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Table S1: Pathomechanics of Joubert Syndrome Table S2: Pathomechanics of Bardet Biedl Syndrome Table S3: Pathomechanics of Neuromyelitis Optica Spectrum Disorder, Genome-wide association study; Phenome-wide association study; Genetic diagnosis; Molecular mechanisms; Cerebral visual impairment; Multiple sclerosis; Joubert syndrome; Mowat Wilson disease; Zellweger spectrum disorder; Neuromyelitis optica spectrum disorder

OBJECTIVE The relationship between autoimmune rheumatic diseases, inflammatory bowel diseases, and carpal tunnel syndrome is unclear. We aimed to survey the occurrence and characteristics of carpal tunnel syndrome in autoimmune rheumatic diseases and inflammatory bowel diseases, compared with the general population. METHODS We used the Longitudinal Health Insurance Database 2015 from Taiwan's National Health Insurance Research Database. Patients diagnosed with autoimmune rheumatic diseases/inflammatory bowel diseases were identified. The incidence rates and surgical rates of carpal tunnel syndrome among individual diseases were calculated. The hazard ratios when compared with age and sex matched, and 1:1 ratio control groups were surveyed. RESULTS A total of 2591 women and 701 men were identified. The incidence rate of carpal tunnel syndrome was highest in Crohn disease (1001 per 100,000 person-years, 95% confidence interval = 0-2747), followed by scleroderma and Sjogren syndrome. The incidence rate in the control group was 571 per 100,000 person-years (95% confidence interval = 314-829). Significantly increased adjusted hazard ratios were seen in Sjogren syndrome (1.44, 95% confidence interval = 1.09-1.90) and rheumatic arthritis (1.33, 95% confidence interval = 1.05-1.70). The overall surgical rate was 0.2% in patients with autoimmune rheumatic diseases/inflammatory bowel diseases and 0.3% in the control group, without a significant difference (P = 0.85). CONCLUSIONS Patients with Sjogren syndrome and rheumatic arthritis are susceptible to carpal tunnel syndrome. Patients with autoimmune rheumatic diseases/inflammatory bowel diseases have similar surgical rates as general population.

The pandemic of severe acute respiratory syndrome coronavirus 2 has spread across the world, causing causalities and inflicting chronic complications in those who survive the infection. Disruptions in the immune system and lowered physical levels caused by quarantine protocols are the major causes of chronic dysfunction and impaired life independency, especially in elderly patients. Multiple exercise suggestions and novel program delivery methods, including telerehabilitation/tele-exercise programs, home-gym strategies, and exergames, have emerged. Patients with underlying obesity, diabetes mellitus, malnutrition, or binge-eating problems are at a high risk of serious infection and sequela. Adequate education and coping strategies can lessen the potential infection complications and improve the quality of life. Acknowledging the possible benefits and risks of nonsteroidal anti-inflammatory drug usage in chronic pain patients, and the supplementation of vitamin D may also aid in treating post-infected patients.

A 56-year-old surgeon was seen for numbness in his left hand lasting over one year. This numbness had first developed over the hypothenar area and then progressed to the whole palm. He stated that the discomfort was worse as he raised his left shoulder, causing difficulty during surgery. His medical history was otherwise noncontributory.

Patients with bilateral vestibular hypofunction (BVH) often suffer from imbalance, gait problems, and oscillopsia. Noisy galvanic vestibular stimulation (GVS), a technique that non-invasively stimulates the vestibular afferents, has been shown to enhance postural and walking stability. However, no study has investigated how it affects stability and neural activities while standing and walking with a 2 Hz head yaw turning. Herein, we investigated this issue by comparing differences in neural activities during standing and walking with a 2 Hz head turning, before and after noisy GVS. We applied zero-mean gaussian white noise signal stimulations in the mastoid processes of 10 healthy individuals and seven patients with BVH, and simultaneously recorded electroencephalography (EEG) signals with 32 channels. We analyzed the root mean square (RMS) of the center of pressure (COP) sway during 30 s of standing, utilizing AMTI force plates (Advanced Mechanical Technology Inc., Watertown, MA, USA). Head rotation quality when walking with a 2 Hz head yaw, with and without GVS, was analyzed using a VICON system (Vicon Motion Systems Ltd., Oxford, UK) to evaluate GVS effects on static and dynamic postural control. The RMS of COP sway was significantly reduced during GVS while standing, for both patients and healthy subjects. During walking, 2 Hz head yaw movements was significantly improved by noisy GVS in both groups. Accordingly, the EEG power of theta, alpha, beta, and gamma bands significantly increased in the left parietal lobe after noisy GVS during walking and standing in both groups. GVS post-stimulation effect changed EEG activities in the left and right precentral gyrus, and the right parietal lobe. After stimulation, EEG activity changes were greater in healthy subjects than in patients. Our findings reveal noisy GVS as a non-invasive therapeutic alternative to improve postural stability in patients with BVH. This novel approach provides insight to clinicians and researchers on brain activities during noisy GVS in standing and walking conditions in both healthy and BVH patients.

