Visualization of the Facial Nerve with Ultra-high-Frequency Ultrasound.

Background: Profound variations in facial nerve branching, combined with the severe impact of facial palsy on the patient's quality of life, make surgery in this region challenging. Recent advancements in ultrasound (US) technology, including the improved visualization of small structures, have led to a sharp increase in its medical indications in various medical disciplines. We aimed to prove the feasibility of using ultra-high-frequency (UHF) US to visualize the facial nerve and to guide surgeons during surgery on and around the facial nerve. Methods: A cadaveric study was performed on one hemi-face with a UHF US imaging system and state-of-the-art transducers. Firstly, a transcutaneous US was performed, and the facial nerve branches of interest (zygomatic, buccal, and marginal mandibular branches) were marked using US-guided color-injections of filler mixed with methylene blue. Skin and subcutaneous fat were then removed to simulate the intraoperative field. Secondly, an "intraoperative" US examination was performed, and the same branches were marked by US-guided color-injections of filler mixed with indocyanine green. Anterograde facial nerve dissection was performed, and the distance between the nerve branches and the injected filler was measured. Results: All color-injections (mixed with both methylene blue and indocyanine green) were positioned right next to the nerve branches (<1 mm). The image quality of the US below the skin was observed to be far superior to that of the transcutaneous US. Conclusion: UHF US can be used to visualize the facial nerve with high precision both transcutaneously and intraoperatively (after elevation of the skin flap).

Ultrasound-guided core needle biopsy and incisional biopsy of the parotid gland are comparable in diagnosis of primary Sjögren's syndrome.

OBJECTIVES: Salivary gland lymphocytic infiltrates are a hallmark of primary SS (pSS), but traditional biopsy techniques hold several disadvantages. Ultrasound-guided core needle (US-guided CN) parotid gland biopsy is minimally invasive and reliable for diagnosis of lymphoma in pSS. This proof-of-concept study aimed to explore this technique in the diagnostic work-up of pSS and is the first to address its value in a consecutive cohort independently of the presence of salivary gland swelling. METHODS: Combined incisional and US-guided CN parotid biopsy was performed in 20 patients with suspected or confirmed pSS from the Belgian Sjögren's Syndrome Transition Trial (BeSSTT). Surface area and presence of a focus score (FS) of at least one, germinal centres and lymphoepithelial lesions were recorded. RESULTS: Salivary gland tissue was interpretable in 19 patients. Fourteen patients had ≥4 mm2 salivary gland tissue by both techniques, in four US-guided CN biopsies salivary gland tissue was <4 mm2. Paired biopsies ≥4 mm2 displayed a concordance of 90% for FS ≥ 1. Presence of lymphoepithelial lesions and germinal centres showed absolute concordance. Of four US-guided CN biopsies <4 mm2, three interpretable incisional biopsies were available, 2/3 with perfect concordance. When including biopsies of <4 mm2 salivary gland tissue, presence of FS ≥ 1 or germinal centres gave a sensitivity of 70% in incisional and of 69% in US-guided CN biopsy. CONCLUSIONS: US-guided CN biopsy of the parotid gland is at least equivalent to incisional biopsy of the parotid gland in the diagnostic work-up of pSS.

The value of separate detection of anti-Ro52, anti-Ro60 and anti-SSB/La reactivities in relation to diagnosis and phenotypes in primary Sjögren's syndrome.

OBJECTIVES: Autoantibody detection is an essential step in pSS diagnosis. However, the value of separate anti-Ro52, anti-Ro60 and anti-SSB/La detection in pSS diagnosis and phenotyping has not been extensively studied. This study aimed to explore disease characteristics of anti-SSA/Ro positive, suspected and definite pSS patients, in relation to serological profiles based on anti-Ro52, anti-Ro60 and anti-SSB/La reactivity. METHODS: Of 187 anti-SSA/Ro positive patients included in the Belgian Sjögren's Syndrome Transition Trial (BeSSTT), 155 were considered definite pSS patients, due to fulfilment of the 2016 ACR-EULAR classification criteria, and 32 suspected, due to reactivity against SSA/Ro without presence of other criteria. None of the patients met any of the ACR-EULAR exclusion criteria for pSS. Patients were grouped based on the presence of anti-Ro52, anti-Ro60 and anti-SSB/La antibodies. RESULTS: Mono-reactivity against Ro60 or Ro52, double reactivity against Ro52/Ro60 and triple reactivity against Ro52/Ro60 and SSB was detected in respectively 30, 23, 70 and 60 patients. Mono-anti-Ro60 positive patients showed the least pSS features. Mono-anti-Ro52 positive patients reported a significantly higher dryness burden (p=0.016) and tended toward more salivary gland ultrasound (SGUS) abnormalities (p=0.054) than mono-anti-Ro60 positives. Double positive patients showed similar characteristics as mono-anti-Ro52 positive patients, whereas triple positive patients showed lowest unstimulated salivary flow rates (p=0.002) and Schirmer tests (p=0.002), highest ocular staining scores (p<0.001), most positive labial salivary gland biopsies (p=0.039), most laboratory abnormalities compatible with B-cell hyperactivity and highest SGUS scores (p<0.001) compared to other patient groups. CONCLUSIONS: These data indicate that separate detection of anti-Ro52, anti-Ro60 and anti-SSB/La reactivity is not only relevant towards pSS diagnosis, but markedly aids in patient stratification and evaluation of disease burden. Our results suggest a stepwise model in which mono-reactivity against Ro60 displayed the least objective and subjective glandular pSS features, whereas glandular abnormalities and signs of B-cell hyperactivity were most present in patients showing triple reactivity against Ro60, Ro52 and SSB/La.

