This study aims to analyze the changes in dermal thickness in patients with systemic scleroderma (SSc) in comparison with normal skin and also compare clinical forms with diffuse and limited cutaneous involvement. The study group consisted of female patients diagnosed with SSc with a disease history not exceeding 5 years. The areas of interest for ultrasound examination included the proximal phalanx of the third finger, the second intermetacarpal space, and the extension surface of the lower third of the forearm. The study included 20 patients diagnosed with SSc and 14 controls. SSc patients were subdivided into two subgroups based on the clinical form. Compared to the control group, patients with SSc had higher mean measurements in all three skin areas, with statistically significant differences in the hand and forearm areas. Patients with diffuse SSc displayed, on average, higher skin thickness compared to limited SSc in all skin areas examined, with a statistically significant difference only in the forearm area. Based on disease manifestations, significant differences were observed only with regard to the presence of pulmonary hypertension in the diffuse SSc group. In conclusion, skin ultrasound is a useful and accessible imaging method for diagnosing and quantifying dermal fibrosis in systemic scleroderma.

OBJECTIVE: This study was undertaken to determine whether hippocampal T2 hyperintensity predicts sequelae of febrile status epilepticus, including hippocampal atrophy, sclerosis, and mesial temporal lobe epilepsy.
METHODS: Acute magnetic resonance imaging (MRI) was obtained within a mean of 4.4 (SD = 5.5, median = 2.0) days after febrile status on >200 infants with follow-up MRI at approximately 1, 5, and 10 years. Hippocampal size, morphology, and T2 signal intensity were scored visually by neuroradiologists blinded to clinical details. Hippocampal volumetry provided quantitative measurement. Upon the occurrence of two or more unprovoked seizures, subjects were reassessed for epilepsy. Hippocampal volumes were normalized using total brain volumes.
RESULTS: Fourteen of 22 subjects with acute hippocampal T2 hyperintensity returned for follow-up MRI, and 10 developed definite hippocampal sclerosis, which persisted through the 10-year follow-up. Hippocampi appearing normal initially remained normal on visual inspection. However, in subjects with normal-appearing hippocampi, volumetrics indicated that male, but not female, hippocampi were smaller than controls, but increasing hippocampal asymmetry was not seen following febrile status. Forty-four subjects developed epilepsy; six developed mesial temporal lobe epilepsy and, of the six, two had definite, two had equivocal, and two had no hippocampal sclerosis. Only one subject developed mesial temporal epilepsy without initial hyperintensity, and that subject had hippocampal malrotation. Ten-year cumulative incidence of all types of epilepsy, including mesial temporal epilepsy, was highest in subjects with initial T2 hyperintensity and lowest in those with normal signal and no other brain abnormalities.
CONCLUSIONS: Hippocampal T2 hyperintensity following febrile status epilepticus predicted hippocampal sclerosis and significant likelihood of mesial temporal lobe epilepsy. Normal hippocampal appearance in the acute postictal MRI was followed by maintained normal appearance, symmetric growth, and lower risk of epilepsy. Volumetric measurement detected mildly decreased hippocampal volume in males with febrile status.

BACKGROUND: Progressive systemic sclerosis or systemic scleroderma (SS) is a chronic and rare autoimmune disease that mainly affects the skin and various internal organs. Raynaud\'s phenomenon and digital ulcers are some of the symptoms that affect the foot, causing a decrease in the quality of life of patients. The objective of this study is to determine the functionality of the feet in patients with SS and determine the impact on their daily lives.
METHODS: A sample of 165 patients (154 women, 11 men) diagnosed with SS with a mean age of 46.29 ± 11.36 years and a mean body mass index (BMI) of 24.90 ± 5.77 was recruited. Each participant completed the Foot Function Index (FFI) questionnaire and the Systemic Sclerosis Questionnaire (SySQ). A multivariate analysis was performed to determine which factors were related to a higher score in both questionnaires.
RESULTS: 32.1% of the participants (n = 53) had claw toe deformities, 79.4% (n = 131) Raynaud\'s disease and 20% (n = 33) a history of foot ulcers. 51.5% of the participants (n = 85) presented symptoms in their nails, the most frequent sign being thickening, hardening and yellow coloration. The final score of the FFI questionnaire was 3.51 ± 2.41 (0-9.9), the pain subscale being the highest, with a score of 5.06 ± 2.75, followed by foot disability (3.26 ± 2.91) and difficulty performing activities (1.55 ± 2.22). The final score of the SySQ questionnaire was 0.95 ± 0.45 (0.18-2.45), and the subscales with the highest score were symptom frequency (1.30 ± 0.47), symptom intensity (1.11 ± 0.55), and general skill limitation (0.47 ± 0.51). A high correlation was observed between the final FFI score and the final SySQ score (r = 0.712; p=<0.001). Also, between foot activity limitation and general skill limitation (r = 0.658; p=<0.001). A moderate correlation was observed between foot pain score and overall symptom intensity (r = 0.482; p=<0.001). Also, between foot disability and overall symptom frequency (r = 0.556; p=<0.001). The multivariate analysis (R2 0.51) showed that the final FFI score had a significant relationship with the final SySQ score (p < 0.001). No significant correlation was found between age (p = 0.15), gender (p = 0.49), BMI (p = 0.74) or time of diagnosis (p = 0.57) and FFI.
CONCLUSIONS: SS is a disease that affects foot functionality in patients, with a greater impact on the pain scale. There is a correlation between the final FFI score and the final SySQ score, so improving foot functionality could help to improve the overall functionality of the patient with sclerosis.