Infection with the novel severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causes the development of the novel 2019 coronavirus disease (COVID-19) and associated clinical symptoms, which typically presents as an upper respiratory syndrome such as pneumonia. Growing evidence indicates an increased prevalence of neurological involvement (e.g., in the form of stroke) during virus infection. COVID-19 has been suggested to be more than a lung infection because it affects the vasculature of the lungs and other organs and increases the risk of thrombosis. Patients with stroke are vulnerable to secondary events as a result not only of their poor vascular condition but also of their lack of access to rehabilitation resources. Herein, we review current knowledge regarding the pathophysiology of COVID-19, its possible association with neurological involvement, and current drug therapies. Suggestions are also offered regarding the potential for current neurorehabilitation therapies to be taught and practiced at home.

Sciatica is a common musculoskeletal complaint, but it is rarely attributed to peripheral nerve tumors. Until now, there is little literature reporting sciatica caused by a sciatic schwannoma at the proximal thigh. A 27-year-old male had left posterior proximal thigh pain for more than two years. Compression of the tender point caused numbness radiating to his low back, buttock and leg regions. Due to poor response to conservative treatments, he was referred for an ultrasound examination, which revealed a solid mass on the track of the sciatic nerve. The subsequent magnetic resonance imaging showed a well-defined tumor sized 2.3 × 1.8 × 2.3 cm beside the sciatic nerve, and a schwannoma was confirmed by postsurgical pathology. In conclusion, ultrasound is helpful in differentiating between the various causes of posterior thigh pain, which, in this case, facilitated detection of a sciatic nerve schwannoma and subsequent surgical removal.

The brachial plexus (BP) is a complicated neural network, which may be affected by trauma, irradiation, neoplasm, infection, and autoimmune inflammatory diseases. Magnetic Resonance Imaging is the preferred diagnostic modality; however, it has the limitations of high cost and lack of portability. High-resolution ultrasound has recently emerged as an unparalleled diagnostic tool for diagnosing postganglionic lesions of the BP. Existing literature describes the technical skills needed for prompt ultrasound imaging and guided injections for the BP. However, it remains particularly challenging for beginners to navigate easily while scanning its different parts. To address this, we share several “clinical pearls” for the sonographic examination of the BP as well as its common pathologies.

Background Severe inflammation plays a vital role in the pathogenesis of meconium aspiration syndrome (MAS). Intratracheal (IT) instillation of corticosteroids may be beneficial for MAS in optimizing local effect and reducing systemic adverse effects, but the optimum dosing course remains open to question. Methods Thirty meconium-injured newborn piglets were enrolled into six study groups. The first four groups consisted of the IT instillation of 0.25/0.5 mg/kg using either one (IT-B251/IT-B501) or two (IT-B252/IT-B502) doses of budesonide, while the other two groups were the intravenous (IV) dexamethasone (0.5 mg/kg) (IV-Dex) group and the control group (Ctrl). Vital signs and cardiopulmonary functions were monitored throughout the experiments. Pulmonary histology was examined after completing the experiments. Results Both the IV-Dex and IT-B501 groups got significant improvement in oxygenation (P

Background Venomous snakebites are common during hot seasons in Taiwan. However, rarely is venom spat directly into the subject's eyes, causing eye injury. Despite being uncommon, analytical data regarding venom-spit ophthalmia in Taiwan have been lacking. This study thus aimed to conduct an epidemiological survey on Naja atra venom-spit ophthalmia in Taiwan during the past decades to improve future care of such patients. Methods Registered records of cases with snake venom injuries at the Taiwan National Poison Control Center from 1990 to 2016 were retrospectively reviewed, enrolling those with records of cobra venom-spit eye injuries and excluding exotic species. Demographic data, clinical symptoms, snake species, ocular conditions, management, and outcomes were recorded and analyzed. Results A total of 39 cases suffering from Naja atra venom-spit ophthalmia were enrolled. The overall incidence rate was 1.6 cases per million people from 1990 to 2016. Among the included cases, most were unilaterally injured (82%), male (95%), aged 18 to 59 years (90%), injured during catching (51%), and injured in spring and summer (92%). Ocular symptoms occurred in 90% of the cases, majority of which included ocular pain (90%) and redness (85%). Conjunctivitis (67%) and corneal injury (59%) of involved eyes were common. Immediate water irrigations were done in all cases, most of whom received further topical eye drops, including antibiotics, corticosteroids, and vasoconstrictors. Although topical or intravenous antivenoms were administered in 11 cases, no obviously superior outcome was observed. Most cases (77%) were symptom free after the acute stage. Conclusion Although Naja atra venom-spit ophthalmia in Taiwan is uncommon, the risk for transient ocular symptoms and corneal/conjunctival injury does exists. Prompt ocular irrigation and professional ophthalmic care after envenomation help prevent serious ocular sequelae. Moreover, superior outcomes were not achieved with the use of antivenom. Nonetheless, further studies are required to clarify the role antivenoms play in venom-spit ophthalmia.

