Localizing apraxia in corticobasal syndrome: a morphometric MRI study.

Apraxia localization has relied on voxel-based, lesion-symptom mapping studies in left hemisphere stroke patients. Studies on the neural substrates of different manifestations of apraxia in neurodegenerative disorders are scarce. The primary aim of this study was to look into the neural substrates of different manifestations of apraxia in a cohort of corticobasal syndrome patients (CBS) by use of cortical thickness. Twenty-six CBS patients were included in this cross-sectional study. The Goldenberg apraxia test (GAT) was applied. 3D-T1-weighted images were analyzed via the automated recon-all Freesurfer version 6.0 pipeline. Vertex-based multivariate General Linear Model analysis was applied to correlate GAT scores with cortical thickness. Deficits in imitation of meaningless gestures correlated with bilateral superior parietal atrophy, extending to the angular and supramarginal gyri, particularly on the left. Finger imitation relied predominantly on superior parietal lobes, whereas the left angular and supramarginal gyri, in addition to superior parietal lobes, were critical for hand imitation. The widespread bilateral clusters of atrophy in CBS related to apraxia indicate different pathophysiological mechanisms mediating praxis in neurodegenerative disorders compared to vascular lesions, with implications both for our understanding of praxis and for the rehabilitation approaches of patients with apraxia.

Waist Circumference and Body Mass Index as Predictors of Disability Progression in Multiple Sclerosis: A Systematic Review and Meta-Analysis.

Background: While obesity has been shown to elevate the risk of developing multiple sclerosis (MS), there is a lack of strong evidence regarding its role in the disability progression and status of MS patients. Methods: This systematic review and meta-analysis aimed to provide comparative estimates of WC and BMI in patients with MS (PwMS) and to investigate potential associations between the waist circumference (WC) and body mass index (BMI) and demographic and specific MS characteristics. Adhering to PRISMA guidelines, a detailed search of the MEDLINE PubMed, Cochrane Library, and Scopus databases was conducted. Results: A total of 16 studies were included. The pooled mean WC and BMI among PwMS was estimated to be 87.27 cm (95%CI [84.07; 90.47]) and 25.73 (95%CI [25.15; 26.31]), respectively. Meta-regression models established a significant bidirectional relationship between WC and the Expanded Disability Scale (EDSS) (p < 0.001) but not between BMI and EDSS (p = 0.45). Sensitivity analyses showed no association between WC and age (p = 0.48) and a tendency between WC and disease duration (p = 0.08). Conclusions: Although WC measurements classify PwMS as normal weight, BMI measurements classify them as overweight. Therefore, WC should complement BMI evaluations in clinical practice. Additionally, our findings highlight the significant association between abdominal fat, as indicated by WC, and disease progression. Considering the heightened risk of cardiovascular comorbidity and mortality among PwMS, we recommend integrating both WC and BMI as standard anthropometric measurements in routine clinical examinations and targeted prevention strategies for PwMS.

A Novel and Simple Technique to Reduce the Rate of Pharyngocutaneous Fistula Formation Following a Total Laryngectomy - our Initial Results.

Objective: Pharyngocutaneous fistula (PCF) is a common and often devastating complication of total laryngectomy. Patients undergoing a total laryngectomy need enhancement of the neopharynx to reduce the risk of PCF formation. Our study aimed to evaluate the formation of a PCF following a total laryngectomy in patients that underwent a modified closure technique of their neopharynx. This technique included the recruitment of a flap of the muscular division of the pretracheal fascia that invests the strap muscles as a protective blanket. We called this surgical technique the 'curtain call'. Methods: We conducted a retrospective study for patients who underwent a total laryngectomy in our department between May 2022 and May 2023. Results: Twelve patients were identified. Our results demonstrated that the recruitment of this modified closure technique to cover the neopharynx resulted in a very low rate of postoperative PCF formation (8.3%). Conclusion: The 'curtain call' technique is an excellent method to support the neopharynx with extremely low rates of postoperative chronic dysphagia and with no evidence of impairing the development of esophageal speech. It could sometimes substitute much more time-consuming techniques like major pectoralis flap and supraclavicular flap. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-04343-7.

