Injury to the circuit of Papez: An overlooked cause of recurrent seizures.

Key Clinical Message: Papez' circuit is a unique neural pathway in the limbic system that is correlated with seizure activity. Injuries affecting Papez' circuit are often small and unusual in location but can be identifiable in MRI and functional imaging modalities, which can be helpful in the workup of refractory epilepsy. Abstract: The Papez circuit is a unique neural pathway in the limbic system of the brain. We review a patient presenting with recurrent seizures as the main manifestation of Papez' circuit pathology. The radiologic features of ischemia involving the mammillothalamic tract in Papez' circuit were correlated with the seizure activity.

Cerebral developmental venous anomalies in children with mismatch repair deficiency.

BACKGROUND: Constitutional mismatch repair deficiency (CMMRD) is one of the rare cancer predisposition syndromes. The aim of this study was to evaluate the cerebral developmental venous anomalies in children with central nervous system tumors associated with CMMRD, an area in which there is extremely little experience. METHODS: Data from children diagnosed with medulloblastoma and high grade central nervous sytem tumor were retrospectively collected. According to the European CMMRD criteria, nine patients were diagnosed as CMMRD syndrome and the others consisted of the group without CMMRD. All radiological examinations of these children were retrospectively reviewed. Whole exome sequencing was performed to index cases` germline DNA. RESULTS: Nine children from four families, six females and three males, were studied. The median age at the first tumor diagnosis was 4.5 years (range, 9 months to 14 years). All CMMRD patients had café au lait spots, but none fulfilled the diagnostic criteria for neurofibromatosis. The patients developed high-grade glial tumor (n: 7) and medulloblastoma (n: 2). The affected genes in the three families were MSH6 [c.478C > T (p.Gln160Ter)], MSH6 [c.2871dupC (p.Phe958LeufsTer5)] and MLH1 [c.236G > A(p.Arg79Lys)], respectively. Seven patients had multiple developmental venous anomalies; six patients had leptomeningeal enhancement; and five patients had cavernomas. None of these findings were present in the group without CMMRD. CONCLUSIONS: Constitutional mismatch repair deficiency should be considered when multiple developmental venous anomalies, cavernomas, and leptomeningeal enhancement are detected, especially in patients with café au lait spots.

Can Dynamic Susceptibility Contrast Perfusion Imaging be Utilized to Detect Isocitrate Dehydrogenase Gene Mutation in Gliomas?

AIM: To explore the ability of dynamic susceptibility contrast perfusion imaging (DSC-PI) to detect isocitrate dehydrogenase (IDH) gene mutation in gliomas. MATERIAL AND METHODS: Preoperative DSC-PI data on histopathologically proven gliomas obtained between January 2015 and December 2019 were reviewed retrospectively. All magnetic resonance imaging (MRI) examinations were performed using a 1.5-T scanner. The maximum relative cerebral blood volume (rCBVmax), percentage signal recovery (PSR), and normalized PSR of tumor cores were calculated. Differences in these values between IDH-mutant and wild-type gliomas were compared, and receiver operating characteristic curves were generated. RESULTS: The patients (32 females, 47 males) were aged 21-76 years (mean 50.7 ± 15 years). The rCBVmax and all PSR values differed significantly between patients with IDH-mutant and those with wild-type tumors (p < 0.01 for all comparisons). CONCLUSION: The rCBVmax and PSR values obtained by DSC-PI may facilitate noninvasive detection of the IDH mutation status of gliomas. PSR provided more reliable values for differentiation of IDH-mutant gliomas from wild-type gliomas.

Hyponatremia and extrapontine myelinolysis in a patient with COVID-19: A case report.

Until we have strong evidence to the contrary, symptomatic hyponatremia should be treated with extra caution in COVID-19 co-infection patients as the latter could be another risk factor for the development of extrapontine myelinolysis.

An Incidental Finding of Scalp Angiosarcoma: A Case Report.

