Meningioma as a rare cause of underlying clinical stroke.

In this case report a 54-year-old woman had an anterior clinoid process meningioma. She was initially diagnosed as having a cerebrovascular disease, however, her stroke-like symptoms were most likely caused by internal carotid artery compression or vasospasm due to meningiomal involvement, but initially overlooked. Meningiomas are rarely reported as a cause of a stroke. A detailed evaluation can provide a high degree of confidence in differentiating stroke and non-stroke medical conditions, known as stroke mimics or chameleons, to be considered when a diagnosis of stroke has not been confirmed.

Progressive multifocal leukoencephalopathy in a patient with multiple myeloma: a case report and analysis of the FDA adverse event reporting system.

This paper demonstrates a case of progressive multifocal leukoencephalopathy (PML) in a patient with multiple myeloma (MM) treated with nine different MM therapies. This case report contributes to the already published 16 cases of PML in patients with MM. Additionally, this paper presents an analysis of cases from the United States Food and Drug Administration Adverse Event Report System database (n = 117) with a description of demographics and MM-specific therapies. Patients with MM, that developed PML, were treated with immunomodulatory drugs (97%), alkylating agents (52%), and/or proteasome inhibitors (49%). Prior to PML diagnosis, 72% of patients received two or more MM therapies. These results indicate that PML in MM is underreported and could be related to treatment with multiple immunosuppressive therapies rather than MM as a disease itself. Physicians should be aware of potential PML in the late stage of heavily treated MM patients.

Cerebral infarction as initial manifestation of meningovascular neurosyphilis in an immunocompetent patient - A case report with long term follow-up.

To present a 29-year-old immunocompetent patient with neurosyphilitic changes characterized by multiple acute ischemic brain strokes along with significant narrowing of several large intracranial arteries. Ceftriaxone treatment for 14 days followed by benzathine benzylpenicillin weekly for additional 3 weeks, showed improvement in meningovascular changes.

[Spinal cord ischaemia as a rare form of stroke].

This is a case report about spinal cord ischaemia (SCI) in a 47-year-old woman with no known cerebrovascular risk factors. SCI is a rare entity with debilitating consequences, which typically presents with acute pain corresponding to the involved cord level, followed by flaccid paralysis, decreased sensation and autonomic dysfunction. The most common cause is aortic atheromatosis. Progression of symptoms, normal cerebrospinal fluid analysis and MRI are of key importance for the diagnosis of this entity, yet the latter can initially be normal. Anticoagulant therapy is the initial treatment of choice followed by long-term rehabilitation.

Opioid-Induced Leukoencephalopathies: A Report of Two Cases.

Acute toxic leukoencephalopathy (ATL) and delayed post-hypoxic leukoencephalopathy (DPHL) are two possible adverse entities related to opioid intoxication (OI), each having a distinct clinical course. While ATL shows a monophasic course with gradual neurological deterioration, DPHL has a distinct biphasic course. We report a case of ATL along with a case of DPHL happening in young male patients with OI, including their clinical courses as well as imaging characteristics with comparable time intervals. Initially, both leukoencephalopathies typically show magnetic resonance imaging findings with confluent and symmetric white matter (WM) abnormalities in the periventricular regions on T2 and fluid-attenuated inversion recovery images along with restricted diffusion on diffusion-weighted imaging. The DPHL patient however also presented with WM cystic substance loss in the deterioration phase, several weeks after hospital admission, which was previously described in a case of DPHL. Interestingly, similar WM changes have recently been observed in virus-associated necrotizing disseminated acute leukoencephalopathy in patients with coronavirus disease 2019 which may suggest a common pathophysiological mechanism. Knowing the distinct imaging features of ATL and DPHL along with their typical clinical courses can provide a faster and more reliable differentiation between these two entities.

Convolutional neural network performance compared to radiologists in detecting intracranial hemorrhage from brain computed tomography: A systematic review and meta-analysis.

