Multiparametric analysis from dynamic susceptibility contrast-enhanced perfusion MRI to evaluate malignant brain tumors.

BACKGROUND AND PURPOSE: Dynamic susceptibility contrast-enhanced (DSC) MR perfusion is a valuable technique for distinguishing brain tumors. Diagnostic potential of measurable parameters derived from preload leakage-corrected-DSC-MRI remains somewhat underexplored. This study aimed to evaluate these parameters for differentiating primary CNS lymphoma (PCNSL), glioblastoma, and metastasis. METHODS: Thirty-nine patients with pathologically proven PCNSL (n = 14), glioblastoma (n = 14), and metastasis (n = 11) were analyzed. Five DSC parameters-relative CBV (rCBV), percentage of signal recovery (PSR), downward slope (DS), upward slope (US), and first-pass slope ratio-were derived from tumor-enhancing areas. Diagnostic performance was assessed using receiver operating characteristic curve analysis. RESULTS: RCBV was higher in metastasis (4.58; interquartile range [IQR]: 2.54) and glioblastoma (3.98; IQR: 1.87), compared with PCNSL (1.46; IQR: 0.29; p = .00006 for both). rCBV better distinguished metastasis and glioblastoma from PCNSL, with an area under the curve (AUC) of 0.97 and 0.99, respectively. PSR was higher in PCNSL (88.11; IQR: 21.21) than metastases (58.30; IQR: 22.28; p = .0002), while glioblastoma (74.54; IQR: 21.23) presented almost significant trend-level differences compared to the others (p≈.05). AUCs were 0.79 (PCNSL vs. glioblastoma), 0.91 (PCNSL vs. metastasis), and 0.78 (glioblastoma vs. metastasis). DS and US parameters were statistically significant between glioblastoma (-109.92; IQR: 152.71 and 59.06; IQR: 52.87) and PCNSL (-47.36; IQR: 44.30 and 21.68; IQR: 16.85), presenting AUCs of 0.86 and 0.87. CONCLUSION: Metastasis and glioblastoma can be better differentiated from PCNSL through rCBV. PSR demonstrated higher differential performance compared to the other parameters and seemed useful, allowing a proper distinction among all, particularly between metastasis and glioblastoma, where rCBV failed. Finally, DS and US were only helpful in differentiating glioblastoma from PCNSL.

Pseudo-eye-of-the-tiger sign in cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS).

The well-known eye-of-the-tiger sign features bilateral and symmetrical changes in the globus pallidus, with a central area of high signal and peripheral low signal on T2-weighted MRI. Although formally considered pathognomonic of pantothenate kinase-associated neurodegeneration (PKAN), there are other neurodegenerative or genetic diseases showing similar findings. Cerebellar ataxia with neuropathy and vestibular areflexia syndrome (CANVAS) is a late-onset ataxia, that was recently associated with biallelic AAGGG repeat expansion in the RFC1 gene. Although its predominant MRI finding is cerebellar atrophy, there may be other less common associated findings. Our aim is to present two cases of CANVAS with associated (pseudo-)eye-of-the-tiger sign, highlighting the possibility of yet another differential diagnosis for this imaging sign.

Imaging Nasal Obstruction: An Objective Evaluation for a Subjective Complaint.

Nasal obstruction (NO) is a common clinical symptom characterized by a subjective sensation of insufficient airflow through the nasal cavity and may result from various factors, including changes in nasal anatomy, inflammatory conditions, tumoral lesions, and other etiologies. While a thorough medical history and physical examination can often identify its cause, imaging is usually necessary to fully understand the problem. Computed tomography (CT) is the primary imaging modality used to evaluate the nasal cavity and paranasal sinuses, allowing for the identification of potential causes and structural abnormalities. However, when soft tissue characterization is required, magnetic resonance imaging (MRI) is also useful. Understanding the anatomical and pathological basis of NO is crucial for accurate diagnosis and appropriate management. Imaging techniques provide valuable information for identifying the underlying causes of NO and guiding treatment decisions. This article reviews the normal anatomy of the nasal cavity and adjacent paranasal sinuses as well as the several conditions that may affect breathing comfort (tumors, inflammatory diseases, bony and cartilaginous anatomical variants, and "nonobstructive" mucosal thickenings), showing their normal presentation on CT and MRI.

Imaging of pediatric skull lytic lesions: A review.