The Discovery of an S-shaped Kidney in a Patient With Prostate Cancer: A Rare Finding.

Crossed fused renal ectopia (CFRE) constitutes a rare congenital anomaly of the urinary tract, typically characterized by its predominantly asymptomatic nature and frequent incidental discovery. This case report delineates the clinical profile of a 56-year-old male admitted to our Prostate Cancer Outpatient Clinic due to elevated prostate-specific antigen (PSA) levels, ultimately leading to the diagnosis of prostate cancer. The patient was asymptomatic, with no family or surgical background. Notably, a fused ectopic kidney was incidentally identified during the staging process involving abdominal computed tomography (ACT) scanning. Remarkably, no additional abnormalities of the urinary tract or renal dysfunction manifested in this specific case. The significance of this report lies in the underscored emphasis on the importance of employing precise imaging techniques and tailored management strategies for patients harboring such anatomical variations.

Paraneoplastic Neurological Syndromes as Initial Presentation of Tumors: An Eight-Year Single-Center Experience.

BACKGROUND: Paraneoplastic Neurological Syndromes (PNS) comprise a diverse group of disorders propagated by immune-mediated effects of malignant tumors on neural tissue. METHODS: A single-center longitudinal study was performed including consecutive adult patients treated at a tertiary academic hospital between 2015 and 2023 and diagnosed with PNS. PNS were ascertained using the 2004 and the revised 2021 PNS-Care diagnostic criteria. RESULTS: Thirteen patients who fulfilled the 2004 definite PNS criteria were included. PNS comprise diverse neurological syndromes, with neuromuscular junction disorders (54%) and limbic encephalitis (31%) being predominant. PNS-related antibodies were detected in 85% of cases, including anti-AChR (n = 4), anti-P/Q-VGCC (n = 3), anti-Hu (n = 3), anti-Yo (n = 1), anti-Ma (n = 1), anti-titin (n = 1), anti-IgLON5 (n = 1), and anti-GAD65 (n = 1). Thymoma (31%), small-cell lung cancer (23%), and papillary thyroid carcinoma (18%) were the most frequent tumors. Imaging abnormalities were evident in 33% of cases. Early immunotherapy within 4-weeks from symptom onset was associated with favorable outcomes. At a mean follow-up of 2 ± 1 years, two patients with anti-Hu and anti-Yo antibodies died (18%). Four and three patients fulfilled the 2021 PNS-Care diagnostic criteria for definite and probable PNS, respectively. CONCLUSIONS: This study highlights the clinical heterogeneity of PNS, emphasizing the need for early suspicion and prompt treatment initiation for optimal outcomes.

Neuropsychological profiles comparison between Multiple Sclerosis patients and Multiple Sclerosis patients with overlapping features of Systemic Lupus Erythematosus.

AIM: Multiple sclerosis (MS) and Systemic Lupus Erythematosus (SLE) are autoimmune disorders that may lead to cognitive impairment. This study aimed to compare the neuropsychological profiles of patients with MS, and MS and coexisting SLE features. METHODS: We included a total of 90 participants, divided into 3 groups: 30 patients with clinically definite relapsing remitting MS, 30 with coexisting MS and incomplete SLE (overlap group) and 30 healthy controls (HC). All participants underwent neuropsychological assessment with the Montreal Cognitive Assessment (MoCA), Symbol Digit Modalities Test (SDMT), Paced Auditory Serial Addition Test (PASAT), and Selective Reminding Test (SRT). RESULTS: Both groups scored lower on the MoCA compared to the HC (p < .001). The overlap group showed the lowest performance on the SDMT and PASAT compared to the other two groups (p < .01), while the MS group scored similarly to the HC in the PASAT (p > .05). Regarding the learning rate and long-term recall, the overlap group had lower scores compared to both the MS and HC (p < .001), but it outperformed both groups in the retention efficacy score (p < .001). The MS group did not differ significantly from the HC in these memory domains (p > .05). CONCLUSION: The overlap group exhibited a broader range of impairments, including slower processing speed, decreased working memory, reduced learning rate, and long-term retrieval deficits. Their retention ability remained intact. The coexistence of MS with SLE pathology had additive impacts on cognitive function.