OBJECTIVE: Epilepsy patients are often grouped together by clinical variables. Quantitative neuroimaging metrics can provide a data-driven alternative for grouping of patients. In this work, we leverage ultra-high-field 7-T structural magnetic resonance imaging (MRI) to characterize volumetric atrophy patterns across hippocampal subfields and thalamic nuclei in drug-resistant focal epilepsy.
METHODS: Forty-two drug-resistant epilepsy patients and 13 controls with 7-T structural neuroimaging were included in this study. We measured hippocampal subfield and thalamic nuclei volumetry, and applied an unsupervised machine learning algorithm, Latent Dirichlet Allocation (LDA), to estimate atrophy patterns across the hippocampal subfields and thalamic nuclei of patients. We studied the association between predefined clinical groups and the estimated atrophy patterns. Additionally, we used hierarchical clustering on the LDA factors to group patients in a data-driven approach.
RESULTS: In patients with mesial temporal sclerosis (MTS), we found a significant decrease in volume across all ipsilateral hippocampal subfields (false discovery rate-corrected p [pFDR] < .01) as well as in some ipsilateral (pFDR < .05) and contralateral (pFDR < .01) thalamic nuclei. In left temporal lobe epilepsy (L-TLE) we saw ipsilateral hippocampal and some bilateral thalamic atrophy (pFDR < .05), whereas in right temporal lobe epilepsy (R-TLE) extensive bilateral hippocampal and thalamic atrophy was observed (pFDR < .05). Atrophy factors demonstrated that our MTS cohort had two atrophy phenotypes: one that affected the ipsilateral hippocampus and one that affected the ipsilateral hippocampus and bilateral anterior thalamus. Atrophy factors demonstrated posterior thalamic atrophy in R-TLE, whereas an anterior thalamic atrophy pattern was more common in L-TLE. Finally, hierarchical clustering of atrophy patterns recapitulated clusters with homogeneous clinical properties.
CONCLUSIONS: Leveraging 7-T MRI, we demonstrate widespread hippocampal and thalamic atrophy in epilepsy. Through unsupervised machine learning, we demonstrate patterns of volumetric atrophy that vary depending on disease subtype. Incorporating these atrophy patterns into clinical practice could help better stratify patients to surgical treatments and specific device implantation strategies.