Skull lesions in pediatric population are common findings on imaging and sometimes with heterogeneous manifestations, constituting a diagnostic challenge. Some lesions can be misinterpreted for their aggressiveness, as with larger lesions eroding cortical bone, containing soft tissue components, leading to excessive and, in some cases, invasive inappropriate etiological investigation. In this review, we present multiple several conditions that may present as skull lesions or pseudolesions, organized by groups (anatomic variants, congenital and development disorders, traumatic injuries, vascular issues, infectious conditions, and tumoral processes). Anatomic variants are common imaging findings that must be recognized by the neuroradiologist. Congenital malformations are rare conditions, such as aplasia cutis congenita and sinus pericranii, usually seen at earlier ages, the majority of which are benign findings. In case of trauma, cephalohematoma, growing skull fractures, and posttraumatic lytic lesions should be considered. Osteomyelitis tends to be locally aggressive and may mimic malignancy, in which cases, the clinical history can be the key to diagnosis. Vascular (sickle cell disease) and tumoral (aneurismal bone cyst, eosinophilic granuloma, metastases) lesions are relatively rare lesions but should be considered in the differential diagnosis, in the presence of certain imaging findings. The main difficulty is the differentiation between the benign and malignant nature; therefore, the main objective of this pictorial essay is to review the main skull lytic lesions found in pediatric age, describing the main findings in different imaging modalities (CT and MRI), allowing the neuroradiologist greater confidence in establishing the differential diagnosis, through a systematic and simple characterization of the lesions.

Portal cavernoma in type 1 neurofibromatosis: A fortuitous or causal association?

Neurofibromatosis type 1 (NF-1) is a multisystem genetic disorder affecting the NF1 tumor suppressor gene. Patients typically develop superficial (cutaneous) and internal (plexiform) neurofibromas. The latter may rarely involve the liver locating in the hilum and encasing the portal vessels, leading to portal hypertension. Vascular abnormalities (NF-I vasculopathy) are a well-recognized manifestation of NF-1. Although the pathogenesis is not well-known, NF-1 vasculopathy involves arteries of both peripheral and cerebral territories, with venous thrombosis being exceptionally reported. Portal venous thrombosis (PVT) is the leading cause of portal hypertension in childhood and has been associated with several risk factors. Nevertheless, predisposing conditions remain unknown in more than 50% of the cases. The treatment options are limited, and its management is nonconsensual in the pediatric age. We report the case of a 9-year-old boy with clinically and genetically confirmed NF-1, diagnosed with portal venous cavernoma after an episode of gastrointestinal bleeding. There were no identifiable risk factors for PVT and intrahepatic peri-hilar plexiform neurofibroma was excluded by MRI imaging. To the best of our knowledge, this is the first report of PVT in NF-1. We speculate that NF-1 vasculopathy may have been a pathogenic factor, or instead, it was a fortuitous association.

Brain imaging findings in CLCN2-related leukoencephalopathy.

CLCN2-related leukoencephalopathy is a rare autosomal-recessive disease caused by a loss-of-function mutation in the ClC-2 chloride channel, which is fundamental in ion and water brain homeostasis. With only 31 cases published in the literature, its precise pathophysiology is uncertain, clinical manifestations are nonspecific and little is known in terms of prognosis. Neuroimaging plays a fundamental role in the identification of CLCN2-related leukoencephalopathy, which has a typical magnetic resonance imaging pattern that, when recognized, should promote proper genetic study for diagnostic confirmation. We report a paediatric clinical case of CLCN2-related leukoencephalopathy with genetically verified c.1709G > A p(Trp570*) mutation, highlighting typical neuroimaging findings and the importance of imaging in the diagnostic approach.

Thalamic Deep Brain Stimulation for Neuropathic Pain: Efficacy at Three Years' Follow-Up.

OBJECT: Chronic neuropathic pain is estimated to affect 3-4.5% of the worldwide population, posing a serious burden to society. Deep Brain Stimulation (DBS) is already established for movement disorders and also used to treat some "off-label" conditions. However, DBS for the treatment of chronic, drug refractory, neuropathic pain, has shown variable outcomes with few studies performed in the last decade. Thus, this procedure has consensus approval in parts of Europe but not the USA. This study prospectively evaluated the efficacy at three years of DBS for neuropathic pain. METHODS: Sixteen consecutive patients received 36 months post-surgical follow-up in a single-center. Six had phantom limb pain after amputation and ten deafferentation pain after brachial plexus injury, all due to traumas. To evaluate the efficacy of DBS, patient-reported outcome measures were collated before and after surgery, using a visual analog scale (VAS) score, University of Washington Neuropathic Pain Score (UWNPS), Brief Pain Inventory (BPI), and 36-Item Short-Form Health Survey (SF-36). RESULTS: Contralateral, ventroposterolateral sensory thalamic DBS was performed in sixteen patients with chronic neuropathic pain over 29 months. A postoperative trial of externalized DBS failed in one patient with brachial plexus injury. Fifteen patients proceeded to implantation but one patient with phantom limb pain after amputation was lost for follow-up after 12 months. No surgical complications or stimulation side effects were noted. After 36 months, mean pain relief was sustained, and the median (and interquartile range) of the improvement of VAS score was 52.8% (45.4%) (p = 0.00021), UWNPS was 30.7% (49.2%) (p = 0.0590), BPI was 55.0% (32.0%) (p = 0.00737), and SF-36 was 16.3% (30.3%) (p = 0.4754). CONCLUSIONS: DBS demonstrated efficacy at three years for chronic neuropathic pain after traumatic amputation and brachial plexus injury, with benefits sustained across all pain outcomes measures and slightly greater improvement in phantom limb pain.