Scalp angiosarcoma is a malignant tumor of the vascular endothelial cells. We present the case of an elderly male patient with a history of urinary bladder transitional cell cancer and trauma (falling on his head) who came to the emergency department with scalp swelling, which was found on brain imaging to infiltrate into the skull, reaching the dural matter. A biopsy was done, which showed angiosarcoma, which is rare for that area. Further studies are recommended to establish if there is a possible genetic association between both cancers (urinary bladder transitional cell cancer and scalp angiosarcoma) as both arise from endothelial cells.

Delayed Radiation Myelopathy in a Child With Hodgkin Lymphoma and ARTEMIS Mutation.

The authors present a case of delayed radiation myelopathy in a 12-year-old girl with Hodgkin lymphoma and Artemis mutation. This is the first of such a case presented in the literature.

Spectrum of neuroimaging findings in COVID-19.

An outbreak of corona virus disease 2019 (COVID-19) began in China in December 2019, and rapidly spread to become a worldwide pandemic. Neurological complications encountered in hospitalized patients include acute arterial ischemic cerebrovascular stroke, cerebral venous thrombosis, critical illness-associated cerebral microbleeds, hypertensive hemorrhagic posterior reversible encephalopathy, meningoencephalitis/flare up of infections, flare up of multiple sclerosis, acute disseminated encephalomyelitis, cerebral hemodynamic/hypoxic changes such as watershed ischemic changes and hypoxic ischemic encephalopathy, and spine manifestations of Guillain Barre syndrome and viral myelitis. The purpose of our study is to illustrate the different neuroimaging features in critically ill hospitalized COVID-19 positive patients in the State of Qatar.

Precise discrimination between meningiomas and schwannomas using time-to-signal intensity curves and percentage signal recoveries obtained from dynamic susceptibility perfusion imaging.

BACKGROUND AND PURPOSE: Meningiomas and schwannomas are common extra-axial brain tumors. Discrimination is challenging in some locations when characteristic imaging features are absent. This study investigated the accuracy of percentage signal recoveries obtained from dynamic susceptibility contrast perfusion imaging (DSC-PI) in discriminating meningiomas and schwannomas. MATERIAL AND METHODS: Retrospective database research was conducted. Sixty nine meningioma and 15 schwannoma having DSC-PI between January 2016 and February 2020 were included. Time to signal intensity curves (TSIC) were analyzed and grouped as T1-dominant leakage, T2*-dominant leakage and return to baseline. Relative cerebral blood volume (rCBV), relative mean transit time (rMTT), percentage signal recovery 1 (PSR 1) and PSR 2 values were calculated. The differences between the groups were investigated. Receiver operating characteristic curves were operated. RESULTS: rCBV, rMTT, PSR 1 and PSR 2 values were statistically different between meningiomas and schwannomas. PSR 2 provided the best discrimination. With the cut off value of 1.08 for PSR 2, meningiomas and schwannomas were differentiated with 95.7% sensitivity and 93.3% specificity. TSICs were also different between two groups. Most of meningiomas showed T2*-dominant leakage (78.2%), whereas most of shwannomas showed T1-dominant leakage (93.3%). CONCLUSION: DSC-PI is a useful imaging tool for non-invasive discrimination of meningiomas and schwannomas. Particularly, percentage signal recoveries discriminates meningiomas and schwannomas with high sensitivity and specificity.

Correction to: Advanced imaging in adult diffusely infiltrating low-grade gliomas.

The original article [1] contains errors in Table 1 in rows ktrans and Ve; the correct version of Table 1 can be viewed in this Correction article.

Advanced imaging in adult diffusely infiltrating low-grade gliomas.

The adult diffusely infiltrating low-grade gliomas (LGGs) are typically IDH mutant and slow-growing gliomas having moderately increased cellularity generally without mitosis, necrosis, and microvascular proliferation. Supra-total resection of LGG significantly increases the overall survival by delaying malignant transformation compared with a simple debulking so accurate MR diagnosis is crucial for treatment planning. Data from meta-analysis support the addition of diffusion and perfusion-weighted MR imaging and MR spectroscopy in the diagnosis of suspected LGG. Typically, LGG has lower cellularity (ADCmin), angiogenesis (rCBVmax), capillary permeability (Ktrans), and mitotic activity (Cho/Cr ratio) compared to high-grade glioma. The identification of 2-hydroxyglutarate by MR spectroscopy can reflect the IDH status of the tumor. The initial low ADCmin, high rCBVmax, and Ktrans values are consistent with the poor prognosis. The gradual increase in intratumoral Cho/Cr ratio and rCBVmax values are well correlated with tumor progression. Besides MR-based technical artifacts, which are minimized by the voxel-based assessment of data obtained by histogram analysis, the problems derived from the diversity and the analysis of imaging data should be solved by using artificial intelligence techniques. The quantitative multiparametric MR imaging of LGG can either improve the diagnostic accuracy of their differential diagnosis or assess their prognosis.