UNASSIGNED: Morphea is a rare disease of unknown etiology without satisfactory treatment for skin sclerosis and soft tissue atrophy.
UNASSIGNED: To provide clinical, histologic, and transcriptome evidence of the antisclerotic and regenerative effects of sequential fat grafting with fresh fat and cryopreserved stromal vascular fraction gel (SVF gel) for morphea.
UNASSIGNED: This single-center, nonrandomized controlled trial was conducted between January 2022 and March 2023 in the Department of Plastic and Reconstructive Surgery of Nanfang Hospital, Southern Medical University and included adult participants with early-onset or late-onset morphea who presented with varying degrees of skin sclerosis and soft tissue defect.
UNASSIGNED: Group 1 received sequential grafting of fresh fat and cryopreserved SVF gel (at 1 and 2 months postoperation). Group 2 received single autologous fat grafting. All patients were included in a 12-month follow-up.
UNASSIGNED: The primary outcome included changes in the modified Localized Scleroderma Skin Severity Index (mLoSSI) and Localized Scleroderma Skin Damage Index (LoSDI) scores as evaluated by 2 independent blinded dermatologists. The histologic and transcriptome changes of morphea skin lesions were also evaluated.
UNASSIGNED: Of 44 patients (median [IQR] age, 26 [23-33] years; 36 women [81.8%]) enrolled, 24 (54.5%) were assigned to group 1 and 20 (45.5%) to group 2. No serious adverse events were noted. The mean (SD) mLoSSI scores at 12 months showed a 1.6 (1.50) decrease in group 1 and 0.9 (1.46) in group 2 (P = .13), whereas the mean (SD) LoSDI scores at 12 months showed a 4.3 (1.34) decrease in group 1 and 2.1 (1.07) in group 2 (P < .001), indicating that group 1 had more significant improvement in morphea skin damage but not disease activity compared with group 2. Histologic analysis showed improved skin regeneration and reduced skin sclerosis in group 1, whereas skin biopsy specimens of group 2 patients did not show significant change. Transcriptome analysis of skin biopsy specimens from group 1 patients suggested that tumor necrosis factor α signaling via NFκB might contribute to the immunosuppressive and antifibrotic effect of sequential fat grafting. A total of 15 hub genes were captured, among which many associated with morphea pathogenesis were downregulated and validated by immunohistochemistry, such as EDN1, PAI-1, and CTGF.
UNASSIGNED: The results of this nonrandomized trial suggest that sequential fat grafting with fresh fat and cryopreserved SVF gel was safe and its therapeutic effect was superior to that of single autologous fat grafting with improved mLoSSI and LoSDI scores. Histological and transcriptomic changes further support the effectiveness after treatment.
UNASSIGNED: Chinese Clinical Trial Registry identifier: ChiCTR2200058003.

OBJECTIVE: Stereo-electroencephalography-guided three-dimensional radiofrequency thermocoagulation (SEEG-3D RFTC) is a minimally invasive treatment for mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). This study aimed to investigate the long-term prognosis after SEEG-3D RFTC treatment in patients with MTLE-HS.
METHODS: This single-center retrospective study included 28 patients with MTLE-HS treated with SEEG-3D RFTC from January 2016 to May 2018. Postoperative curative effects were evaluated using the Engel classification, and the patients were followed up for 5 years.
RESULTS: The proportions of patients categorized as Engel I between 1 and 5 years after surgery were 72.41% (12 months after surgery), 67.86% (18 months after surgery), 62.07% (24 months after surgery), 50.00% (36 months after surgery), 42.86% (48 months after surgery), and 42.86% (60 months after surgery), respectively. Regarding long-term efficacy, based on the Engel classification, SEEG-3D RFTC showed room for improvement.
CONCLUSIONS: This was the first study to evaluate the efficacy of SEEG-3D RFTC for MTLE-HS with long-term follow-up. SEEG-3D RFTC is a promising alternative for patients with MTLE-HS.
CONCLUSIONS: This study explored the potential of stereoelectroencephalography-guided three-dimensional radiofrequency thermocoagulation, a minimally invasive approach, for treating medial temporal lobe epilepsy with hippocampal sclerosis. Involving 28 patients, the research tracked the treatment\'s success over five years using the Engel classification. Initial results were promising, with 72.41% of patients achieving the most favorable outcome (Engel I) at one year. While there was a gradual decrease in this proportion over time, 42.86% of patients maintained this positive outcome at five years, highlighting the treatment\'s potential for long-term efficacy.

OBJECTIVE: The aim of this study is to evaluate the changes in the temporomandibular joint (TMJ) in patients with temporomandibular disorder (TMD) and the relationship between age, sex, and types of TMJ change using Cone Beam Computed Tomography (CBCT).
METHODS: CBCT records of 200 patients (123 women and 67 men) were retrieved and assessed. Right and left TMJs were evaluated separately, resulting in a total of 400 TMJs. The images were analyzed using On demand 3D Application The radiographic findings were classified as erosion, proliferative changes mainly, including flattening and osteophytes of the condyle, sclerosis, Ely cyst, hypoplasia and hyperplasia of the condyles, ankylosis, and joint cavity. Data analysis was performed using descriptive statistics, paired T-tests, and repeated measure ANOVA (Analysis of Variance) in SPSS Software.
RESULTS: The most prevalent types of condylar bony changes observed was osteophyte (63.5%) followed by flattening of the articular surface (42%), erosion (40%), ankylosis (10%) and sclerosis (10%). 7.5% of joints showed hyperplastic condyles but only 2% showed hypoplasia. The least prevalent change observed was Ely Cyst (1%). Osteophyte was the most prevalent change observed in all age groups and both sexes except for men aged 31 ~ 50, where flattening was more frequent. A statistically significant difference was found between sex and prevalence of erosion in the age group of 10 ~ 30 (P = 0.001); as well as between sex and condylar hyperplasia in the same age group.
CONCLUSIONS: Based on the findings of this research, the prevalence of bony changes of TMJ from highest to lowest is as follows: osteophyte, flattening of the articular surface, erosion, ankylosis, sclerosis, hyperplastic condyles, hypoplastic condyles and Ely Cyst. CBCT is an accurate 3 dimensional imaging modality for assessment of TMJ bony structures.

