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BackgroundFatigue and depression are among the most common manifestations of primary Sjögren syndrome (pSS), but information is lacking on the relationship with brain function and microstructural changes.PurposeTo investigate microstructural changes and brain connectivity in pSS, and to evaluate their relationship with fatigue and depression.Material and MethodsThe study included 29 patients with pSS (mean age 61.2 ± 12.1 years; disease duration 10.5 ± 5.9 years) and 28 controls (mean age 58.4 ± 9.2 years). All the patients completed the Beck’s depression and Fatigue Assessment Scale questionnaires. The imaging protocol consisted of: (i) standard magnetic resonance imaging (MRI) pulse sequences (FLAIR, 3D T1W); (ii) a diffusion tensor imaging pulse sequence; and (iii) a resting state functional MRI pulse sequence. Resting state brain networks and maps of diffusion metrics were calculated and compared between patients and controls.ResultsCompared with the controls, the patients with pSS and depression showed increased axial, radial, and mean diffusivity and decreased fractional anisotropy; those without depression showed decreased axial diffusivity in major white matter tracts (superior longitudinal fasciculus, inferior longitudinal fasciculus, corticospinal tract, anterior thalamic radiation, inferior fronto-occipital fasciculus, cingulum, uncinate fasciculus, and forceps minor-major). Decreased brain activation in the sensorimotor network was observed in the patients with pSS compared with the controls. No correlation was found between fatigue and structural or functional changes of the brain.ConclusionpSS is associated with functional connectivity abnormalities of the somatosensory cortex and microstructural abnormalities in major white matter tracts, which are more pronounced in depression.

Background On brain magnetic resonance imaging, both diffusion-weighted imaging (DWI) and perfusion-weighted imaging (PWI) are used to evaluate cerebral tumors. The purpose of this meta-analysis was to evaluate and compare the diagnostic performance of DWI and PWI in differentiating between pseudoprogression and true tumor progression of glioblastoma. Methods We performed a systematic review of the PubMed database from January 2000 to December 2019 for relevant studies. After application of specific inclusion and exclusion criteria, the eligible articles were evaluated for methodologic quality and risk of bias using the updated Quality Assessment of Diagnostic Accuracy (QUADAS-2) tool. From the published study results, the pooled sensitivity, pooled specificity, positive likelihood ratio, negative likelihood ratio, and diagnostic odds ratio and their corresponding confidence intervals (% CI), and the area under the curve, were calculated individually for DWI and PWI. Results The meta-analysis included 24 studies, with a total of 900 patients. DWI was found to be slightly superior in terms of sensitivity and specificity, 0.88 (% CI 0.83–0.92) and 0.85 (% CI 0.78–0.91), respectively, compared with the respective values of PWI, 0.85 (% CI 0.81–0.89) and 0.79 (% CI 0.74–0.84). On comparison of the overall diagnostic accuracy of the MRI modalities using their respective area under the curve values (0.9156 for DWI, 0.9072 for PWI), no significant difference was demonstrated between the 2. Conclusions Both DWI and PWI provided optimal diagnostic performance in differentiating pseudoprogression from true tumor progression in cerebral glioblastoma, and neither technique proved to be superior.

Purpose: To present an unusual case of posterior encephalopathy syndrome (PRES) preceded by intracranial hypotension.Materials and Methods: We present a case of a 27-year-old parturient with an uneventful pregnancy that shortly after labor developed a persistent headache with characteristics compatible with intracranial hypotension. The patient had undergone epidural anesthesia for caesarian section. Results: The symptomatology of intracranial hypotension was attributed to inadvertent dural puncture during epidural anesthesia. The MRI revealed multiple white matter lesions located in frontal, temporal and parietal regions of both hemispheres. The type of lesions was suggestive of PRES. Pachymeningeal enhancement was also observed. The patient was managed conservatively. The symptoms improved gradually and the imaging findings resolved completely. Conclusions: This case demonstrates the need for clinical alertness for PRES in patients with prolonged and possibly atypical symptoms of intracranial hypotension. As probable causal relationship between these disorders we propose a sympathetic over-activation as a result of cerebrospinal fluid leakage leading to vasospasm and manifestation of PRES.

Glioblastoma (GBM) is the most common primary malignant type of brain neoplasm in adults and carries a dismal prognosis. The current standard of care for GBM is surgical excision followed by radiation therapy (RT) with concurrent and adjuvant temozolomide-based chemotherapy (TMZ) by six additional cycles. In addition, antiangiogenic therapy with an antivascular endothelial growth factor (VEGF) agent has been used for recurrent glioblastoma. Over the last years, new posttreatment entities such as pseudoprogression and pseudoresponse have been recognized, apart from radiation necrosis. This review article focuses on the role of different imaging techniques such as conventional magnetic resonance imaging (MRI), diffusion-weighted imaging (DWI), diffusion tensor imaging (DTI), dynamic contrast enhancement (DCE-MRI) and dynamic susceptibility contrast (DSE-MRI) perfusion, magnetic resonance spectroscopy (MRS), and PET/SPECT in differentiation of such treatment-related changes from tumor recurrence.

The purpose of the present study was to investigate the pattern of white matter (WM) changes associated with Parkinson's disease (PD)-related cognitive impairment by using fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD) measures. Diffusion Tensor Imaging (DTI) was performed in 21 PD-patients with dementia (PDD) and in an age-matched control group including 40 PD-patients without dementia (PD-CTRL). The Parkinson's disease-Cognitive Rating Scale (PD-CRS) was used for patients' neuropsychological assessment. Local microstructural WM differences associated with the presence of cognitive impairment were tested using tract-based spatial statistics analysis. Multiple regression models investigated the association of DTI indices with total PD-CRS score, PD-CRS raw items and other clinical measures across the whole study sample. Significant FA decreases were found in PDD compared to PD-CTRL patients mainly in the body of corpus callosum, corona radiata and cingulum. Lower PD-CRS score was significantly associated with decreased FA, MD and AD values in multiple WM tracts primarily located in prefrontal and limbic areas as well as across the corpus callosum. Lower performance in specific PD-CRS raw items was also associated with FA decreases in major WM tracts. The results suggest that multifocal microstructural changes of WM accompany the transition from normal to demented cognitive state in PD-patients. The corpus callosum, the corona radiata and the cingulum are among the regions mostly affected during this course. A progressive axonal degeneration is proposed as a key underlying mechanism.