Enfermedad de neuro-Behçet con paquimeningitis en una niña / Neuro-Behçet disease presented with pachymeningitis in a child

La enfermedad de Behçet (EB) es un trastorno de vasculitis sistémica poco frecuente, de etiología desconocida, que se caracteriza por la presencia de aftas bucales, úlceras genitales y uveítis recurrentes. Afecta los sistemas nerviosos central y periférico; raramente se produce durante la niñez. La meningitis aséptica aguda aislada es muy poco frecuente. En este artículo, se describe el caso de una paciente de 14 años con diagnóstico de enfermedad de Behçet con afectación neurológica (neuro-Behçet). La paciente tenía cefalea aguda, diplopia, papiledema e irritación meníngea. Tenía antecedentes de úlceras bucales recurrentes. Las imágenes por resonancia magnética de cerebro revelaron paquimeningitis. Los hallazgos en el líquido cefalorraquídeo fueron pleocitosis y aumento de la presión. Pese al tratamiento médico, sus síntomas no se resolvieron. No se detectó uveítis y la prueba de patergia fue negativa. El alelo HLA-B51 fue positivo. Se consideró que los hallazgos apuntaban a la poco frecuente enfermedad de Behçet con afectación neurológica. La paciente mejoró drásticamente luego del tratamiento con corticoesteroides. En el diagnóstico diferencial de meningitis, se debe considerar la EB, a menos que se demuestre la presencia de un agente infeccioso. Hasta donde sabemos, anteriormente no se había descrito un caso de paquimeningitis con neuro-Behçet en la población pediátrica.

Behçet's disease (BD) is a rare systemic vasculitis disorder of unknown etiology characterized by recurrent oral and genital apthae and uveitis. It involves the central or peripheral nervous system; occurs rarely during childhood. Isolated acute aseptic meningitis is extremely uncommon. We report here a case of Neuro-Behçet disease (NBD) diagnosed in a 14-year-old girl. The patient presented acute headache, diplopia, papilla edema, and meningeal irritation. She had a history of recurrent oral ulcers. Brain magnetic resonance imaging revealed pachymeningitis. Pleocytosis and pressure increase were the cerebrospinal fluid findings. Although medical therapy, her complaints were not resolved. Uveitis was not detected, pathergy test was negative. HLA-B51 allele was positive. The findings were considered to unusual NBD. The patient improved dramatically after steroid therapy. BD should be considered in differential diagnosis of meningitis unless an infectious agent is demonstrated. To our knowledge, a case of pachymeningitis with NBD, was not described in children.

Neuro-Behçet disease presented with pachymeningitis in a child. / Enfermedad de neuro-Behçet con paquimeningitis en una niña.

Behçet's disease (BD) is a rare systemic vasculitis disorder of unknown etiology characterized by recurrent oral and genital apthae and uveitis. It involves the central or peripheral nervous system; occurs rarely during childhood. Isolated acute aseptic meningitis is extremely uncommon. We report here a case of Neuro-Behçet disease (NBD) diagnosed in a 14-year-old girl. The patient presented acute headache, diplopia, papilla edema, and meningeal irritation. She had a history of recurrent oral ulcers. Brain magnetic resonance imaging revealed pachymeningitis. Pleocytosis and pressure increase were the cerebrospinal fluid findings. Although medical therapy, her complaints were not resolved. Uveitis was not detected, pathergy test was negative. HLA-B51 allele was positive. The findings were considered to unusual NBD. The patient improved dramatically after steroid therapy. BD should be considered in differential diagnosis of meningitis unless an infectious agent is demonstrated. To our knowledge, a case of pachymeningitis with NBD, was not described in children.

