Terminology for contrast-enhanced sonography: a practical glossary.

Objective. The purpose of this glossary is to offer an updated guide to the correct terminology for contrast-enhanced sonography. Methods. This report was prepared by a panel of radiologists from the Sonography Section of the Italian Association of Medical Radiology. A leading author prepared a list of terms based on a comprehensive literature survey. The draft was analyzed by 3 experts on the topic of contrast-enhanced sonography. These reviewers reached a consensus and prepared the final version. Results. A list of 137 terms is included. These terms are briefly defined. Their proper application is discussed, with special reference to potential misleading uses. Conclusions. Contrast-enhanced sonography is a relatively new diagnostic tool, now entering clinical practice in several countries. Use of appropriate, universal terminology is mandatory in the scientific setting to allow comparison between different published experiences. Additionally, use of clear, standardized terminology is necessary in the clinical setting to facilitate report understanding by the referring physician. Standardized, nonequivocal nomenclature may also help future diffusion of sonographic contrast media in countries where their application is still not approved

MRI cortical feature of bulbar impairment in patients with amyotrophic lateral sclerosis

The decline of voluntary bulbar functions such as speech and swallowing are among the clinical manifestations of amyotrophic lateral sclerosis (ALS) influencing a worst prognosis. Differential diagnosis between the contribution of upper motor neuron (UMN) and lower motor neuron degeneration to the bulbar impairment is often hard. Thinning and T2* hypointensity of the primary motor cortex have been recently suggested as possible MRI markers of UMN impairment in ALS patients, but little research has purposely targeted the orofacial region of the primary motor cortex (fM1). With the aim of finding an MRI marker of UMN impairment responsible for bulbar dysfunction, we investigated the T2* signal intensity of fM1 and the relationship with bulbar impairment in ALS patients. Fifty-five ALS patients were examined with 3 T MRI. Their fM1 was evaluated both qualitatively in terms of T2* signal intensity and quantitatively by measuring its magnetic susceptibility with Quantitative Susceptibility Mapping (QSM). Bulbar functions were assessed clinically, by neurological examination and using the items 1–3 of the ALSFRS-R, and with neurophysiological tests. The marked hypointensity of fM1 was detected in 25% of ALS patients, including all patients with bulbar onset, and was 74% sensitive, 100% specific and 91% accurate in diagnosing functional bulbar impairment. Such hypointensity involved the middle and ventral part of fM1 and was usually visible in both hemispheres. The magnetic susceptibility was significantly higher in patients with marked fM1 hypointensity than in the other patients (p ≤ .001). The relationship with clinical and neurophysiological data suggests that such feature could be a marker of UMN degeneration for voluntary bulbar functions

ESPGHAN Revised Porto Criteria for the Diagnosis of Inflammatory Bowel Disease in Children and Adolescents.

BACKGROUND:: The diagnosis of pediatric-onset IBD (PIBD) can be challenging in choosing the most informative diagnostic tests and correctly classifying PIBD into its different subtypes. Recent advances in our understanding of the natural history and phenotype of PIBD, increasing availability of serological and fecal biomarkers, and the emergence of novel endoscopic and imaging technologies taken together have made the previous Porto criteria for the diagnosis of PIBD obsolete. METHODS:: We aimed to revise the original Porto criteria utilizing an evidence-based approach and consensus process to yield specific practice recommendations for the diagnosis of PIBD. These revised criteria are based on the Paris classification of PIBD and the original Porto criteria whilst incorporating novel data, such as for serum and fecal biomarkers. A consensus of at least 80% of participants was achieved for all recommendations and the summary algorithm. RESULTS:: The revised criteria depart from existing criteria by defining two categories of ulcerative colitis (UC; typical and atypical); atypical phenotypes of UC should be treated as UC. A novel approach based on multiple criteria for diagnosing IBD-unclassified (IBDU) is proposed. Specifically, these revised criteria recommend upper GI endoscopy and ileocolonscopy for all suspected PIBD patients, with small bowel imaging (unless typical UC after endoscopy and histology) by magnetic resonance enterography (MRE) or wireless capsule endoscopy. CONCLUSIONS:: These revised Porto criteria for the diagnosis of PIBD have been developed to meet current challenges and developments in PIBD and provide up to date guidelines for the definition and diagnosis of the IBD spectrum