Purpose To assess macro- and microstructural brain changes in patients with pseudoexfoliation syndrome (PXS). Materials and methods Comprehensive ophthalmic examination and brain MRI were conducted on 20 patients with PXS without glaucoma (aged 62.75 ± 0.4 years) and 20 controls (aged 62 ± 0.6 years). White matter (WM) integrity was evaluated on FLAIR and single-shot multisection SE-EPI diffusion tensor imaging (DTI) sequences. The presence and the number of white matter hyperintensities (WMHIs) on FLAIR images was compared between all patients and control subjects. Microstructural WM changes on DTI was evaluated using Tract-based spatial statistics (TBSS). DTI metrics of the optic tracts were assessed by the region-of-interest (ROI) method. Results A significantly higher number of WMHIs was found in the patients with PXS than in the control subjects (P ≤ 0.002). On DTI the patients showed bilateral increase in the mean diffusivity (MD), axial diffusivity (AD) and radial diffusivity (RD) values in the anterior thalamic radiation, the inferior fronto-occipital fasciculus, the superior longitudinal fasciculus, the inferior longitudinal fasciculus and the forceps minor. TBSS revealed no significant difference in fractional anisotropy (FA) values, but ROIs analysis of the optic tracts revealed decreased FA values in the patients. Conclusion MRI in patients with PXS detects abnormalities in the brain and the optic tracts at a subclinical stage. Early detection of microstructural changes could be useful to guide appropriate treatment to impede the disease process.

Inherited optic neuropathies are a genetically diverse group of disorders mainly characterized by visual loss and optic atrophy. Since the first recognition of Leber’s hereditary optic neuropathy, several genetic defects altering primary mitochondrial respiration have been proposed to contribute to the development of syndromic and non-syndromic optic neuropathies. Moreover, the genomics and imaging revolution in the past decade has increased diagnostic efficiency and accuracy, allowing recognition of a link between mitochondrial dynamics machinery and a broad range of inherited neurodegenerative diseases involving the optic nerve. Mutations of novel genes modifying mainly the balance between mitochondrial fusion and fission have been shown to lead to overlapping clinical phenotypes ranging from isolated optic atrophy to severe, sometimes lethal multisystem disorders, and are reviewed herein. Given the particular vulnerability of retinal ganglion cells to mitochondrial dysfunction, the accessibility of the eye as a part of the central nervous system and improvements in technical imaging concerning assessment of the retinal nerve fiber layer, optic nerve evaluation becomes critical – even in asymptomatic patients – for correct diagnosis, understanding and early treatment of these complex and enigmatic clinical entities.

Objectives To evaluate risk factors for the development of cervical spine spondylosis (CSS) in patients with multiple sclerosis (MS) and to propose a pathogenetic mechanism. Methods Forty-two consecutive patients aged 23–66 years with MS and 42 age and sex matched controls were evaluated retrospectively; Clinical disability was evaluated with the expanded disability status scale (EDSS) and spasticity with the Asworth score. Total brain lesion volume (BLV), total grey matter (GM) volume and deep GM volume were assessed. In the cervical spine CSS indices (disk dehydration, disk protrusion, abnormal posture and osteophytosis) and the spinal cord lesion load (SLL) was evaluated. The association of CSS indices with the presence of MS, the clinical scales and the brain and spinal cord imaging measurements were assessed. Results Presence of MS was positively associated with abnormal posture (P = 0.002), disk dehydration at C6–C7 (P = 0.049) and posterior disk protrusion at C5–C6 (P = 0.033) and C6–C7 (P = 0.001). All patients had spasticity. Patients with abnormal posture were younger (37.5 ± 11.1 years) than those with normal (45.4 ± 8.6 years), P = 0.024. Age (P = 0.008), EDSS (P = 0.045) and BLV (P = 0.084) were significant independent predictors of abnormal posture. Younger age combined with worse EDSS and increased BLV predicted abnormal posture. Conclusions Patients with MS present more frequently spondylosis which is associated with younger age, more severe disability and extensive lesions in the brain. Spasticity induced by the brain lesions and abnormal expression of extracellular matrix proteins in the brain and the intervertebral disk constitute a possible pathogenetic mechanism.

We report a 61-year-old woman who presented because of gradually progressive speech disorders. MRI scan revealed left temporal lobe atrophy with ex vacuo dilatation of the temporal horn and atrophy of the left inferior frontal gyrus and the left inferior parietal lobe. These findings in brain perfusion SPECT corresponded to a generalized hypoperfusion of the left hemisphere, particularly prominent on the rear parietal cortex and the temporal lobe. After taking into consideration the clinical presentation and imaging findings on MRI and SPECT, the diagnosis of the logopenic variant of primary progressive aphasia was finally reached.

Brain tumors in children are the second most common type of pediatric tumors after leukemia and the most common occurring solid tumor. Brain tumors diagnosed during the first year of life have different features than those presenting later in childhood and are usually supratentorial. Magnetic resonance imaging is the modality of choice for the diagnosis and characterization of these tumors. Herewith, we provide a review of the imaging characteristics on conventional and advanced neuroimaging techniques of the most common supratentorial intracranial tumors in children.

Structural and functional changes of the brain have been reported in premature babies. To evaluate the relationship of functional and structural connectivity with gestational age, body growth and brain maturation in very preterm babies. We studied 18 very preterm babies (gestational age: mean ± standard deviation, 29.7±1.7 weeks). We examined functional connectivity by multivariate pattern analysis of resting-state functional MRI data. We assessed structural connectivity by analysis of diffusion tensor imaging data and probabilistic tractography. The average functional connectivity of the medial orbitofrontal cortex with the rest of the brain was positively associated with gestational age (P

Teratomas involving the spinal cord are very rare tumors that affect more commonly children than adult. We report a rare case of an intradural extramedullary teratoma arising in the region of conus medullaris in a previously healthy adult patient. The lesion was totally excised and the postsurgical outcome was favorable. Teratomas should be taken into consideration in the differential diagnosis in previously healthy adults with sudden onset of lower back pain or neurological deficits of the lower extremities and with a tumoral lesion of the spinal cord. Total surgical excision is the indicative treatment of choice.