Discriminative power of salivary gland ultrasound in relation to symptom-based endotypes in suspected and definite primary Sjögren's Syndrome.

OBJECTIVES: Salivary gland ultrasound (SGUS) is emerging as essential tool in primary Sjögren's Syndrome (pSS), but its link to symptom-based endotypes is unknown. Therefore, we explored SGUS outcomes in relation to endotypes in patients with definite and suspected pSS. METHODS: Definite pSS patients (n = 171) fulfilling the 2016 ACR/EULAR classification criteria, and suspected pSS patients (n = 119), positive for at least one criterion, were included in the Belgian Sjögren's Syndrome Transition Trial (BeSSTT). Stratification into endotypes according to the Newcastle Sjögren's Stratification Tool resulted in low symptom burden (LSB), pain dominant with fatigue (PDF), dryness dominant with fatigue (DDF) and high symptom burden (HSB). SGUS was assessed with Hocevar score (0-48). The dataset was randomly divided into a discovery (n = 203) and replication (n = 87) cohort. RESULTS: SGUS had strong discriminative power for pSS classification (AUC=0.74), especially in DDF (AUC=0.89). In definite pSS, Hocevar scores in DDF were high compared to other endotypes (38 (20-44) versus 18 (9-33); p < 0.001). Patients with highest SGUS-scores showed more sicca and laboratory abnormalities. Moreover, a subset of young, anti-SSA/Ro positive patients not fulfilling classification criteria showed clear SGUS abnormalities. Replication showed similar results. CONCLUSIONS: SGUS-scores were significantly higher in definite pSS with DDF endotype, providing the first evidence of imaging abnormalities in salivary glands matching distinct biological profiles ascribed to pSS endotypes. Additionally, a subset of patients with potential early disease was detected based on presence of anti-SSA antibodies and high SGUS-scores. These results underscore the role of SGUS as powerful tool both in pSS classification and stratification.

Moyamoya Disease Revealed by Ruptured Intraventricular Aneurysm.

Teaching point: Narrowing and occlusion of the distal carotids in Moyamoya causes a change in blood flow dynamics, increasing the risk for intracranial aneurysm formation.

Is diffuse axonal injury on susceptibility weighted imaging a biomarker for executive functioning in adolescents with traumatic brain injury?

Traumatic brain injury (TBI) is a heterogeneous disorder in which diffuse axonal injury (DAI) is an important component contributing to executive dysfunction. During adolescence, developing brain networks are especially vulnerable to acceleration-deceleration forces. We aimed to examine the correlation between DAI (number and localization) and executive functioning in adolescents with TBI. We recruited 18 adolescents with a mean age of 15y8m (SD = 1y7m), averaging 2.5 years after sustaining a moderate-to-severe TBI with documented DAI. Susceptibility Weighted Imaging sequence was administered to localize the DAI lesions. The adolescents performed a neurocognitive test-battery, addressing different aspects of executive functioning (working memory, attention, processing speed, planning ability) and their parents completed the Behavior Rating Inventory of Executive Function (BRIEF) - questionnaire. Executive performance of the TBI-group was compared with an age and gender matched control group of typically developing peers. Based on these results we focused on the Stockings of Cambridge test and the BRIEF to correlate with the total number and location of DAI. Results revealed that the anatomical distribution of DAI, especially in the corpus callosum and the deep brain nuclei, may have more implications for executive functioning than the total amount of DAI in adolescents. Results of this study may help guide targeted rehabilitation to redirect the disturbed development of executive function in adolescents with TBI.