PURPOSE: To compare the diagnostic accuracy of convolutional neural networks (CNN) with radiologists as the reference standard in the diagnosis of intracranial hemorrhages (ICH) with non contrast computed tomography of the cerebrum (NCTC). METHODS: PubMed, Embase, Scopus, and Web of Science were searched for the period from 1 January 2012 to 20 July 2020; eligible studies included patients with and without ICH as the target condition undergoing NCTC, studies had deep learning algorithms based on CNNs and radiologists reports as the minimum reference standard. Pooled sensitivities, specificities and a summary receiver operating characteristics curve (SROC) were employed for meta-analysis. RESULTS: 5,119 records were identified through database searching. Title-screening left 47 studies for full-text assessment and 6 studies for meta-analysis. Comparing the CNN performance to reference standards in the retrospective studies found a pooled sensitivity of 96.00% (95% CI: 93.00% to 97.00%), pooled specificity of 97.00% (95% CI: 90.00% to 99.00%) and SROC of 98.00% (95% CI: 97.00% to 99.00%), and combining retrospective and studies with external datasets found a pooled sensitivity of 95.00% (95% CI: 91.00% to 97.00%), pooled specificity of 96.00% (95% CI: 91.00% to 98.00%) and a pooled SROC of 98.00% (95% CI: 97.00% to 99.00%). CONCLUSION: This review found the diagnostic performance of CNNs to be equivalent to that of radiologists for retrospective studies. Out-of-sample external validation studies pooled with retrospective studies found CNN performance to be slightly worse. There is a critical need for studies with a robust reference standard and external data-set validation.

Onset of Schizophrenia Prior to the End of Brain Maturation Alters Grey Matter Volume Loss.

BACKGROUND: Brain maturation is considered completed around the age of 25, when prefrontal cortex maturation has been achieved. The aim of our study was to investigate the alterations of grey matter (GM) in patients with the onset of schizophrenia before and after the completion of brain maturation. SUBJECTS AND METHODS: The study group included 100 schizophrenia patients, while the control group comprised 50 healthy individuals. Brain magnetic resonance imaging was acquired on a 1.5 T scanner. Voxel-based morphometry (VBM) analyses were performed between groups. RESULTS: GM of the schizophrenic patients is reduced in many regions (p<0.005 FDR corrected). Most widespread reduction is detected in frontal cortex and cerebellum, the other regions being limbic cortex, insula, cuneus, precuneus, superior temporal gyrus and motor cortex. The decrease of grey matter volume (GMV) increases with the increase in number of psychotic episodes and is more pronounced in the patients with earlier onset of the disease. CONCLUSIONS: The age of the onset of the disease is important for both total and relative loss of GMV. Earlier onset of schizophrenia, prior to full brain maturation results in significant reduction of GM in comparison with healthy subjects and patients with later, post full brain maturation onset of the disease.

Influence of Psychotic Episodes on Grey Matter Volume Changes in Patients with Schizophrenia.

BACKGROUND: Schizophrenia is a severe illness whose clinical course is characterized by various numbers of psychotic episodes (PE). The neurotoxic hypothesis (NH) of schizophrenia assumes that psychosis is biologically toxic. The aim of the study was to investigate whether schizophrenia patients (SP) with multiple PE have greater grey matter volume (GMV) reduction compared to SP with fewer PE. SUBJECTS AND METHODS: We enrolled 106 adult SP and 63 healthy controls. Demographic and clinical data were collected and statistically analysed for all included subjects. Magnetic resonance imaging (MRI) of the brain was acquired on a 1.5 T scanner. SP were grouped according to the number of PE into a group with up to 3 PE (SCHG-1) and with 4 or more PE (SCHG-2). SCHG-1 was further subdivided into two groups regarding to disease duration (DD). Voxel-based morphometry (VBM) analyses were performed between SP groups as well as between SP groups and the healthy controls group (HCG). RESULTS: No relevant GMV differences were detected between SP groups. Comparison between HCG and SCHG-1 showed only 3 regions with reduced GMV, while multiple regions with reduced GMV were detected when comparing HCG and SCHG-2. CONCLUSIONS: GMV reduction in schizophrenia varies depending on the number of PE when compared to HCG, regardless of disease duration (DD), but PE is not the only contributing factor that leads to neurotoxicity.

Carotid plaque composition by CT angiography in asymptomatic subjects: a head-to-head comparison to ultrasound.