The multimodal imaging investigation of excessive daytime sleepiness (EDS) in Parkinson's disease (PD). The role of dopaminergic treatment and other clinical parameters was also evaluated.Seventeen non-demented PD patients with EDS (PD-EDS) and 17 PD patients without EDS were enrolled. Clinical, treatment and MRI data were acquired. Gray matter (GM) volume was examined with voxel-based morphometry, while white matter (WM) integrity was assessed with diffusion tensor imaging by means of fractional anisotropy, mean diffusivity, axial diffusivity (AD) and radial diffusivity measures.Increased regional GM volume was found in the PD-EDS group bilaterally in the hippocampus and parahippocampal gyri. Increased AD values were also shown in the PD-EDS group, in the left anterior thalamic radiation and the corticospinal tract and bilaterally in the superior corona radiata and the superior longitudinal fasciculus. Levodopa equivalent dose differed significantly between the groups and was the only predictor of EDS, while the only predictor of the Epworth sleepiness scale score in the PD-EDS group was the dopamine-agonist dose. Increased frequency of gamblers was also observed in the PD-EDS group.Regional GM increases and increased AD values in certain WM tracts were found in the PD-EDS group. The changes could result from disinhibited signaling pathways or represent compensatory changes in response to anatomical or functional deficits elsewhere. The study findings support also the contribution of the total dopaminergic load in the development of EDS, while the dose of dopamine agonists was found to predict the severity of the disorder.

Introduction Treatment induced necrosis is a relatively frequent finding in patients treated for high-grade glioma. Differentiation by imaging modalities between glioma recurrence and treatment induced necrosis is not always straightforward. This is a comparative study of diffusion tensor imaging (DTI), dynamic susceptibility contrast MRI and 99mTc-Tetrofosmin brain single-photon emission computed tomography (SPECT) for differentiation of recurrent glioma from treatment induced necrosis. Methods A prospective study was made of 30 patients treated for high-grade glioma who had suspected recurrent tumor on follow-up MRI. All had been treated by surgical resection of the tumor followed by standard postoperative radiotherapy with chemotherapy. No residual tumor had been found on brain imaging immediately after the initial treatment. All the patients were studied with dynamic susceptibility contrast brain MRI and, within a week, 99mTc-Tetrofosmin brain SPECT. Results Both 99mTc-Tetrofosmin brain SPECT and dynamic susceptibility contrast MRI could discriminate between tumor recurrence and treatment induced necrosis with 100% sensitivity and 100% specificity. An apparent diffusion coefficient (ADC) ratio cut-off value of 1.27 could differentiate recurrence from treatment induced necrosis with 65% sensitivity and 100% specificity and a fractional anisotropy (FA) ratio cut-off value of 0.47 could differentiate recurrence from treatment induced necrosis with 57% sensitivity and 100% specificity. A significant correlation was demonstrated between 99mTc-Tetrofosmin uptake ratio and rCBV (P = 0.003). Conclusions Dynamic susceptibility contrast MRI and brain SPECT with 99mTc-Tetrofosmin had the same accuracy and may be used to detect recurrent tumor following treatment for glioma. DTI also showed promise for the detection of recurrent tumor, but was inferior to both dynamic susceptibility contrast MRI and brain SPECT.

To investigate structural brain changes in inflammatory bowel disease (IBD). Brain magnetic resonance imaging (MRI) was performed on 18 IBD patients (aged 45.16 ± 14.71 years) and 20 aged-matched control subjects. The imaging protocol consisted of a sagittal-FLAIR, a T1-weighted high-resolution three-dimensional spoiled gradient-echo sequence, and a multisession spin-echo echo-planar diffusion-weighted sequence. Differences between patients and controls in brain volume and diffusion indices were evaluated using the voxel-based morphometry (VBM) and tract-based spatial statistics (TBSS) methods, respectively. The presence of white-matter hyperintensities (WMHIs) was evaluated on FLAIR images. VBM revealed decreased grey matter (GM) volume in patients in the fusiform and the inferior temporal gyrus bilaterally, the right precentral gyrus, the right supplementary motor area, the right middle frontal gyrus and the left superior parietal gyrus (p

Resume Objectifs Les spondylarthrites (SpA) sont associees a un risque cardiovasculaire accru. Nous avons etudie les facteurs de risque cardiovasculaire chez les patients atteints de SpA. Methodes Les facteurs de risque suivants ont ete evalues chez les patients atteints de SpA et chez les temoins sains : tabagisme, antecedents familiaux de cardiopathie ischemique precoce, obesite, lipides seriques, apolipoproteines (apo), urate et epaisseur intima-media de la carotide (EIM). Resultats Cent cinquante patients (73 atteints de spondylarthrite ankylosante [SA], 71 de rhumatisme psoriasique [RPs] et 6 d’autres types de SpA) ont ete inclus. En general, les patients SpA etaient significativement plus souvent fumeurs, alors que l’obesite abdominale etait plus elevee chez les patients RPs. Les patients atteints de SA avaient des niveaux significativement plus bas de triglycerides, d’HDL, d’apoB, d’apoE et de Lp (a), et un indice atherogene plus eleve (cholesterol total [CT]/lipoproteines de haute densite [HDL]). Les patients atteints de RPs avaient des niveaux significativement plus bas d’HDL, d’apoAI et d’apoE, un index atherogene plus eleve et une uricemie superieure. En analyse multivariee, l’indice atherogene etait positivement associe a la SpA dans tous les groupes de patients, independamment du tabagisme et des autres parametres lipidiques. L’EIM carotidienne chez les patients atteints de SpA (0,71 mm) etait plus eleve que chez les temoins (0,63 mm, p = 0,017), mais la difference n’etait plus significative apres ajustement avec le parametre de tabagisme. Le traitement des patients sans traitement prealable induisait une baisse legere mais significative des niveaux d’apoB apres six mois (p = 0,045), mais plus apres 12 mois. Conclusion Le risque cardiovasculaire est plus eleve chez les patients atteints de SpA en raison de la prevalence elevee du tabagisme et un plus grand indice atherogene. Les patients RPs ont plus de graisse abdominale et des niveaux d’acide urique eleves. Les traitements immunosuppresseurs de la SpA ont des effets mineurs et temporaires sur le profil lipidique.