The triangular fibrocartilage complex (TFCC) serves as the major stabilizer of the wrist. Its injuries can result from trauma or degeneration, both of which are strongly correlated with the loading stress on the ulnar shaft and carpal joints. The TFCC is made of the articular disc, meniscus homologue, ulnocarpal ligament, radioulnar ligament, ulnotriquetral ligament, ulnolunate ligament, and subsheath of the extensor carpi ulnaris tendon. Because of its complexity, it is challenging to confirm the exact component affected in TFCC injuries. The Palmer classification is widely used for investigation of TFCC lesions using magnetic resonance imaging. Recently, high-resolution ultrasound (US) has become more popular in diagnosing musculoskeletal disorders. However, the utility of US imaging in TFCC lesions is less common because its anatomy under US imaging is not described in the current literature. Accordingly, in this review, we aimed to propose a standard US scanning protocol for the TFCC, present relevant images for its pathologies, and illustrate appropriate US-guided injection techniques for their management.

Severe respiratory distress syndrome (RDS) is still a major cause of mortality and morbidity in premature infants. The combined use of intratracheal corticosteroid and surfactant in severe RDS, which bypasses the systemic circulation, may not only help recruit the lungs but also alleviates pulmonary inflammation without an increase in systemic adverse effects. Twelve newborn piglets received repeated pulmonary saline lavage to create surfactant-depleted lungs that mimic neonatal RDS, and then were randomly grouped into a control group (standard intratracheal instillation of surfactant-Survanta 100 mg/kg); and a budesonide (Bude) group (intratracheal instillation with the mixed suspension of Budesonide 0.25 mg/kg and Survanta 100 mg/kg). Blood samples were examined, and the observation period was 24 hr. The results showed that oxygenation was significantly better in Bude group compared to the control group over time (P = 0.016). The proinflammatory cytokines tumor necrosis factor-α and interleukin-1 β showed a reduced trend in the Bude group, but was not significantly different from the control group (P > 0.05). Comparing the histological lung injury scores, the Bude group had a significantly lower score than the control group at both dependent and non-dependent sites (P

Background: Periventricular leukomalacia (PVL) is serious ischemic brain damage that occurs in extreme preterm infants. It is traditionally diagnosed by cranial echography. The purpose of this study was to investigate the relationship between serum S100 calcium-binding protein B (S100B) concentrations and ischemic brain damage, and to find the cutoff value for the early identification of ischemic brain damage in high-risk preterm infants. Methods: At the age of 3 days, 7 days, 14 days, and 21 days, and before discharge, 22 extremely premature infants (i.e., gestational age 1.0 μg/L.

Severe respiratory distress syndrome (RDS) is still a major cause of mortality and morbidity in premature infants. The combined use of intratracheal corticosteroid and surfactant in severe RDS, which bypasses the systemic circulation, may not only help recruit the lungs but also alleviates pulmonary inflammation without an increase in systemic adverse effects. Twelve newborn piglets received repeated pulmonary saline lavage to create surfactant-depleted lungs that mimic neonatal RDS, and then were randomly grouped into a control group (standard intratracheal instillation of surfactant-Survanta 100 mg/kg); and a budesonide (Bude) group (intratracheal instillation with the mixed suspension of Budesonide 0.25 mg/kg and Survanta 100 mg/kg). Blood samples were examined, and the observation period was 24 hr. The results showed that oxygenation was significantly better in Bude group compared to the control group over time (P = 0.016). The proinflammatory cytokines tumor necrosis factor-α and interleukin-1 β showed a reduced trend in the Bude group, but was not significantly different from the control group (P 0.05). Comparing the histological lung injury scores, the Bude group had a significantly lower score than the control group at both dependent and non-dependent sites (P 0.05). In conclusion, in piglets with severe RDS, intratracheal instillation of budesonide in addition to surfactant seems to results in a sustained improvement in pulmonary outcome over 24 hr.