OBJECTIVES: To describe carotid plaque composition by computed tomography angiography (CTA) in asymptomatic subjects and to compare this to carotid plaque assessment by ultrasound, coronary plaques by coronary CTA, and inflammatory biomarkers in plasma. METHODS: Middle-aged asymptomatic men, n = 43, without known cardiovascular disease and diabetes were included. Plaques in coronary and carotid arteries were evaluated using CTA. Total plaque volumes and plaque composition were assessed by a validated plaque analysis software. The 60% centile cut point was used to divide the population into low or high carotid total plaque volumes. The occurrence of carotid plaques and intima-media thickness (IMT) was estimated by ultrasound. RESULTS: Carotid plaque by ultrasound was undiagnosed in 13 of 28 participants (46%) compared to CTA. Participants having carotid plaques by ultrasound had significantly higher absolute volumes of all CTA-defined carotid plaque subtypes and a higher fraction of calcified plaque. A high carotid total plaque volume was independently associated with age (adjusted odds ratio (OR) 1.41 [95% confidence interval (CI) 1.14-1.74], p = 0.001), IMT (adjusted OR 2.26 [95% CI 1.10-4.65], p = 0.03), and D-dimer (adjusted OR 8.86 [95% CI 1.26-62.37], p = 0.03). All coronary plaque features were significantly higher in participants with a high carotid total plaque volume. CONCLUSION: The occurrence of carotid plaques in asymptomatic individuals is underestimated by ultrasound compared to plaque assessment by CTA. Carotid plaque composition by CTA is different in individuals with and without carotid plaques by ultrasound. KEY POINTS: • The occurrence of carotid plaques by ultrasound was underestimated in 46% of participants who had plaques by carotid CTA. • Participants with carotid plaques by ultrasound had higher volumes of all plaque subtypes and a higher calcified plaque component as determined by carotid CTA compared to participants without carotid plaques by ultrasound. • A high carotid total plaque volume was independently associated with age, intima-media thickness, and D-dimer.

The multifactorial origin of posterior reversible encephalopathy syndrome in cyclophosphamide-treated lupus patients.

The cyclophosphamide as a predisposing factor for Posterior Reversible Encephalopathy Syndrome (PRES) and therapeutic option for systemic lupus erythematosus (SLE) is still confusing. The first and only case of PRES, probably induced by cyclophosphamide, in Croatia followed by the findings of 36 SLE patients diagnosed with PRES after treatment with cyclophosphamide worldwide are described. An 18-year-old Caucasian female patient with a 1-year history of SLE was admitted to the hospital due to lupus nephritis and acute arthritis. After the second dose of cyclophosphamide was administered, according to the Euro-lupus protocol, the patient presented with a grand mal status epilepticus. The differential diagnosis of neurolupus, cerebrovascular insult, and infection were excluded. The MRI findings showed brain changes in corresponding to PRES. The treatment consisted of antihypertensives, antiepileptics, antiedema therapy, mechanical ventilation, and avoiding further cyclophosphamide use. A Naranjo Adverse Drug Reaction Probability Scale total score of five and a probable reaction related to drug therapy (cyclophosphamide, PRES) was confirmed. In this systematic review, along with cyclophosphamide use, the main predisposing factors involved in PRES occurrence in SLE patients were active SLE and renal involvement. Due to the high number of simultaneously involved predisposing factors (max. six) and their overlapping effect, it is still not possible to clearly establish the role of every factor on PRES onset. The use of cyclophosphamide, as a contributing factor for PRES onset, should be carefully assessed, based on clinicians' experience and knowledge, in the setting of active SLE.

Encephalocraniocutaneous Lipomatosis Without Ocular Malformations.

BACKGROUND: Encephalocraniocutaneous lipomatosis is a rare congenital neurocutaneous syndrome resulting from ectomesodermal dysgenesis and characterized by unique hairless scalp lesions in the form of nevus psiloliparus, ipsilateral ocular malformations, and central nervous system anomalies. According to the 2009 diagnostic criteria proposed by Moog et al., ocular abnormalities are supposed to be the most consistent feature of encephalocraniocutaneous lipomatosis. PATIENT DESCRIPTION: We describe an 18-year-old girl with most of the central nervous system manifestations of encephalocraniocutaneous lipomatosis, major skin alterations including nevus psiloliparus, but no ocular involvement. CONCLUSION: Our patient suggests more variability in clinical features and a more complex genetic/embryonic etiology of encephalocraniocutaneous lipomatosis.

Coexistence of left internal carotid agenesis, klippel-feil syndrome and postaxial polydactyly.

BACKGROUND: Internal carotid artery agenesis is a rare anomaly that can be clinically asymptomatic. Klippel-Feil syndrome is a skeletal malformation characterized by vertebral fusion. Presence of postaxial polydactyly is suggestive of an underlying syndrome. CASE REPORT: We report a rare case of a 44-year-old patient with non-specific symptoms and an association between these three rare abnormalities. Vascular anomalies were found using intracranial MR angiography and multi-detector CT angiography of the supraaortic arteries. CONCLUSIONS: Presence of a single aforementioned anomaly requires cautious imaging assessment in order to detect possible associated anomalies and avoid diagnostic pitfalls. A possible common genetic background could explain the coexistence of these three anomalies.