Objectives The spondyloarthritides (SpA) are associated with an increased cardiovascular risk. We studied cardiovascular risk factors in patients with SpA. Methods The following risk factors were assessed in SpA patients and healthy controls: smoking, family history of premature ischemic heart disease, obesity, serum lipids, apolipoproteins, urate and carotid intima media thickness (IMT). Results Overall 150 patients (73 with ankylosing spondylitis [AS], 71 with psoriatic arthritis [PsA] and six with other SpA types) were included. Generally SpA patients were significantly more often smokers, while PsA patients had greater values of abdominal obesity. AS patients had significantly lower levels of triglyceride, HDL, ApoB, ApoE and Lp(a) and a higher atherogenic index (total cholesterol/HDL). PsA patients had significantly lower levels of HDL, ApoAI and ApoE, an elevated atherogenic index and higher serum urate. In multivariate analysis the atherogenic index was positively associated with SpA across all patient groups independently of smoking and other lipid parameters. Carotid IMT in SpA patients (0.71 mm) was higher than controls (0.63 mm, P = 0.017), although after adjusting for smoking this ceased to be significant. Treatment of patients with previously untreated disease resulted in a small but significant decline in ApoB levels at 6 months ( P = 0.045), which, however, was no longer evident at 12 months. Conclusion Spondyloarthritis patients are at a greater cardiovascular risk owing to the higher prevalence of smoking and a higher atherogenic index. PsA patients have more abdominal fat and higher urate levels. Immunosuppressive treatment of SpA produces minor and temporary effects on the lipid profile.

Prematurity and intrauterine growth restriction are associated with neurodevelopmental disabilities. To assess the relationship between growth status and regional brain volume (rBV) and white matter microstructure in premature babies at around term-equivalent age. Premature infants (n= 27) of gestational age (GA): 29.8 ± 2.1 weeks, with normal brain MRI scans were studied at corrected age: 41.2 ± 1.4 weeks. The infants were divided into three groups: 1) appropriate for GA at birth and at the time of MRI (AGA), 2) small for GA at birth with catch-up growth at the time of MRI (SGAa) and 3) small for GA at birth with failure of catch-up growth at the time of MRI (SGAb). The T1-weighted images were segmented into 90 rBVs using the SPM8/IBASPM and differences among groups were assessed. Fractional anisotropy (FA) was measured bilaterally in 15 fiber tracts and its relationship to GA and somatometric measurements was explored. Lower rBV was observed in SGAb in superior and anterior brain areas. A positive correlation was demonstrated between FA and head circumference and body weight. Body weight was the only significant predictor for FA (P

BACKGROUND AND PURPOSE: Histopathologic studies have demonstrated WM damage in primary Sjögren syndrome. The purpose of this study was to evaluate WM microstructural changes by use of DTI-derived parameters in patients with primary Sjögren syndrome. MATERIALS AND METHODS: DTI was performed in 19 patients with primary Sjögren syndrome (age, 64.73 ± 9.1 years; disease duration, 11.5 ± 7.56 years) and 16 age-matched control subjects. Exclusion criteria were a history of major metabolic, neurologic, or psychiatric disorder and high risk for cardiovascular disease. Data were analyzed by use of tract-based spatial statistics, for which the WM skeleton was created, and a permutation-based inference with 5000 permutations was used with a threshold of P < .01, corrected for multiple comparisons to enable identification of abnormalities in fractional anisotropy, mean diffusivity, radial diffusivity, and axial diffusivity. RESULTS: Tract-based spatial statistics showed decreased fractional anisotropy in multiple areas in patients with primary Sjögren syndrome compared with control subjects, located mainly in the corticospinal tract, superior longitudinal fasciculus, anterior thalamic radiation, inferior fronto-occipital fasciculus, uncinate fasciculus, and inferior longitudinal fasciculus. Increased mean diffusivity and radial diffusivity and decreased axial diffusivity were observed in most of the fiber tracts of the brain in patients with primary Sjögren syndrome, compared with control subjects. CONCLUSIONS: Patients with primary Sjögren syndrome show loss of WM microstructural integrity, probably related to both Wallerian degeneration and demyelination.

Purpose Increased Body-Mass-Index (BMI) has been associated with brain atrophy in both gray and white matter structures. However, little is known concerning the integrity of white matter tracts in obesity. The purpose of the study was to evaluate the pattern of changes in white matter microstructure in human adiposity. Material and methods The study included 268 participants (52 obese, 96 overweight and 120 normal-weight) that were retrospectively evaluated by Diffusion Tensor Imaging. The fractional anisotropy, axial, radial and mean diffusivity values were compared between the above groups using Tract Based Spatial Statistics. Results The analysis revealed that the increased BMI was related with decreased fractional anisotropy in several white matter regions including the anterior and posterior thalamic radiation, the inferior fronto-occipital fasciculus, the inferior and superior longitudinal fasciculus, the corpus callosum (callosal body and forceps minor), the uncinate fasciculus, the internal capsule, the corticospinal tract and the cingulum (cingulate gyrus and hippocampus). Conclusions Anisotropic diffusion of anatomic regions governing important brain circuits such as reward seeking inhibition, motivation/drive and learning/conditioning decreases with increasing BMI.

Purpose We prospectively investigated the relationship between diffusion tensor imaging (DTI), dynamic susceptibility perfusion (DSP) MRI metrics and grade, subtype and Ki-67 labelling index of meningiomas. Materials and Μethods Thirty-nine patients operated for meningioma were included in the study. DTI and DSP were performed within a week prior to surgical excision. Lesion/normal (L/N) tissue ratios and peritumoral area/normal tissue (P/N) ratios were calculated for the apparent diffusion coefficient (ADC), fractional anisotropy (FA) and relative cerebral blood volume (rCBV). In the tumor specimens Ki-67 antigen expression was evaluated by the MIB-1 immunostaining method. Results There were 31 grade I, 7 grade II and one grade III meningiomas. Grade I meningiomas had a significantly lower L/N rCBV ratios than grade II/III meningiomas (median 5.1 vs 6.4, p=0.031). Grade I meningiomas revealed significantly lower P/N rCBV ratios than grade II/III meningiomas (median 0.78 vs 1.1, p=0.0077). Grade I meningiomas had significantly higher FA ratios than grade II/III meningiomas (median 0.5 vs 0.31, p=0.012). Meningiomas of meningothelial type had a significantly higher L/N rCBV ratio than other grade I meningiomas (median 5.4 vs 3.8, p=0.0136). There was no significant correlation between rCBV, ADC, FA and Ki-67 index. Conclusion Dynamic susceptibility perfusion indexes in lesion/normal and peritumoral/normal tissue ratios are useful for the differentiation grade I from grade II/III menigiomas. Meningothelial meningiomas showed higher lesion/normal tissue rCBV ratios from the other benign meningioma subtypes.