Enhancing Meniscal Repair: Investigating the Impact of an Exogenous Fibrin Clot.

This study conducted a comparative analysis of meniscal rupture repair, evaluating outcomes with and without the application of an exogenous fibrin clot to enhance meniscus repair. The research incorporated a relatively large sample size (24 patients) and employed a randomized control group with similar age characteristics and morphological types of meniscal ruptures as the study group. Notably, two postoperative follow-up times, at the third and 12th postoperative months, were utilized, distinguishing this study from related research. In the third postoperative month assessment, the fibrin clot technique demonstrated a significant advantage over simple stapling, as evidenced by markedly improved Tegner Lysholm Knee Scoring Scale (TLKSS) and Modified Cincinnati Rating System Questionnaire (MCRSQ) clinical assessment scores. Subsequent MRI scans at 12 months post-treatment revealed a high rate (91.67%) of complete healing in menisci treated with a fibrin clot, with only 4.17% exhibiting incomplete healing. This study expanded on previous research by including longitudinal ruptures and bucket-handle ruptures in addition to radial ruptures. The findings highlight a notable early improvement (third postoperative month) in the clinical assessment of longitudinal and bucket-handle ruptures treated with a fibrin clot during meniscus repair. This research contributes valuable insights into the efficacy of fibrin clots in enhancing meniscus repair, suggesting positive clinical and radiological outcomes, especially in the early stages postoperatively.

Clinical Behavior of the Mucoepidermoid Carcinoma of the Larynx-A Scoping Review.

OBJECTIVE: Mucoepidermoid carcinomas (MEC) of the larynx account for less than 1% of all laryngeal tumors. The unique features and clinical behavior of these rare entities remain unknown. To fill this knowledge gap, we performed a scoping review of every reported case of laryngeal MEC to study the clinical behavior and the treatment modalities in this rare entity. REVIEW METHODS: We followed the guidelines outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for scoping reviews (PRISMA-ScR) statement. DATA SOURCES: MedLine, Google Scholar, and Science Direct databases were searched to identify eligible studies. RESULTS: We concluded with 25 studies and overall 66 cases. Our study revealed that the average age of cases was 55.7 years, and the range was from 12 to 81 years, with younger onset in females. Supraglottic (60%) is the most common subsite of laryngeal MEC s followed by glottis (27.3%). Supraglottic carcinomas frequently present with metastatic neck disease at the time of the initial diagnosis and require more extensive surgical approaches. Surgery with negative surgical margins seems to be the cornerstone in the treatment of MEC. Radiation therapy has not been tested widely as monotherapy but is considered a useful adjuvant modality. CONCLUSION: This study suggests that negative surgical margins seem to be associated to higher disease control rate and that high-grade supraglottic cases likely benefit from addressing neck disease simultaneously. LEVEL OF EVIDENCE: NA Laryngoscope, 2023.

Alzheimer's Disease CSF Biomarkers as Possible Indicators of Tap-Test Response in Idiopathic Normal Pressure Hydrocephalus.

The aim of the present study is the evaluation of established Alzheimer's disease (AD) cerebrospinal fluid (CSF) biomarkers in patients with idiopathic normal-pressure hydrocephalus (iNPH), both individually and as a total profile, and the investigation of their use as potential predictors of Tap-test responsiveness. Fifty-three patients with iNPH participated in the study. Aß42, Aß40, total Tau and phospho-Tau proteins were measured in duplicate with double-sandwich ELISA assays. Clinical evaluation involved a 10 m timed walk test before an evacuative lumbar puncture (LP) and every 24 h for three consecutive days afterwards. Neuropsychological assessment involved a mini-mental state examination, frontal assessment battery, 5-word test and CLOX drawing test 1 and 2, which were also performed before and 48 h after LP. Response in the Tap-test was defined as a 20% improvement in gait and/or a 10% improvement in neuropsychological tests. The Aß42/Aß40 ratio was found to be significantly higher in Tap-test responders than non-responders. Total Tau and phospho-Tau CSF levels also differed significantly between these two groups, with Tap-test responders presenting with lower levels compared to non-responders. Regarding the AD CSF biomarker profile (decreased amyloid and increased Tau proteins levels), patients with a non-AD profile were more likely to have a positive response in the Tap-test than patients with an AD profile.