Differentiation of pyogenic and fungal brain abscesses with susceptibility-weighted MR sequences.

INTRODUCTION: Conventional magnetic resonance imaging (MRI) techniques are insufficient to determine the causative agent of brain abscesses. We investigated: (1) the value of susceptibility-weighted MR sequences (SWMRS) in the differentiation of fungal and pyogenic brain abscesses; and (2) the effect of different SWMRS (susceptibility-weighted imaging (SWI) versus venous blood oxygen level dependent (VenoBOLD)) for the detection of specific imaging characteristics of pyogenic brain abscesses. METHODS: We studied six patients with fungal and ten patients with pyogenic brain abscesses. Imaging characteristics on conventional MRI, diffusion-weighted imaging (DWI) and SWMRS were recorded in all abscesses. All lesions were assessed for the presence of a "dual-rim sign" on SWMRS. RESULTS: Homogenously hyperintense lesions on DWI were present in 60 % of patients with pyogenic abscesses, whereas none of the patients with fungal abscesses showed such lesions. On SWMRS, 90 % of patients with pyogenic abscesses and 60 % of patients with fungal abscesses had only lesions with a low-signal-intensity rim. On SWI, the dual-rim sign was apparent in all pyogenic abscesses. None of the fungal abscesses on SWI (P = 0.005) or any of the pyogenic abscesses on VenoBOLD (P = 0.005) were positive for a dual-rim sign. CONCLUSIONS: In fungal abscesses, the dual-rim sign is not present but a prominent peripheral rim or central susceptibility effects on SWI will be seen. The appearance of pyogenic abscesses on SWMRS depends on the used sequence, with the dual-rim sign a specific feature of pyogenic brain abscesses on SWI.

Susac's syndrome during pregnancy - the first Croatian case.

Susac's syndrome (SS) is an infrequent neurological disorder characterized by the clinical triad of encephalopathy, branch retinal artery occlusion and hearing loss due to an autoimmune endotheliopathy associated with anti-endothelial cell antibodies. At the onset of the disease SS rarely appears with the complete clinical triad. The most important diagnostic procedures involved in the diagnosis of SS are brain MRI, audiometric testing and retinal fluorescein angiography. Presence of at least two components of the SS clinical triad accompanied by specific brain MRI findings is highly suggestive of SS. We report a case of a young pregnant woman with a history of encephalopathy, hearing loss and walking impairment. Brain MRI revealed a spectrum of findings previously described in patients with SS. We induced labor at 37 weeks' gestation to start with immunosuppressive treatment and avoid possible fetal toxicity. To the best of our knowledge this is the first report of SS in Croatia.

Simultaneous appearance of cerebral venous thrombosis and subdural hematomas as rare cause of headache in puerperium following epidural analgesia: a case report.

The aim of this study is to report the first case of simultaneous appearance of cerebral venous thrombosis (CVT) and bilateral subdural hematomas (SDHs) following epidural analgesia for labor and delivery and to point out the difficulty of establishing such a diagnosis in the presence of postpartum headache. A 26-year old primigravida with a history of epilepsy received epidural analgesia for delivery. Three days after the uneventful spontaneous vaginal delivery she complained about the headache. Patient responded very well to the pain medication and oral hydration, and the headache was relieved. Ten days after the delivery, the headache reoccurred, and an epidural blood patch was performed that successfully relieved her symptom. Stronger progressive headache with nausea reappeared two days later and the parturient was readmitted to hospital. Urgent neuroimaging examinations detected CVT of right the transverse sinus, ipsilateral cortical veins, and partially occluded superior sagittal sinus, as well as bilateral subacute/chronic SDHs. The treatment of the patient with low molecular weight heparin and antiaggregation therapy was effective. In this case, the diagnosis was delayed because of atypical clinical presentation and potentially confounding events (epidural analgesia and assumption that it was a case of PDPH). It is important to carefully observe patients in such conditions and promptly conduct suitable diagnostic tests. Otherwise, unrecognized intracranial complications and delay of appropriate therapy could be life-threatening.

Effect of Met66 allele of the BDNF rs6265 SNP on regional gray matter volumes in patients with multiple sclerosis: A voxel-based morphometry study.