Kearns–Sayre syndrome (KSS) is a rare mitochondrial DNA deletion syndrome defined as the presence of ophthalmoplegia, pigmentary retinopathy, onset less than age 20 years, and one of the following: cardiac conduction defects, cerebellar syndrome, or cerebrospinal fluid protein above 100 mg/dl. KSS may affect many organ systems causing endocrinopathies, encephalomyopathy, sensorineural hearing loss, and renal tubulopathy. Clinical presentation at diagnosis is quite heterogeneous and, usually, few organs are affected with progression to generalized disease early in adulthood. We present the case of a boy with KSS presenting at the age of 5 years with myopathy, Addison's disease, primary hypoparathyroidism, and Fanconi syndrome. The proper replacement treatment along with the administration of mitochondrial metabolism-improving agents had a brief ameliorating effect, but gradual severe multisystemic deterioration was inevitable over the next 5 years. Conclusion This report highlights the fact that in case of simultaneous presentation of polyendocrinopathies and renal disease early in childhood, KSS should be considered.

We present the case of an 11-year-old obese girl who presented with idiopathic intracranial hypertension affecting first the lateral abducens nerve. She received acetazolamide, but 5 days later she developed lateral, peripheral facial palsy. Imaging evaluation was normal, which primarily excluded cerebral venous thrombosis and sustained the initial diagnosis. Despite some complicating factors (obesity, elevated intracranial pressure), prednisolone was administered for a short-term period to counteract the facial palsy. Ophthalmological residuals resolved within almost 1.5 months, while facial palsy receded after 4 months. Peripheral facial palsy is an extremely rare, but not unknown condition in idiopathic intracranial hypertension. As a symptom, it should be investigated thoroughly, primarily to exclude cerebral venous sinus thrombosis, before it can be attributed to idiopathic intracranial hypertension. As far as treatment is concerned, corticosteroids can be added to the initial treatment with acetazolamide, without worsening already elevated intracranial hypertension or ophthalmologic findings.

Periocular xanthogranulomatous diseases are a rare group of disorders which are characterized by a predilection to affect the orbit and ocular adnexa and special histopathological features, in particular infiltrates comprising non-Langerhans-derived foamy histiocytes and Touton giant cells. The differential diagnosis is difficult and occasionally definite diagnosis cannot be established even after clinical and histopathological findings are taken together. We describe a case of a middle-aged man who presented with a 10-year history of voluminous eyelid swelling with concomitant late-onset atopic manifestations, namely bronchial asthma and allergic rhinitis with nasal polyps. After thorough clinical and laboratory investigation, including a biopsy of the eyelid, we classified the patient’s disease to a rare entity that has been relatively recently described: periocular xanthogranuloma associated with adult-onset asthma. In a review of the literature, no prospective trials concerning the treatment of this disease were found. The literature mainly contained case reports and case series in which corticosteroids and chemotherapy with alkylating agents have been reported to be beneficial. We treated our patient with a combination of oral corticosteroids and cyclophosphamide pulses and we observed substantial regression of the eyelid masses together with a normalization of systemic immunologic abnormalities.

Neuropathological studies in experimental and human glaucoma have shown degenerative changes in the optic pathway. The purpose of the study was to evaluate, with conventional MRI and magnetisation transfer imaging, the brain and the optic pathway of patients with primary open-angle glaucoma (POAG). 26 patients, aged 67.4+/-8.6 years, and 26 control subjects were studied. The presence of white matter hyperintensities (WMH) was evaluated on fluid-attenuated inversion recovery images of the brain. The area of the optic nerves was assessed on coronal short tau inversion recovery images. Magnetisation transfer ratio (MTR) was measured in the chiasm and in the grey and white matter (CGM and CWM) of the calcarine fissure. More WMH were observed in patients (total 261, mean 10.8, standard deviation 12.7) than in control subjects (total 127, mean 4.7, standard deviation 5.7; p

Grey matter (GM) maturation has not been previously studied in healthy preterm children. The purpose of this study was to evaluate the age dependency of GM development in 116 GM areas in preterm subjects. Sixty one preterm infants (corrected age: 13.7+/-9.92 months, gestational age: 33.4+/-1.9 weeks) with normal structural appearance on MRI were included in the study. Using a T1-weighted high resolution 3D spoiled gradient echo sequence, volumes of 116 GM areas were calculated after their segmentation using the Voxel Based Morphometry Toolboxes and the Individual Brain Atlas Statistical Parametric Mapping (IBASPM) software packages. Non linear regression analysis assessed age dependency of volume data for every GM area using the monoexponential function y=A-Bexp(-x/C). All supratentorial GM areas followed the monoexponential function model reasonably well. Cerebellar structures had a poor goodness of fit. Volume increase of the individual GM areas followed an inferior to superior and a posterior to anterior pattern. The putamen, thalamus, and caudate nucleus reached 99% of the final volume earlier than most cortical GM areas. The visual cortex and the postcentral and precentral cortices matured earlier than the parietal, frontal and temporal cortices. The fronto-occipital asymmetry or torque seen in adults was observed in the preterm infants; the left occipital areas reached maturation earlier than the right, while the right prefrontal and frontal areas matured earlier than the left. To conclude, GM development progresses in a region-specific manner coinciding with functional, phylogenetical and regional white matter (WM) maturation. Neuroimage

BACKGROUND AND PURPOSE Multiple system atrophy (MSA) is a progressive neurodegenerative disorder divided into a parkinsonian (MSA-P) and a cerebellar variant. The purpose of this study was to assess regional brain atrophy and iron content using Voxel-based morphometry (VBM) and Voxel-based relaxometry (VBR) respectively, in MSA-P. METHODS Using biological parametric mapping the effect of brain atrophy was evaluated in T2 relaxation time (T2) measurements by applying analysis of covariance (ANCOVA) and correlation analysis to the VBM and VBR data. Eleven patients with MSA-P (aged 61.9 ± 11.7 years, disease duration 5.42 ± 2.5 years) and 11 controls were studied. RESULTS In comparison to the controls the patients showed decreased gray matter in the putamen, the caudate nuclei, the thalami, the anterior cerebellar lobes, and the cerebral cortex, and white matter atrophy in the pons, midbrain, and peduncles. VBR analysis showed prolonged T2 in various cortical regions. On ANCOVA, when controlling for gray and white matter volume, these regions of prolonged T2 were shrunk. Negative correlation was demonstrated between T2 and gray and white matter volume. CONCLUSIONS Diffuse brain atrophy, mainly in the motor circuitry is observed in MSA-P. Normalization for atrophy should always be performed in T2 measurements.