OBJECTIVE: This cross-sectional study aimed to evaluate the presence of bone changes on magnetic resonance imaging (MRI) scans of patients with temporomandibular joint (TMJ) anterior disc displacement (ADD) with reduction (ADDWR) and without reduction (ADDWoR).
METHODS: TMJ-MRI scans were screened for the presence of ADD. 285 scans presented ADD, being further divided into ADDWR (n = 188) and ADDWoR (n = 97). Bone changes on the mandibular condyle and articular eminence were also assessed and computed. The chi-square test compared the association of these bone changes with the presence of ADDWR and ADDWoR, with a significance level of 5 %. Also, the prevalence ratio (PR) was calculated.
RESULTS: In the mandibular condyle, subchondral cyst (p = 0.035, PR = 1.08) and bone edema (p = 0.044, PR = 2.40), more prevalent on ADDWR, and generalized sclerosis (p = 0.015, PR = 1.04), more prevalent on ADDWoR, presented significant association with ADD. On the articular eminence, generalized sclerosis (p = 0.015, PR = 1.04) and articular surface flattening (p = 0.003, PR = 1.19) presented significant association with ADD, both more prevalent on ADDWoR.
CONCLUSIONS: Bone changes are usual findings in TMJ with ADD diagnosis. The real influence of ADD is not fully clear, although clinicians should be aware of patients with this condition, to provide an early diagnosis and improve patient´s prognosis.

In this project, the possibility of drug delivery application of three anti-Multiple sclerosis (MS) agents (containing diroximel fumarate (DXF), dimethyl fumarate (DMF), and mono methyl fumarate (MMF)) by using some heteroatom decorated graphitic carbonitride (g-C3N4) (as nano-sized carriers) have been systematically investigated. The results of the study have indicated that As-g-C3N4 QD is not a suitable candidate for drug delivery (at least in the cases of DMF, and DXF drugs); while, it would be an accurate semiconductor sensor for selective detection of each mentioned agents. Also, the use of the P-doped as well as pristine g-C3N4 QD could lead to weak electronic signals with relatively same values (in electronvolts). It means that P-g-C3N4, and g-C3N4 QDs are not good sensors for detection of each of the three considered drugs. However, those two sorbents would be suitable carriers for delivering of all three mentioned pharmaceuticals.

BACKGROUND: Stiff skin syndrome (SSS) is a rare disease characterized by sclerosis of the skin. Cases of both widespread and segmental SSS have been reported.
OBJECTIVE: To report the clinical and histopathological characteristics of a large series of SSS.
METHODS: We retrospectively analysed the clinical and histopathological characteristics of widespread and segmental SSS collected from a dermatology department. We also compared histopathology between segmental SSS and morphea.
RESULTS: Thirty-one cases, including three widespread SSS and 28 segmental SSS, were collected. Skin lesions of widespread SSS generally showed skin sclerosis concentrating over the lumbar, buttocks, thighs, proximal part of limbs, and shoulders with specific abnormal gait and posture. Skin lesions of segmental SSS generally showed sclerotic plaques involving the thigh, lumbar area and buttocks, associated with hypertrichosis, hyperpigmentation and a cobblestone appearance. Segmental SSS did not typically cause joint limitation or serious physical discomfort. Histopathologically, SSS showed proliferation of fibroblasts and sclerosis of collagen in the dermis or subcutaneous tissue. Compared with morphea, SSS showed more prominent proliferation of fibroblasts and completely lacked lymphocyte infiltration.
CONCLUSIONS: Segmental SSS represents the major variant of SSS. Histopathologically, SSS shows proliferation of fibroblasts, sclerosis and an absence of inflammation.