The Dynamic Relationship between the Glymphatic System, Aging, Memory, and Sleep.

The process of memory entails the activation of numerous neural networks and biochemical pathways throughout the brain. The phenomenon of memory decline in relation to aging has been the subject of extensive research for several decades. The correlation between the process of aging and memory is intricate and has various aspects to consider. Throughout the aging process, there are various alterations that take place within the brain and, as expected, affect other functions that have already been linked to memory and its function such as involving microcirculation and sleep. Recent studies provide an understanding of how these mechanisms may be interconnected through the relatively new concept of the glymphatic system. The glymphatic system is strongly correlated to sleep processes. Sleep helps the glymphatic system remove brain waste solutes. Astrocytes expand and contract to form channels for cerebrospinal fluid (CSF) to wash through the brain and eliminate waste. However, the details have not been totally elusive, but the discovery of what we call the glymphatic system enables us to connect many pieces of physiology to understand how such factors are interconnected and the interplay between them. Thus, the purpose of this review is to discuss how the glymphatic system, sleep, memory, and aging are interconnected through a network of complex mechanisms and dynamic interactions.

The Presence of a Zygomatic Nerve Plexus in a Cadaveric Specimen Without a Zygomatic Branch of the Facial Nerve.

The extratemporal course of the facial nerve distributes in facial mimic muscles in a complex pattern. The traditional depictions of five main branches without anastomoses are not common. Davis classification remains the gold standard in the classification of facial nerve branching patterns. During a routine dissection of an 74-year-old male cadaveric specimen, we detected a very rare anatomical variation. The zygomatic branch of the facial nerve was totally absent. The temporofacial division of the main trunk was bifurcated to a temporal and a buccal branch. The anterior temporal and posterior buccal branches formed a plexus to supply the orbicularis oculi muscle. This unique variability highlights the complexity of the extratemporal facial nerve course. Retrograde facial nerve dissection requires deep knowledge of every anatomical variation of the facial nerve course to avoid an iatrogenic injury. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03601-y.

Rehabilitation on cerebellar ataxic patients with multiple sclerosis: A systematic review.

Multiple Sclerosis (MS) is a chronic inflammatory, autoimmune disease of the Central Nervous System with a vast spectrum of clinical phenotypes. A major aspect of its clinical presentation is cerebellar ataxia where physiotherapy and treatment modalities play a significant role on its management. This systematic review aims to investigate the physiotherapeutic rehabilitation techniques regarding the management of cerebellar ataxia due to MS and secondary to stratify each protocol as part of a multi structural personalized rehabilitation approach based on the gravity of the symptoms. A Pubmed Medline, Scopus and Web of Science research was performed using the corresponding databases. The results were screened by the authors in pairs. In our study, six (6) non-pharmacological interventional protocols, 3 Randomized Controlled Trials and 3 pilot studies, were included with a total of 145 MS patients. Physiotherapeutic techniques, such as NDT-Bobath, robotic and visual biofeedback re-education protocols and functional rehabilitation techniques were included. In most cases cerebellar ataxic symptoms were decreased post-treatment. The overall quality of the studies included was of moderate level (level B). Rehabilitation in cerebellar ataxia due to MS should be based on multicentric studies with the scope of adjusting different types of treatments and physiotherapeutic techniques based on the severity of the symptom.

The Prevalence of Diabetes Mellitus Type II (DMII) in the Multiple Sclerosis Population: A Systematic Review and Meta-Analysis.