There is evidence of microstructural changes in normal-appearing white matter of patients with tuberous sclerosis complex.To evaluate major white matter tracts in children with tuberous sclerosis complex using tract-based spatial statistics diffusion tensor imaging (DTI) analysis.Eight children (mean age ± standard deviation: 8.5 ± 5.5 years) with an established diagnosis of tuberous sclerosis complex and 8 age-matched controls were studied. The imaging protocol consisted of T1-weighted high-resolution 3-D spoiled gradient-echo sequence and a spin-echo, echo-planar diffusion-weighted sequence. Differences in the diffusion indices were evaluated using tract-based spatial statistics.Tract-based spatial statistics showed increased axial diffusivity in the children with tuberous sclerosis complex in the superior and anterior corona radiata, the superior longitudinal fascicle, the inferior fronto-occipital fascicle, the uncinate fascicle and the anterior thalamic radiation. No significant differences were observed in fractional anisotropy, mean diffusivity and radial diffusivity between patients and control subjects. No difference was found in the diffusion indices between the baseline and follow-up examination in the patient group.Patients with tuberous sclerosis complex have increased axial diffusivity in major white matter tracts, probably related to reduced axonal integrity.

Objectives Flow cytometry provides a powerful tool to assess cells in G 0 /G 1 , S and G 2 /M phase and ploidy. The purpose of the present study was to investigate the correlation between diffusion tensor (DTI) and dynamic susceptibility contrast (DSC) MRI metrics with cell cycle analysis findings in gliomas. Patients and methods We studied thirty patients who were operated on for glioma. DTI and DSC MRI were performed within a week prior to surgical excision. Lesion/normal ratios were calculated for the ADC, FA and rCBV. In an excised tumour sample flow cytometric analysis was performed. Results There were 24 glioblastomas, 2 anaplastic astrocytomas, 1 oligoastrocytoma and 3 diffuse astrocytomas. There were significant differences between low and high-grade gliomas for rCBV and ADC values. Low grade tumours had higher G 0 /G 1 phase fraction and lower S-phase, G 2 /M, S + G 2 /M and S + G 2 /M/G 0 /G 1 fractions There was a significant negative correlation between rCBV and G 0 /G 1 phase fraction and a positive correlation with G 2 /M, S + G 2 /M and the S + G 2 /M/G 0 /G 1 fraction. Significant correlation was also observed between FA ratio and S + G 2 /M/G 0 /G 1 . There was a negative significant correlation between ADC and S + G 2 /M and the S + G 2 /M/G 0 /G 1 fraction. There were 21 (70%) diploid and 9 (30%) aneuploid tumours. No significant difference was found between diploid and aneuploid tumours with respect to rCBV, ADC and FA values. Conclusion Dynamic susceptibility contrast MRI and diffusion tensor imaging metrics are correlated to tumour aggressiveness as assessed by cell cycle analysis.

The purpose of the study was to evaluate brain myelination by measuring the magnetization transfer ratio (MTR) and to measure grey (GMV) and white matter volume (WMV) in macrocephalic children with neurofibromatosis type 1 (NF1). Seven NF1 patients (aged 0.65-16.67 years) and seven age- and gender-matched controls were studied. A three-dimensional (3D) gradient echo sequence with and without magnetization transfer (MT) prepulse was used for MTR assessment. Volume measurements of GM and WM were performed by applying segmentation techniques on T2-weighted turbo spin echo images (T2WI). MTR of unidentified bright objects (UBOs) on T2WI in cerebellar white matter (52.8+/-3.3), cerebral peduncles (48.5+/-1.5), hippocampus (52.6+/-1.1), internal capsule (55.7+/-0.3), globus pallidus (52.7+/-3.9), and periventricular white matter (52.6+/-1.2) was lower than in the corresponding areas of controls (64.6+/-2.5, 60.8+/-1.3, 56.4+/-0.9, 64.7+/-1.9, 59.2+/-2.3, 63.6+/-1.7, respectively; p0.05). MTR of normal-appearing brain tissue in patients was not significantly different than in controls. Surface area (mm(2)) of the corpus callosum (809.1+/-62.8), GMV (cm(3)) (850.7+/-42.9), and white matter volume (WMV) (cm(3)) (785.1+/-85.2) were greater in patients than in controls (652.5+/-52.6 mm(2), 611.2+/-92.1 cm(3), 622.5+/-108.7 cm(3), respectively; p0.05). To conclude, macrocephaly in NF1 patients is related to increased GMV and WMV and corpus callosum enlargement. MTR of UBOs is lower than that of normal brain tissue.

To study the progress of myelination in preterm-born subjects by measuring the MT ratio (MTR) from birth, up to 24 months of corrected age. One hundred twenty-five preterm subjects (64 males and 61 females of gestational age 33+/-2.4 weeks with chronologic and corrected age of 9.3+/-5.1 and 7.7+/-5.1 months, respectively) with normal brain MR using classic sequences were further evaluated for MTR by using a three-dimensional gradient-echo sequence (TR=32/TE=8/flip angle=6 degrees 4 mm/2 mm overlapping sections) with and without magnetization transfer prepulse. The magnetization transfer ratio was calculated as: MTR=(SIo-SIm)/SIox100%, where SIm refers to signal intensity from an image acquired with a MT prepulse and SIo the signal intensity from the image acquired without a MT prepulse. MTR increased asymptotically in the genu (R2=0.85) and splenium (R2=0.85) of the corpus callosum, the white matter of the frontal lobe (R2=0.91) and occipital lobe (R2=0.82), thalamus (R2=0.86), caudate nucleus (R2=0.67) and putamen (R2=0.71), reaching the 95% of the final value at the corrected age 18.7, 17.7, 15.6, 12.9, 10.4, 9.2 and 6.4 months, respectively. This study shows age-related changes of the brain MTR and provides data that may be useful to assess disturbances in the progress of myelination. European Radiology

