concordance rates, while there also was a weak association between tumor grade and concordance rate. The mean extent of tumor resection was 96.7%.Conclusions. Functional MRI is a highly accurate preoperative methodology for sensory-motor mapping. However, in language mapping, DCS remains necessary for accurate localization.

In b-thalassemia, myocardial iron overload contributes to heart failure, despite chelation treatment. We hypothesized that myocardial T2*, an index of iron overload, influences patients' physical activity. We assessed a thalassemic population by both cardiovascular magnetic resonance imaging (CMR) and ergospirometry test. Sixty-six thalassemic patients aged 27 (19-40) years, 30 without (NHF) and 36 with heart failure (HF), were studied. Cardiac T2* and left ventricular ejection fraction (LVEF) were evaluated using a 1.5 T system. VO(2max), AT, Mets and duration of exercise by ergospirometry were also assessed. Myocardial T2* was lower in HF compared to NHF patients (14.7 +/- 6.6 vs. 39 +/- 2 ms, P < 0.001). LVEDV and LVESV were higher in HF group compared to NHF patients (139.9 +/- 16.3 vs. 124.6 +/- 20.86 ml, P < 0.01 and 94.9 +/- 24 vs. 38.3 +/- 10.1 ml, P < 0.001, respectively). Additionally, LVEF in HF was lower compared to NHF patients (21.3 +/- 6.1% vs. 69.6 +/- 3.7, P < 0.001, respectively). All exercise parameters were lower in HF compared to NHF patients (P < 0.001). Patients within the HF group were additionally analyzed according to T2* values (

Introduction b-Thalassemia major (TM) and thalassemia intermedia (TI) are forms of inherited hemoglobinopathies. Our aim was to evaluate a population of asymptomatic TM and TI patients using cardiovascular magnetic resonance (CMR). We hypothesized that the TI group could be differentiated from the TM group based on T2*. We also hypothesized that the TI group would demonstrate significantly higher cardiac output compared to the TM group. Patients and methods Twenty-one consecutive TI patients aged 23(19–25) years, 21 TM patients and 21 age and sex matched controls were studied. Evaluation of heart, liver T2* relaxation time and right and left ventricular parameters was performed using a 1.5 T system. Results Myocardial and liver T2* values were significantly higher in TI patients compared to TM (34.35 ± 2.36 vs 15.77 ± 3.53 m, P < 0.001 and 5.12 ± 6.52 vs 1.36 ± 0.53 ms, P < 0.001, respectively). Controls had myocardial T2* 35.07 ± 4.52 ms (similar to TI patients, but significantly increased compared to TM patients, P < 0.001) and liver T2* 26.28 ± 2.37 ms (significantly increased compared to both TI and TM patients, P < 0.001). Left ventricular end-diastolic (LVEDV), end-systolic (LVESV) volumes and left ventricular ejection fraction (LVEF) were higher in TI patients compared to TM (P < 0.001). Stroke volume (LVSV), cardiac output (LVCO) and cardiac index (LVCI) were similarly increased in TI patients compared to TM (P < 0.001). Right ventricular end-diastolic volume (RVEDV), right ventricular end-systolic volume (RVESV) and right ventricular ejection fraction (RVEF) were higher in TI patients compared to TM (P < 0.001). Conclusions Although in TM iron plays a crucial role in the evolution of the disease, in TI the high output cardiac state seems to be the most prominent finding.