Introduction: The interactions between Diabetes Mellitus type II (DMII) and Multiple Sclerosis (MS) lead to higher levels of fatigue, higher risk of physical disability, faster cognitive decline, and in general a lower quality of life and a higher frequency of depression compared to the general population. All of the above accelerate the disability progression of patients with MS, reduce the patients' functional capacity, and further increase their psychological and economic burden. Methods: This systematic review and meta-analysis aims to calculate the prevalence of DMII in the MS population. Following PRISMA guidelines, a thorough search of the Medline Pubmed, Cochrane Library, and Scopus databases was performed, focusing on the frequency of DMII in the MS population. Results: A total of 19 studies were included in the synthesis. The results of the main meta-analysis of random effects using R studio 3.3.0 for Windows and the Meta r package showed that the prevalence of DMII in the MS population is 5% (95% CI [0.03, 0.07], 19 studies, I2 = 95%, pQ < 0.001). Additional subgroup analysis based on region showed a difference of 4.4% (I2 = 95.2%, pQ < 0.001), psubgroupdifference = 0.003) between European and non-European participants, while demographic- and MS-specific characteristic (EDSS, Disease Duration) did not seem to affect the prevalence of DMII in the MS population (p = 0.30, p = 0.539, p = 0.19, p = 0.838). No publication bias was discovered (Egger's p test value: 0.896). Conclusions: Even though the prevalence of DMII in the MS population is lower than 10% (the reported prevalence of DMII in the general population) the interactions between the two conditions create significant challenges for MS patients, their caregivers, and physicians. DΜΙΙ should be systematically recorded in the case of MS patients to clearly delineate any potential relationship between the two conditions. Additionally, more structured studies investigating the interactions of MS and DMΙΙ as well as the direction of the causation between those two conditions are necessary in order to gain a deeper insight into the nature of the interaction between MS and DMII.

Assessment of Aggregated and Exosome-Associated α-Synuclein in Brain Tissue and Cerebrospinal Fluid Using Specific Immunoassays.

Even though it is currently well-established that α-synuclein aggregation is closely associated with the pathological events in Parkinson's disease (PD) and several other neurodegenerative disorders, collectively called synucleinopathies, the mechanistic link between α-synuclein aggregates and the onset and progression of neurodegeneration in these diseases remain unclear. The process of aggregation initiates from a structurally distorted monomer that gradually oligomerizes to generate a repertoire of fibrillar and oligomeric multimers that deposit within diseased cells in the brain. Total α-synuclein has been proposed as a potential biomarker in PD, but most of the studies do not discriminate between distinct α-synuclein conformers. To correlate protein measurements to disease pathology, we have developed a conformation-specific ELISA method that selectively detects fibrillar and oligomeric forms of α-synuclein without cross-reacting with monomers. We have used this assay to determine the levels of aggregated α-synuclein in human and mouse brain tissue as well as in CSF and CSF-derived exosomes from patients with synucleinopathy and control subjects. Our results verify the ability of the new assay to detect aggregated α-synuclein in complex matrices and support the idea that the levels of these conformers are related to the age of onset in PD patients, while CSF analysis showed that these species exist in low abundance in CSF and CSF-derived exosomes. Future studies will be required to fully assess the diagnostic usefulness of this ELISA in synucleinopathies.

A Cadaveric Case of an Accessory Cleido-Occipital Muscle Over the Common Trunk of the Supraclavicular Nerves.

The current study describes a case of an aberrant cleido-occipital muscle. In particular, this muscle was arising from the middle part of the clavicle, inserted into the medial part of the upper trapezius muscle, and crossed over the supraclavicular nerves with possible compression of them, especially during shoulder abduction. Knowledge of the muscular variability of the posterior cervical triangle is crucial for supraclavicular nerve entrapment syndrome diagnosis and treatment. The appearance of aberrant muscular fascicles may lead to misinterpretation of neck imaging, as well as difficulties during surgical procedures undertaken in the region.

External Jugular Vein Perforation by the Transverse Cutaneous Nerve of The Neck: A Case Report and Review of The Literature.

A very rare anatomical variation of external jugular vein's penetration by the transverse cutaneous nerve of the neck is displayed in the current study. The phenomenon of veins' fenestration or penetration by other structures, such as nerves along with its likely embryologic development are discussed. Moreover, the potential clinical significance of that variation's awareness on behalf the physician is discussed in detail.

Early Trifurcation of the Mandibular Branch of the Facial Nerve in a Cadaveric Specimen.

Rare facial nerve branching patterns, pose dangers due to their unexpected course. Cases with multiple branches may reduce the intraoperative risk, due to the compensation of adjacent branches. We present a case of a cadaveric specimen where an early trifurcation of the mandibular branch of the facial nerve was noted. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-022-03352-2.