The aim of this study was to evaluate whether cyclosporin A (CsA) influences the radiological disease progression in early rheumatoid arthritis (RA) patients in comparison with other disease-modifying drugs (DMARDs). A total of 103 early RA patients, without prior use of DMARDs, were randomized to receive CsA (3 mg/kg per day) or methotrexate (MTX) (0.15 mg/kg per week). In addition, all patients received prednisone (7.5 mg/day). After 42 months of treatment, pairs of hand and wrist radiographs of 41 patients treated with CsA and 42 treated with MTX were evaluated blindly and separately by two investigators, using reference radiographs for scoring. A scale scoring similar to Larsen's standard radiographs with minor modifications was used. The studied radiographs were obtained at the beginning and 42 months after therapy in both groups. Patients in both groups responded beneficially to the above treatment regimens. In the CsA group, 37 patients (71 %) remained radiographically stable and 4 worsened, while in the MTX group 39 patients (76%) remained stable and 3 deteriorated. No significant radiological worsening was found in the CsA-treated patients as compared to those treated with MTX. Early immuno-intervention in RA patients appears to be crucial for the future development of joint damage: CsA can delay radiological disease progression and may inhibit joint damage deterioration in early RA patients.

Although myopathy or myositis may occur in systemic sclerosis (SSc) as well as soft tissue calcification, ossification is not a feature of the disease. Here we present an unusual case of extended calcification and, to a lesser degree, ossification of the right gluteal region, lateral thigh, and knee, with associated myopathy and functional impairment. Heterotopic ossification (HO), or myositis ossificans, has not been reported so far in scleroderma patients, making this case, in our opinion, unique and interesting. However, differential diagnosis from other causes of extraskeletal ossification, such as tumours or tumour-like conditions, is required.

Background Indirect traumatic optic neuropathy (ITON) is a rare occurrence in patients with head injuries, mostly affecting young people. Loss of vision is a feared consequence. Its natural history and optimal care remain undetermined. Randomized controlled studies are missing. Treatment alternatives include mere observation, corticosteroid administration, or surgical decompression of the optic canal. Among these options, an individual approach is considered. Case Reports We report five patients with head injury and ITON: a 63-year-old woman was left permanently blind after a fall; a 33-year-old man lost his vision of the left eye immediately after a car accident; and three young men (aged 26, 21, and 22 years) who were intubated after a road accident noted loss of vision from one eye after they became conscious. The 26-year-old patient in the last group regained his sight 1 month later, whereas the other two never had it returned. All of our patients were treated conservatively: Methylprednisolone was administrated. Conclusion Patients with ITON are managed on an individual basis. Informed consent must be obtained for mere observation or administration of corticosteroids and/or optic canal decompression. The natural history of this disorder is unknown. Only randomized controlled studies can fix standard guidelines of care. Preventive measures to decrease the incidence of head injured patients are mandatory.

Background: Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiologic entity characterized by headache, visual disturbances, seizures, and the presence of edema on MRI scan, predominantly in the posterior white matter. Regarding end-stage renal disease (ESRD) and PRES, only a few cases of children on peritoneal dialysis (PD) and adults on hemodialysis have been described in the literature. Cases: We report 4 cases of adult patients on PD who presented with PRES, all of which were due to hypertension and inadequate management of fluid balance. The patients expressed typical PRES symptoms such as headache, visual disorders, and tonic/clonic seizures. The patients recovered completely and the MRI lesions disappeared after strict control of volume status. Conclusion: Nephrologists should be aware of the syndrome, especially when they manage hypertensive ESRD patients not compliant with the fluid and diet restrictions. MRI scan is the only diagnostic tool for defining the syndrome. Early diagnosis is important, since complete remission is achieved after appropriate treatment.

To evaluate the magnetic resonance imaging (MRI) findings of hand involvement before and 1 year after treatment in patients with early rheumatoid arthritis (RA).MRI of the dominant hand was performed in 22 patients fulfilling the new criteria for early RA. The patients were divided into three groups. Nine had very early RA (VERA; disease duration3 months), seven had early RA (ERA; disease duration6 months), and six had established RA (ESTRA; disease duration12 months). The MRI protocol consisted of fat-suppressed T2, and plain and contrast-enhanced T1-weighted sequences. Assessment of bone marrow oedema, synovitis, and bone erosions was performed by the OMERACT RA MRI scoring system. Patients were treated with methotrexate (MTX) 0.2 mg/kg/body weight/week and prednisone 7.5 mg/day. Clinical assessment was evaluated using the Disease Activity Score for 28 joint indices (DAS28).After treatment, a significant decrease was observed: (a) in DAS28 of VERA (6.2 ± 0.9 vs. 2.4 ± 1.2), ERA (5.3 ± 0.8 vs. 2.8 ± 1.0), and ESTRA patients (5.7 ± 8.0 vs. 2.7 ± 0.7; p0.05); (b) in bone oedema (16.77 ± 13.78 vs. 5.88 ± 6.31) and synovitis (12.44 ± 6.44 vs. 2.88 ± 3.25) of VERA patients; and (c) in synovitis (7.57 ± 6.32 vs. 1.42 ± 1.81) of ERA patients (p0.05). No significant difference was found in erosions in any group.Bone marrow oedema and synovitis decrease significantly when RA is diagnosed and treated early. MRI is useful in the early detection of these changes. MTX treatment resulted in a significant decrease in DAS28 score and significant improvement in bone oedema and synovitis.