Introduction Acupuncture has been widely reported to be a popular and safe intervention for smoking cessation. This study demonstrates the potential effects of acupuncture treatment for smoking cessation using the MRI technique of functional Magnetic Resonance Imaging (fMRI). Purpose Substance dependence or addiction is nowadays uderstood in a multifactorial etiological model, which includes psychological, neurobiological, genetic, social and environmental factors. This study illustrates patterns of fMRI activation in various specific brain anatomies, such as frontal, temporal parietal, occipital and cerebellum that correlate with smoking. Materials and methods Participants of both genders were volunteers willing to quit smoking. The inclusion criteria were age (28–42 y) and rate of smoking >15 cigarrettes per day and had no previous experience of acupuncture treatment. Group A received real acupuncture, Group B sham acupuncture and Group C was the control group with no smokers. Acupuncture points used: Du-20, LI-20, LI-4,TB-5, ST-36, LIV-3 and treatments lasted for 20 min. fMRI was applied prior and post acupuncture treatment during the first 24 hours of smoking cessation. During the fMRI experiment a series of smoking related images were presented to each participant via a set of goggles in a block time-design manner. Results Increased brain response areas were observed, such as: dorsolateral prefrontal, primary motor gyrus, parahippocampal gyrus, hippocampus, insula, posterior cingulate cortex, somatosensory cortex, visual areas and external basal ganglia. Differences between Groups A and B, as well as interesting correlations between acupuncture points, fMRI findings and neurobiological areas are presented. Conclusion Our results provide evidences over a pattern of slightly different brain activation following real and sham acupuncture after a short term smoking abstinence. The observed differences are in areas involved in drug addiction and smoking maintenance/abstinence literature.

Muscle atrophy, particularly of facial and bulbar muscles, seems to be a relatively common long-term consequence of musclespecific tyrosine kinase-myasthenia gravis (MuSK-MG), perhaps reflecting the chronic state of disease or long-term therapy with corticosteroids. We performed magnetic resonance imaging (MRI) to assess muscle wasting in the facial and bulbar muscles in two female MuSK-MG patients, with short duration of symptoms prior to diagnosis and prior to commencement of steroid therapy. The study revealed marked atrophy of temporalis, masseters, and lingual muscles with fatty replacement. MRI evidence of early muscle atrophy in MuSK-MG may indicate that MuSK antibodies per se may predispose to muscle atrophy.

Background & objectives Progressive multifocal leukoencephalopathy (PML) may complicate natalizumab treatment in multiple sclerosis patients. We sought to characterize the clinical and laboratory features of natalizumab-related PML (NR-PML) cases from Greece. Methods Pharmaceutical industry, national drug authorities and all neurology departments within the Greek territory were asked to provide data for cases of NR-PML until October 2012. Collected cases were classified according to their level of diagnostic certainty using the five-level system introduced by Mentzer et al. (2012) . Results Thirteen NR-PML cases were identified by the neurology departments. Data were provided for only 9 cases. PML manifestations appeared after a median number of 40 (21–52) natalizumab infusions. All but two patients were treated with plasma exchange and some were treated adjunctively with mirtazapine while the others were treated with mefloquine. IRIS developed in 6 cases after a median time of 6 (2–10) weeks from PML presentation and were treated with different regimens of corticosteroids. PML was fatal in 3 cases. The median EDSS after a median follow-up time of 12 (8–23) months in the surviving cases was 4.75 (2–8.5). Conclusions Outcomes for collected NR-PML cases varied from death to returning to baseline. Close surveillance is essential for early diagnosis and treatment of NR-PML patients.

Introduction Intravoxel Incoherent Motion (IVIM) imaging is a method that provides quantitative assessment all of the microscopic translational motions that could contribute to the signal acquired with diffusion weighted imaging (DWI) in MRI. The fundamental idea was that the molecular motion of water is randomly oriented in the capillary network at ultralow b values of diffusion imaging, mimicking a random walk. Diffusion parameters derived from IVIM model – perfusion F, true-ADC (D slow ), pseudo-ADC (D fast ) – have roused the researcher’s interest. Purpose The aim of this study is to quantify the IVIM model diffusion parameters in patients with brain lesions compared to the normal appearing symmetric regions of the brain. Materials and methods Patients underwent DWI on a 3T MRI system using an 8-channel sense head coil. DWI was performed with axial single-shot spin echo planar imaging (EPI) sequence with 10b values (0, 10, 15, 50, 80, 100, 200, 400, 700, 1000 s/mm 2 ). The IVIM model diffusion parameters of D slow , D fast and F were calculated using a biexponential curve fitting model based on Levenberg–Marquardt algorithm applied in an in-house platform in Matlab. The statistical analysis between contra lateral regions was based on Student’s t tests. Results Perfusion fraction F measured in brain damage regions showed significantly increased values compared to normal appearing symmetric regions. Parameters D slow and D fast had no statistical significance. Conclusion The F parameter may consist a new sensitive biomarker for providing additional information to conventional diffusion parameters and predicting preliminary brain damage.