The rs6265 single nucleotide polymorphism (SNP) is a genetic variation in the brain-derived neurotrophic factor (BDNF) gene wherein the presence of the A-allele at rs6265 causes replacement of a valine (Val) at position 66 by methionine (Met). We reported recently that the Met66 allele was associated with lower brain damage as evidenced by measurement of gray matter (GM) volume in multiple sclerosis (MS) patients. The objective of this study was to determine the voxel-wise regional GM differences between the Val66Val and Met66 allele groups in MS patients by using voxel-based morphometry (VBM)-optimized analysis corrected for lesion misclassification in Statistical Parametric Mapping (SPM5). High-resolution 3D-T1-weighted SPGR images from a total of 188 MS patients were acquired on a 1.5T MRI. The Val66Val group included 129 MS patients and the Met66 allele group (comprised of Val66Met or Met66Met genotypes) included 59 MS patients. The SPM analysis of covariance tool was used to assess group differences after controlling for variation in head size, MS disease course and gender. VBM analysis did not yield significant family wise error (FWE) corrected results. This was also confirmed with the non-parametric analysis using threshold-free cluster enhancement (TFCE) method. However, the results from VBM as well as the TFCE analyses (p<0.001, uncorrected) showed higher GM volume in the cingulate of MS patients with Met66 allele than those with Val66Val. Future studies are warranted to investigate longitudinally possible protective role of the Met66 allele of the BDNF rs6265 SNP in relation to specific GM regions.

Regionally distinct white matter lesions do not contribute to regional gray matter atrophy in patients with multiple sclerosis.

PURPOSE: To determine to what extent T1- and T2-regional lesion volumes (RLVs) contribute to total and/or regional gray matter (GM) atrophy in multiple sclerosis (MS). METHODS: We studied 110 (67 relapsing-remitting and 43 secondary-progressive) MS patients. SABRE program was used to parcel the brain into 13 regions per hemisphere. Total and regional GM fractions (GMFs) were determined in each region to correct for intraregional size variability. Partial correlations were used to determine associations (holding the converse constant) between RLVs, GMF, and regional GMFs (P < .001 to avoid Type 1 error). RESULTS: Partial correlations between RLVs and regional GMFs (controlling for total GMF) for the total MS group were not significant for any of the 26 regions for T2, whereas they were significant for two of the 26 regions for T1. Partial correlations between RLVs and total GMF (controlling for regional GMF) for the total MS group were significant in 9 of 26 regions for T2 (largest r = right lateral inferior frontal, -.45) and 5 of 26 regions for T1 (largest r = right inferior parietal, -.45). CONCLUSIONS: Results suggest a model whereby a distinct generalized disease process accounts for GM atrophy better than regionally distinct Wallerian degeneration.

Gray matter correlations of cognition in incident Parkinson's disease.

We aimed to investigate whether mild cognitive impairment (MCI) in Parkinson's disease (PD) is characterized by region-specific gray matter (GM) atrophy and to explore correlations between GM and cognition in PD. Magnetic resonance images of 42 newly diagnosed PD patients (of which 11 had MCI) and 37 normal controls were analyzed using voxel-based morphometry. Analyses comparing groups showed no regional atrophy, and in patients there were no significant correlations between cognitive domain test performance and GM loss. In conclusion, GM atrophy does not seem to be a major feature of cognitive dysfunction in incident PD.

Altered NK cell development and enhanced NK cell-mediated resistance to mouse cytomegalovirus in NKG2D-deficient mice.

NKG2D is a potent activating receptor on natural killer (NK) cells and acts as a molecular sensor for stressed cells expressing NKG2D ligands such as infected or tumor-transformed cells. Although NKG2D is expressed on NK cell precursors, its role in NK cell development is not known. We have generated NKG2D-deficient mice by targeting the Klrk1 locus. Here we provide evidence for an important regulatory role of NKG2D in the development of NK cells. The absence of NKG2D caused faster division of NK cells, perturbation in size of some NK cell subpopulations, and their augmented sensitivity to apoptosis. As expected, Klrk1(-/-) NK cells are less responsive to tumor targets expressing NKG2D ligands. Klrk1(-/-) mice, however, showed an enhanced NK cell-mediated resistance to mouse cytomegalovirus infection as a consequence of NK cell dysregulation. Altogether, these findings provide evidence for regulatory function of NKG2D in NK cell physiology.