BACKGROUND AND PURPOSE: Neuropathologic studies in experimental and human glaucoma have demonstrated degenerative changes in the optic pathway. The purpose of this study was to assess the optic pathway in POAG by using VBM and DTI. MATERIALS AND METHODS: Eighteen patients 57.05 +/- 11.38 years of age with POAG of 8.30 +/- 5.14 years' duration and 18 control subjects underwent a complete ophthalmologic examination, including quantification of the RNFLT by using Stratus OCT 3, and brain imaging. The imaging protocol consisted of a T1-weighted high-resolution 3D spoiled gradient-echo sequence and a multisection spin-echo- planar diffusion-weighted sequence. Data preprocessing and analysis were performed by using Matlab 7.0 and SPM 5. RESULTS: Left temporal and right nasal RNFLTs were significantly thinner than right temporal and left nasal RNFLTs. In patients, VBM revealed a significant reduction in the left visual cortex volume, the left lateral geniculate nucleus, and the intracranial part of the ONs and the chiasma. In addition, a significant decrease of FA was observed in the inferior fronto-occipital fasciculus, the longitudinal and inferior frontal fasciculi, the putamen, the caudate nucleus, the anterior and posterior thalamic radiations, and the anterior and posterior limbs of the internal capsule of the left hemisphere (P < .05). CONCLUSIONS: Neurodegenerative changes of the optic pathway and several brain areas associated with the visual system can be observed by using VBM and DTI in patients with POAG, suggesting that glaucoma is a complex neurologic disease. AJNR Am J Neuroradiol

Glioblastoma multiforme (GBM) is the most frequent malignant primary brain tumor in adults, exhibiting poor survival. The efficacy of chemotherapy is often limited by the development of multidrug resistance by the tumor cells. In the current study, we investigated the prognostic significance of the multidrug resistance protein 5 (MRP5) in patients with GBM. We retrospectively studied 33 patients with GBM treated with a combination of surgery, postoperative radiotherapy and adjuvant temozolomide chemotherapy. MRP5 protein expression was determined immunohistochemically and correlated with other prognostic factors and survival. The immunohistochemical expression of MRP5 was observed in 0-45% of tumor cells. Patients with MRP5 index > 11% exhibited significantly worse survival compared to those with MRP5 index a parts per thousand currency sign11 (10.5 vs. 18 months, p = 0.0002). Patients with Ki-67 index lower than 30% had longer survival (15 vs. 11 months, p = 0.0084). Furthermore, patients with a gross total tumor excision had better survival (p = 0.016). No significant difference was observed between preoperative Karnofsky performance score, age, gender and survival. In multivariate analysis, MRP5 index and the extent of tumor resection were identified as factors with independent prognostic power. The present results imply that MRP5 index may hold a prognostic role in patients with GBM. Cancer Chemother Pharmacol

Objective: We prospectively investigated the correlation between diffusion tensor (DTI), dynamic susceptibility contrast (DSC) perfusion MRI metrics and Ki-67 labelling index in glioblastomas. Methods: We studied seventeen patients who were operated on for glioblastoma. DTI and DSC MRI were performed within a week prior to surgical excision. Lesion/normal ratios were calculated for the apparent diffusion coefficient (ADC), fractional anisotropy (FA), relative cerebral blood volume (rCBV), relative cerebral blood flow (rCBF) and relative mean transit time (rMTT) ratio. In the excised tumour specimens Ki-67 antigen expression was evaluated by the MIB-1 immunostaining method. Results: A significant correlation was observed between Ki-67 index and ADC ratio (r = -0.528, p = 0.029) and FA ratio (r = 0.589, p = 0.012). rCBV and rMTT presented a trend towards significant correlation with Ki-67 index (r = 0.628, p = 0.07 and r = 0.644, p = 0.06 respectively). There was a trend towards better survival for patients with gross total tumour excision and FA values lower than 0.48 (p = 0.1 and p = 0.09 respectively). No significant correlation was found between ADC ratio, rCBV, rCBF, rMTT and overall survival. Conclusion: ADC ratio, FA ratio, rCBV and rMTT tumour/normal tissue ratios may represent indicators of glioma proliferation. FA values may hold promise for predicting survival in patients with glioblastoma. (C) 2011 Elsevier B.V. All rights reserved. Clin Neurol Neurosurg

Metastatic brain tumors are the most common intracranial neoplasm in adults. Bladder cancer gives metastasis to the brain in less than 1%. Herewith, we report on a case of a 71-year-old man who was admitted to the emergency department after an episode of loss of consciousness. On neurological examination a left hemiparesis was observed. The patient's previous history entailed a total cystectomy and radical prostatectomy 7 months ago because of a transitional cell carcinoma (TCC) of the urinary bladder. Brain imaging work-up revealed a cystic lesion with perifocal edema in the right frontal lobe. The patient was operated and the histological diagnosis was consistent with a metastatic carcinoma, with morphological, histochemical and immunohistochemical features comparable to those of the primary tumor. Postoperative the patient was in excellent neurological state and received complementary chemotherapy and total brain irradiation. Additional imaging and laboratory examinations excluded other metastatic lesion. The patient died 18 months later due to systemic disease. Although intracranial metastases from TCC of urinary bladder have a low incidence, in follow-up examinations any alterations in neurological status in these patients should be thoroughly evaluated.

We describe a 17-year-old Caucasian adolescent with ulcerative colitis who presented with cerebral venous sinus thrombosis. Laboratory investigation revealed low protein S levels. With successful management the patient remained without neurologic sequalae. Although there may be an association between ulcerative colitis and cerebral venous sinus thrombosis, the exact pathophysiologic mechanism remains unknown.

Introduction Numerous pathogens can cause infective endocarditis, including Haemophilus parainfluenzae. H. parainfluenzae is part of the H. aphrophilus, Actinobacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella corrodens, and Kingella kingae group that may cause about 3% of the total endocarditis cases, and is characterized by a subacute course and large vegetations. Case presentation Acute H. parainfluenzae endocarditis developed in a 54-year-old woman, with no underlying predisposing factors. The patient presented with fever of 3 days duration and a severe headache. Magnetic resonance imaging of the brain revealed multiple cerebral emboli with hemorrhagic foci. Upon suspicion of endocarditis, cardiac transesophageal ultrasonography was performed and revealed massive vegetations. The patient underwent emergency mitral valve replacement, and was further treated with ceftriaxone. Blood cultures grew H. parainfluenzae only after valve replacement, and a 6-week course of ceftriaxone was prescribed. Conclusion We underline the typical presentation of large vegetations in H. parainfluenzae endocarditis, which are associated with embolic phenomena and resulting severity. Although the majority of the few cases reported in the literature are subacute in progress, our case further underlines the possibility that H. parainfluenzae endocarditis may develop rapidly. Thus, awareness of the imaging characteristics of the pathogen may enhance early appropriate diagnosis and therapeutic response.