Studio di Risonanza Magnetica stutturale e funzionale in pazienti affetti da Distrofia Miotonica tipo I

La distrofia miotonica di tipo 1 (DM1) è la forma più frequente di distrofia muscolare dell’età adulta ed caratterizzata da un interessamento multisistemico a carico della muscolatura scheletrica e degli apparati cardiaco, oculare, endocrino, gastrointestinale e del sistema nervoso centrale (SNC). La malattia, di natura genetica, è trasmessa come tratto autosomico dominante ed è dovuta ad una abnorme espansione della tripletta CTG del gene che codifica per una proteina kinasi (DMPK), localizzato sul cromosoma 19q13.3. Non sono ad oggi ancora chiare le basi patogenetiche della malattia. La malattia presenta di frequente un profilo tipico di disturbi a carico del SNC, caratterizzato da un andamento cronico e progressivo. Nella pratica clinica emerge frequentemente che i pazienti presentino una compromissione della capacità di avere una chiara e completa consapevolezza della propria condizione di malattia. Tale disturbo, definito anche con il nome di anosognosia, è una condizione psicologica riscontrabile in varie patologie neurologiche di natura organica o neurodegenerativa, le cui basi neuroanatomiche e neuropsicologiche sono complesse e ad oggi non del tutto note. Allo stato attuale delle conoscenze, non è ancora stato definito un protocollo ottimale per indagare la compromissione del SNC nei pazienti affetti da DM1, sia per quel che riguarda la valutazione neuropsicologica che quella neuroradiologica. Nonostante i numerosi studi di risonanza magnetica sulle alterazioni del SNC di questi soggetti, la frequenza e localizzazione di tali anomalie, nonché la loro relazione con il profilo cognitivo, l’età d’esordio, la durata del decorso e il profilo genetico rimangono controverse. L’anosognosia genera nei pazienti un’ammissione parziale delle loro difficoltà, che interferisce con la sua adattabilità ai trattamenti e nella relazione con le figure di accudimento. La mancanza di consapevolezza circa il proprio stato di malattia è stata già studiata in altre patologie neurologiche di origine organica o neurodegenerativa, ma mai nei pazienti affetti da DM1. In questo studio, 20 pazienti affetti da DM1 sono stati valutati dal punto di vista clinico, neuropsicologico e neuroradiologico per individuare eventuali correlazioni fra questi aspetti. È emerso che una percentuale elevata di pazienti con DM1 ha una compromissione delle funzioni frontali ed esecutive e che molti di essi hanno una scarsa consapevolezza di malattia. Le lesioni della sostanza bianca tipiche della DM1 sono una caratteristica che sembra disgiunta dall’atrofia cerebrale e dalla compromissione neuropsicologica. L’atrofia cerebrale a prevalente distribuzione fronto temporale sembra correlare invece con un profilo neuropsicologico indicativo di compromissione di tali strutture cerebrali. La valutazione funzionale mediante RM indica una ridotta attivazione di aree fronto cingolate mesiali nei pazienti con DM1 che hanno una ridotta consapevolezza di malattia

MR features of upper motor neuron impairment in amyotrophic lateral sclerosis

Purpose – To investigate both sensitivity and accuracy of T2*-weighted images of primary motor cortex in revealing patients affected by ALS. To find with quantitative susceptibility mapping a susceptibility cut-off value which had the best sensitivity and specificity at detecting upper motor neuron burden. To evaluate further relationships between imaging and clinical data. Material and methods - 74 patients and 17 controls were investigated on a 3T MR scanner. Protocol included T2*-weighted acquisitions for both qualitative and quantitative imaging, by obtaining QSM. Magnetic susceptibility was measured within different rolandic regions. Data were compared with clinical assessment of disease in patients. Results - Based on qualitative images alone, 2 blind readers could identify most clinically compromised patients with a high interobserver agreement. A magnetic susceptibility cut-off value of 0,064 ppm was set as threshold to differentiate effectively patients from healthy controls. Appearance on qualitative imaging and susceptibility magnetic values of specific regions in primary motor cortex varied accordingly to symptomatic body districts. Conclusions - T2*-weighted qualitative imaging is a reliable way to assess UMN disease at 3T MRI. T2* hypointensity distribution within primary motor cortex is related to the localization of clinical symptoms. QSM is capable of measuring magnetic susceptibility values in affected cortical regions, identifying ALS patients with great accuracy