Introduction Diffusion Tensor Imaging (DTI) reveals in vivo abnormalities in white matter (WM) fiber structure and has improved our knowledge of disease pathophysiology in several central nervous system (CNS) pathologies with WM involvement, especially through the analysis of different DTI indices. Purpose To investigate the sensitivity of DTI parameters using tract based spatial statistics (TBSS) in amyotrophic lateral sclerosis (ALS), as a CNS disease with predominant WM involvement. Material and methods We included 58 participants (36 patients with ALS and 22 healthy controls). All of them were scanned on 3.0T MR system, with 30-directional DTI and 3D-T1-weighted anatomical sequences. Whole-brain WM analysis was conducted using FSL and TBSS to estimate fractional anisotropy (FA), axial diffusivity (Ad) and radial diffusivity (Rd). Voxelwise statistical analysis was performed via a permutation-based inference for nonparametric statistical thresholding (5000 permutations). Two sample t-tests were applied to evaluate local alterations in DTI parameters (p Results In ALS patients we observed significant decreased FA and increased Rd (p left), including corticospinal tract (CST), body of the corpus callosum and superior longitudinal fasciculus. Rd changes were more diffuse and found along the CST axis (WM of precentral gyrus, corona radiata, internal capsule). No changes were detected for Ad (p > 0.05). Conclusion Decomposing the commonly used DTI parameters (FA and Mean Diffusivity) into Ad and Rd may be useful in identifying widespread WM changes in ALS and proved as a potential sensitive index for CNS neurodegenerative process. Disclosure F.C. acknowledges support from the IKY Fellowships of Excellence for Postgraduate Studies in Greece – Siemens Program . We also acknowledge the Philips Medical System for providing all necessary research keys for MR/DTI sequence acquisition. The funders and/or contributors had no role in study design, data collection and analysis or preparation of the abstract. Finally, we would like to thank the patients with ALS and their families, as well as healthy volunteers for their willingness to participate to the present study.

Introduction MR imaging is widely used method to aid in the detection of multiple sclerosis (MS) lesions in the cord. Improving the sensitivity of the detection of MS lesions can be useful in the medical diagnosis and could lead to a better approach and heal of the disease. Purpose The aim of this study was to compare T2-weighted turbo spin-echo (TSE) combined with spectral adiabatic inversion recovery technique for fat suppression (SPAIR) with proton density (PD) SPAIR in the detection of cervical MS plaques. Materials and methods Twenty five patients with clinically confirmed MS were examined on a 3.0T MR system. T2 and PD TSE SPAIR sequences were obtained in sagittal plane, using the same in plane resolution and slice thickness. The images set were assessed by two independent observers for the lesion detectability and image quality. Quantitative measurements of lesion-to-cord contrast ratio and lesion-contrast-to-noise ratio were obtained. All the lesions were verified with axial T2 multi echo gradient echo images. Results Fifteen of the scanned patients had MS lesion detected. PD_TSE_SPAIR sequence was most efficient in detection of MS lesions, demonstrated increased lesion contrast ratios about 15%, and also provided better image quality than T2_TSE_SPAIR. Twenty percent of lesions (3/20) that were not statistically significantly different from background cord signal on the T2 sequence. Conclusion PD_TSE_SPAIR sequence had high sensitivity to the detection of MS lesions compared to T2_TSE_SPAIR and should be included in the core sequences to the spinal MRI protocol for the assessment of suspected demyelinating disease. Disclosure There is not any disclosure.