Desempeño cardiovascular de dos estirpes de Gallus gallus domesticus sometidas a estrés calórico agudo / Cardiovascular Performance of Two Lineages of Gallus gallus domesticus Subjected to Acute Heat Stress

La selección genética en aves de corral se ha basado en caracteres para crecimiento rápido y no para la adaptabilidad a condiciones estresantes. En esta investigación, se evaluaron variables ambientales y cardiovasculares de dos estirpes de Gallus gallus domesticus con diferentes niveles de domesticación, sometidas a condiciones de estrés calórico agudo. Para la realización del experimento, se utilizaron pollos de engorde comerciales con alto nivel de selección genética (n=40) y pollos criollos (n=40) con bajo nivel de selección. Todos los pollos se criaron bajo las mismas condiciones hasta el día 36 de edad, día en que se evaluó: temperatura ambiental, humedad relativa, temperatura corporal, frecuencia cardiaca, volumen sistólico y gasto cardiaco. El día 37, ambos grupos de animales fueron expuestos a una simulación de estrés calórico agudo (34°C), midiéndose las variables antes mencionadas. Los datos fueron analizados como medidas repetidas (PROC MIXED de SAS, 2005), bajo un diseño de bloques. El día 37, las temperaturas corporales de los pollos comerciales (46,1±0,2°C) fue significativamente mayor (P £ 0,01) que las de los criollos (43,5±0,9°C). El incremento en la frecuencia cardiaca (38±1 lat/min) de los pollos comerciales fue mayor (P ≤0,01), en contraste con la de los pollos criollos (23±0 lat/min), cuando comparamos con el día anterior. De igual modo, cuando se hace la comparación con respecto al día anterior (día 36), el volumen sistólico (0,33±0,03 mL/lat) y el gasto cardiaco (38,50±3,3 ml/min) y disminuyeron (P>0,05) en los pollos comerciales; en contraste, estas variables (volumen sistólico: 0,09±0,00 mL/lat; gasto cardiaco: 59,10±4,8 mL/ min) aumentaron en los criollos. La relación gasto cardiaco sobre peso vivo fue mayor (P≤0,01) en los pollos criollos (0,81±0,03 mL/min/g) con respecto a los comerciales (0,40±0,07 mL/min/g). Estos hallazgos sugieren que el desempeño cardiaco de la estirpe comercial fue menos eficiente, en relación al desempeño cardiaco de la estirpe criolla, frente al estrés calórico, en comparación con los pollos criollos y que estos últimos se encuentran mejor adaptados para hacer frente a situaciones de estrés calórico ambiental.

Genetic selection in poultry has been based on fast growth characters and not on stress adaptability. This investigation assessed cardiovascular variables in two lineages of Gallus gallus domesticus of different domestication levels, subjected to acute heat stress. To carry out the assay, broiler chickens (n=40) with a high-end level of genetic selection and creole chickens (n=40) with a low-end level of genetic selection were evaluated. All chickens were reared under the same conditions until day 36 of age, at which day, the following variables were measured: environmental temperature, relative humidity, body temperature, heart rate, systolic volumen, and cardiac output. On day 37, both flocks were exposed to an acute environmental heat stress simulation (34 ºC). Data were analyzed with repetitive measures test (PROC MIXED, SAS, 2005), with a two-way arrangement. During the heat stress simulation, body temperature of broilers (46.1±0.2°C) was higher (P≤0,01) than that of creole chickens (43.5±0.9°C); likewise, the increase in broilers heart rate (38±1 beats/min) was higher (P≤0,01), when compared to the increase in creole chickens (23±0 beats/min) from the day before. Similarly, a lower (P>0,05) systolic volume (0.33±0.03 mL/beats) and cardiac output (38.5±3.3 mL/min) was registered in broilers in contrast to an increment of these variables (systolic volume: 0.09±0.00 mL/beats; cardiac output: 59.10±4.8 mL/min) in creoles. Cardiac output to body weight ratio was higher (P≤0,01) in creole chickens (0.81±0.03 mL/min/g) in comparison to broilers (0.40 ± 0.07 mL/min/g). These results suggest that chickens from a commercial lineage (broilers) were less effective in terms of cardiac performance, under environmental heat stress, when compared to creole chickens, suggesting that creole chickens are better adapted to cope with environmental heat stress.