La enfermedad de Behçet (EB) es un trastorno de vasculitis sistémica poco frecuente, de etiología desconocida, que se caracteriza por la presencia de aftas bucales, úlceras genitales y uveítis recurrentes. Afecta los sistemas nerviosos central y periférico; raramente se produce durante la niñez. La meningitis aséptica aguda aislada es muy poco frecuente. En este artículo, se describe el caso de una paciente de 14 años con diagnóstico de enfermedad de Behçet con afectación neurológica (neuro- Behçet). La paciente tenía cefalea aguda, diplopia, papiledema e irritación meníngea. Tenía antecedentes de úlceras bucales recurrentes. Las imágenes por resonancia magnética de cerebro revelaron paquimeningitis. Los hallazgos en el líquido cefalorraquídeo fueron pleocitosis y aumento de la presión. Pese al tratamiento médico, sus síntomas no se resolvieron. No se detectó uveítis y la prueba de patergia fue negativa. El alelo HLA-B51 fue positivo. Se consideró que los hallazgos apuntaban a la poco frecuente enfermedad de Behçet con afectación neurológica. La paciente mejoró drásticamente luego del tratamiento con corticoesteroides. En el diagnóstico diferencial de meningitis, se debe considerar la EB, a menos que se demuestre la presencia de un agente infeccioso. Hasta donde sabemos, anteriormente no se había descrito un caso de paquimeningitis con neuro-Behçet en la población pediátrica.

Hydatid disease of the liver with portal vein invasion mimicking portal vein thrombosis.

Hydatid cyst disease is a zoonosis caused by the parasite Echinococcus. It may infest any organ of the body, but it most frequently involves the liver, lungs, and nervous system. Portal vein involvement by hydatid cyst disease is extremely rare with only five cases published in the English literature to our knowledge. We present the ultrasonography (US) and computed tomography (CT) findings of a 77-year-old male with hydatid disease of the liver with portal vein invasion mimicking portal vein thrombosis. Colour Doppler US confirmed the lack of blood flow within the portal vein and stigmata of cavernomatosis. CT clearly demonstrated a communication between the multiloculated lesion and the portal vein and the multiple daughter vesicles obstructing the portal vein. The consideration of this complication will make it possible to distinguish this entity from portal vein thrombosis and, thus, the management of the patients with hydatid cyst disease particulary in endemic regions.

Small Cell Lung Carcinoma Metastasis to Atypical Meningioma: Importance of Perfusion Magnetic Resonance Imaging Graphics in Differential Diagnosis.

We described the imaging findings of small cell lung carcinoma metastasis to atypical meningioma and the importance of magnetic resonance imaging perfusion graphics at the differential diagnosis. To be able to determine subtypes of meningiomas and differentiate whether they are malignant or benign may provide a preoperative idea to determine prognosis and surgical procedure.

An aggressive parameningeal rhabdomyosarcoma with multiple spinal cord metastases: a case report and review of the literature.

PURPOSE: Spinal cord metastasis from rhabdomyosarcoma (RMS) is extremely rare, with three cases reported to date. Herein, we report an aggressive case of RMS of the infratemporal fossa who which developed spinal cord metastases during treatment. CASE PRESENTATION: A 6-year-old girl presented with an enlarging painless mass around her right ear for 3 months. An enhanced magnetic resonance imaging (MRI) revealed a 5 × x4 × x4.5 5 cm mass on her right infratemporal fossa. A tru-cut biopsy was performed, and histopathologic examination revealed the diagnosis of rhabdomyosarcoma. At the time of the diagnosis, cerebrospinal fluid cytology was negative for malignant cells. The patient underwent induction chemotherapy. There was minimal response to chemotherapy, and the patient underwent curative radiotherapy. However, by 12th fraction of RT, the patient developed a progressive weakness on her lower extremity. Spinal MRI revealed multiple gross masses in different parts of the spinal cord. The local radiotherapy was changed toas craniospinal radiotherapy. However, two 2 weeks after the completion of the RT, the patient developed sepsis and expired because of septic shock. CONCLUSION: Parameningeal RMS is a peculiar subgroup of RMS, which needs an aggressive approach. Despite aggressive approach, meningeal spread is the most important cause of the treatment failure. We should keept in mind that during the treatment, there can be meningeal spread towards to either the brain or spinal cord; therefore, we should follow -up the patients closely from this aspect.