Current evidence suggests cardiac involvement and electrocardiographic changes of increasing frequency with age in patients with myotonic dystrophy type 2 (DM2). Myocyte hypertrophy with concurrent fibrosis seems to be the anatomical correlate. Moreover, morphological and functional changes indicative of subclinical cardiomyopathy have been demonstrated by means of cardiac magnetic resonance imaging (CMRI) and spectroscopy in patients with no overt cardiac disease. We present a 68-year-old woman with genetically established DM2 and no clinical, electrocardiographic or echocardiographic signs indicative of cardiac involvement. CMRI revealed delayed contrast enhancement of the anterior portion of the interventricular septum, indicating myocardial involvement. Contrast-enhanced CMRI might be a useful diagnostic tool in assessing cardiac involvement in cases of DM2. The role of delayed contrast enhancement should be further investigated in order to elucidate the cardiac features of this fascinating multisystem disease.

Objective Paleoneurosurgery represents a comparatively new developing direction of neurosurgery dealing with archaeological skull and spine finds and studying their neurosurgical aspects. Trepanation of the cranial vault was a widespread surgical procedure in antiquity and the most convincing evidence of the ancient origin of neurosurgery. The present study considers a case of trepanation from the Middle Bronze Age Greece (1900–1600 B.C.). Methods The skull under study belongs to skeletal material unearthed from Kirra, Delphi (Central Greece). Macroscopic examination and palpation, as well as three-dimensional computed tomography, were used in this study. Results There is osteological evidence that the skull belongs to a man who died at 30–35 years of age. The procedure of trepanation was performed on the right parietal bone. Both macroscopic and computed tomography evaluation demonstrate an intravital bone reaction at the edges of the aperture. Projected on the right surface of the brain, the trepanation is located on the level of the central groove. The small dimensions and the symmetrical shape of this hole give us an indication that it was made by a metal tool. Conclusion We conclude that this paleopathological case provides valuable information about the condition of life and the pre-Hippocratic neurosurgical practice in Bronze Age Greece.

Muscle atrophy, when it occurs in myasthenia gravis (MG), is usually associated with long-standing disease or with chronic corticosteroid treatment. Early muscle atrophy in a steroid-naive patient has been reported previously only in muscle-specific tyrosine kinase (MuSK)-MG. We report a 63-year-old male patient with acetylcholine receptor (AchR)-positive MG with a short duration of disease, no steroid treatment and MRI evidence of muscle atrophy.

Bone marrow transplantation (BMT) is the only complete cure for b-thalassemia. Iron depletion therapy is still required to remove excess iron, accumulated before BMT. Hepatic and myocardial iron load were evaluated using T2* magnetic resonance in 8 ex-thalassemic patients after BMT, aged 19.5 +/- 4.25 years, who were in iron depletion therapy. Average hepatic T2* was 18.8 +/- 11.0 msec (4.1-35.0 msec). In 4 out of 8 patients iron overload was detected, not exceeding however 4 mg/gr dry tissue. Average heart T2* was 31.0 +/- 4.6 msec (25.6-35.2 msec), not significantly different (P = 0.18) from our age-matched normal population (33.0 +/- 4.0). Normal left ventricular ejection fraction was found in 7 out of 8 patients (mean 64.5 +/- 7.0%) with the remaining having a marginal value of 54.1%. Ferritin level before BMT was 1748 +/- 451 mug/l and dropped to 536 +/- 260 microg/l at the end of iron depletion therapy after BMT. Current ferritin level was 271 +/- 253 microg/l and although it was significant lower compared to both ferritin before BMT (P0.001) and after iron depletion (P0.001), evidence of residual hepatic iron load was identified by T2*. Hepatic and myocardial T2* magnetic resonance can be used as a more reliable index than ferritin for evaluation of iron depletion therapy in ex-thalassemic patients after BMT.

Fibrocartilaginous embolism of the intervertebral disc represents an uncommon cause of spinal cord infarction. We present the case of a previously healthy 30-year old ballet dancer who noted acute severe neck pain shortly after an intensive training session and developed weakness and numbness of both arms, as well as difficulties in emptying the bladder and bowel. Her clinical presentation and neuroimaging studies including diffusion weighted imaging were consistent with a spinal cord infarction in the anterior spinal territory at the C3-C6 spinal cord level. Although no histological confirmation was obtained, lack of evidence of other plausible diagnoses in the setting of the patient's clinical presentation and neuroimaging findings made fibrocartilaginous embolism the most likely etiopathogenetic mechanism of spinal stroke.