Is the origin of the superior thyroid artery consistent? A systematic review of 5488 specimens.

PURPOSE: The superior thyroid artery (STA) point of origin is strongly debated with controversial results among studies. External carotid artery (ECA), carotid bulb, and common carotid artery (CCA) have been presented as points of origin with variable percentages among authors. We conducted a systematic review of all existing studies that included cadaveric, surgical, and angiographic specimens and recorded the origin of STA according to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) statement. MATERIALS AND METHODS: Fifty-two studies, with an overall of 5488 specimens were included. RESULTS: Our results indicated ECA as the most common site of origin (55.0 %) followed by carotid bifurcation at 27.5 % and CCA at 15.0 %. Absent STA or branching from the internal carotid artery (ICA) was an extremely rare finding. We proposed a new simple classification system based on our results. CONCLUSIONS: The huge variability in the branching pattern of STA makes head and neck surgery and radiographic interventions challenging and poses the integrity of STA at risk. Therefore, we strongly recommend preoperative angiographic studies for STA identification to prevent an intraoperative iatrogenic injury.

Bilateral Thyroid Foramina in a Completely Ossified Laryngeal Framework: A Case Report.

The presence of a thyroid foramen in the thyroid cartilage of the larynx is not uncommon. It may be occluded by a fibrous layer, or it may be an abnormal path for the neurovascular bundle of the larynx. The superior laryngeal nerve and the superior laryngeal vessels are the most common contents of the thyroid foramen. During the observation of the skeleton of a 32-year-old female, we found a completely ossified laryngeal framework with bilateral double thyroid foramina. Three of the foramina were circular, and one was oval in shape. This is a very rare anatomical variation. Deep knowledge of the thyroid cartilage anatomy is mandatory during laryngeal and thyroid surgery. The meticulous dissection of laryngeal vessels and nerves is of paramount importance to control bleeding and avoid postoperative neurological sequelae due to nerve injury. The surgeon should be aware that in the whole length of the oblique line of the thyroid cartilage, a thyroid foramen may be detected.

Safety of COVID-19 vaccines in multiple sclerosis: A systematic review and meta-analysis.

BACKGROUND: Data are sparse regarding the safety of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines in patients with multiple sclerosis (MS). OBJECTIVE: To estimate (1) the pooled proportion of MS patients experiencing relapse among vaccine recipients; (2) the rate of transient neurological worsening, adverse events, and serious adverse events; (3) the previous outcomes of interest for different SARS-CoV-2 vaccine types. METHODS: Systematic review and meta-analysis of pharmacovigilance registries and observational studies. RESULTS: Nineteen observational studies comprising 14,755 MS patients who received 23,088 doses of COVID-19 vaccines were included. Mean age was 43.3 years (95% confidence interval (CI): 40-46.6); relapsing-remitting, secondary-progressive, primary-progressive MS and clinically isolated syndrome were diagnosed in 82.6% (95% CI: 73.9-89.8), 12.6% (95% CI: 6.3-20.8), 6.7% (95% CI: 4.2-9.9), and 2.9% (95% CI: 1-5.9) of cases, respectively. The pooled proportion of MS patients experiencing relapse at a mean time interval of 20 days (95% CI: 12-28.2) from vaccination was 1.9% (95% CI: 1.3%-2.6%; I2 = 78%), with the relapse risk being independent of the type of administered SARS-CoV-2-vaccine (p for subgroup differences = 0.7 for messenger RNA (mRNA), inactivated virus, and adenovector-based vaccines). After vaccination, transient neurological worsening was observed in 4.8% (95% CI: 2.3%-8.1%) of patients. Adverse events and serious adverse events were reported in 52.8% (95% CI: 46.7%-58.8%) and 0.1% (95% CI: 0%-0.2%) of vaccinations, respectively. CONCLUSION: COVID-19 vaccination does not appear to increase the risk of relapse and serious adverse events in MS. Weighted against the risks of SARS-CoV-2-related complications and MS exacerbations, these safety data provide compelling pro-vaccination arguments for MS patients.