Diagnostic accuracy and effective radiation dose of high pitch dual source multidetector computed tomography in evaluation of coronary artery bypass graft patency.

OBJECTIVE: The aim of the study was to evaluate the diagnostic accuracy and effective radiation dose (ERD) of high pitch dual source multidetector computed tomography (MDCT) for coronary artery bypass graft (CABG) patency. MATERIALS AND METHODS: Fourty-five patients who underwent 128 × 2­slice MDCT angiography with a prospective electrocardiogram-triggering, low-dose, high pitch, dual source, flash spiral acquisition mode after CABG surgery were included in the study. The interobserver agreement of the image quality was evaluated with Cohen κ value. The image quality was compared to the heart rates (HRs) using Mann-Whitney U test and to the graft segments using χ(2) test. The findings for the CABG patency on MDCT were compared to those determined on catheter coronary angiography. Dose-length product (DLP) and ERD were compared to the gender, HRs, and body mass index (BMI) of the patients using Kruskall Wallis and Mann-Whitney U tests. RESULTS: A total of 110 grafts and 330 vessel segments were evaluated with a good interobserver agreement (κ = 0.80). The image quality was better in proximal and middle graft segments (p < 0.05), as well as in the patients with low HRs (p < 0.05). High pitch MDCT had the following sensitivity, specificity, positive predictive value, negative predictive value, and diagnostic accuracy for evaluation of graft patency: 92.8, 99.3, 92.8, 99.3 and 98.8 %, respectively. ERD was correlated to the HRs and BMI. CONCLUSIONS: High pitch 128 × 2­slice dual source CT angiography is a noninvasive imaging modality, and it can be safely and effectively used in evaluation of CABG patency with lower radiation dose.

Quantitative Assessment of Left Ventricular Function and Myocardial Mass: A Comparison of Coronary CT Angiography with Cardiac MRI and Echocardiography.

BACKGROUND: The purpose of this study was to compare the left ventricular parameters obtained from multi-detector row computed tomography (MDCT) studies with two-dimensional echocardiography (2DE), and magnetic resonance imaging (MRI), which is accepted as the gold standard in the evaluation of left ventricular functions. The study also aimed to evaluate whether or not there is a relationship between the MR-Argus and CMR tools software programs which are used in post-process calculations of data obtained by MRI. MATERIAL/METHODS: Forty patients with an average age of 51.4±14.9 years who had been scanned with cardiac MDCT were evaluated with cardiac MRI and 2DE. End-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), ejection fraction (EF), cardiac output (CO), and myocardial mass values calculated by MDCT, MRI, and 2DE were compared with each other. Two different MR software programs were used to compare left ventricular functions. The CMR tools LV tutorials method is accepted as the gold standard because it can be used in three-dimensional functional evaluation. The Pearson Correlation and Bland-Altman analysis were performed to compare the results from the two MR methods (MR-Argus and CMR tools) and the results from both the MDCT and the 2DE with the CMR tools results. RESULTS: Strong positive correlations for EF values were found between the MDCT and CMR tools (r=0.702 p<0.001), and between the MR-Argus and CMR tools (r=0.746 p<0.001). The correlation between the 2DE and CMR tools (r=0.449 p<0.004), however, was only moderate. Similar results were obtained for the other parameters. The strongest correlation for ESV, EDV, and EF was between the two MR software programs. The correlation coefficient between the MDCT and CMR tools is close to the correlation coefficient between the two software programs. While the correlation between 2DE and CMR tools was satisfactory for ESV, EDV, and CO values, it was at a moderate level for the other parameters. CONCLUSIONS: Left ventricular functional analysis can be performed easily and reliably with cardiac MDCT used for coronary artery evaluation and it also gives more accurate results than 2DE.