Traumatic atlantoaxial anteroinferior subluxation with dens and Hangman fractures: A case report

Rationale:Traumatic atlantoaxial anteroinferior subluxation associated with a dens fracture and a Hangman fracture is a very rare and complex injury. Therefore, appropriate surgical strategy is not established.Patient concerns:An 85-year-old female presented with posterior neck pain and atypical neck position caused after rolling down a hill. Although neurological examinations for motor, sensory, gait, and reflex tests were normal, the patient complained of an abnormal neck posture.Diagnoses:Radiological examinations revealed an atlantoaxial anteroinferior subluxation with kyphosis, a type IIA dens fracture (Anderson and D’Alonzo classification) with an anterolateral rotatory angulation of type IIA dens fracture fragment, and a type I Hangman fracture (Levine and Edwards classification). Nevertheless, the transverse atlantal ligament was intact.Interventions:We considered that the intact transverse atlantal ligament and kinking of the type IIA dens fracture fragment into the left lateral mass of C1 prevented a spinal cord injury by blocking a further displacement of C1 to C2. Due to the patient''s osteoporosis and the anterolateral rotatory angulated type IIA dens fracture fragment, a forceful reduction of the atlantoaxial anteroinferior subluxation with kyphosis could pose a high risk of fixation failure and spinal cord injury. Therefore, we performed in-situ posterior C1-2 fusion using a C1 lateral mass screw and C2 lamina screw fixations.Outcomes:At 1 year after surgery, the bone union of all fractures was achieved in the kyphosis state. Furthermore, the patient''s clinical symptoms were improved with no neurological deficit.Lessons:A thorough radiological examination and appropriate surgical strategy are important for successful diagnosis and treatment of a complex C1-2 injury.     

The relationship between radiologic parameters and transverse atlantal ligament injury obtained from MRI scans in patients with an isolated atlas burst fracture: A retrospective observational study

The treatment of an atlas burst fracture depends on whether transverse atlantal ligament (TAL) injury is present. We compared the radiologic parameters associated with the presence of a TAL injury as detected using magnetic resonance imaging (MRI), and verified whether the lateral mass displacement (LMD) criteria currently used to diagnose TAL injuries in atlas burst fractures are reliable or need revision.Thirty patients who presented with isolated atlas burst fractures were included in this retrospective observational study. We measured radiologic parameters, including LMD, atlanto-dental interval, basion-dens interval, internal lateral mass displacement, and external lateral mass displacement, in each patient at the time of initial presentation. The presence of TAL injury was evaluated using MRI. We compared the radiologic parameters and characteristics of patients who presented with TAL injury. We also determined the sensitivity and specificity of an LMD test to accurately diagnose TAL injury based on MRI. Finally, we compared the radiologic parameters according to the presence of surgical treatment and patient union status.Twenty patients presented with an intact TAL, while 10 patients had a TAL injury on MRI. LMD was significantly higher in patients with TAL injury (9.61 vs 3.73 mm, P < .001). In multivariable logistic regression analysis, LMD was also significantly higher in patients with TAL injury. The sensitivity and specificity of LMD for diagnosing TAL injury based on MRI in patients with isolated C1 fractures were 90% and 100%, respectively. The incidence of an LMD greater than 8.1 mm was statistically higher in patients than in those without TAL injury (90% vs 0%, P < .001).Nine patients underwent surgery for isolated atlas burst fractures, and 21 did not. LMD (9.56 vs 4.03 mm, P < .001) and fracture gap (7.96 vs 4.01 mm, P < .001) were significantly higher in patients who underwent surgery.Among the various radiologic parameters, LMD closely correlated with the presence of TAL injury, as patients with an LMD greater than 8.1 mm were more likely to have a TAL injury in the case of atlas burst fractures. LMD is a good method for predicting the presence of TAL injury if MRI is not available.     

Acute autoimmune transverse myelitis following COVID-19 vaccination: A case report

Rationale:Transverse myelitis is an infectious or noninfectious inflammatory spinal cord syndrome. We report a rare case of transverse myelitis following vaccination against COVID-19.Patient concerns:A 70-year-old male presented with progressive sensorimotor dysfunction of the bilateral lower limbs 7 days after receiving the mRNA-1273 vaccine against COVID-19. Spinal magnetic resonance imaging revealed intramedullary lesions with gadolinium enhancement on the Th1/2 and Th5/6 vertebral levels. Cerebrospinal fluid (CSF) testing showed a mildly increased level of total protein and positive oligoclonal bands (OCB).Diagnosis:The patient was diagnosed with acute transverse myelitis.Intervention:The patient received 5 days of intravenous methylprednisolone pulse (1000 mg/day) followed by oral prednisolone (30 mg/day with gradual tapering).Outcomes:The patient fully recovered from muscle weakness of the lower limbs. He was discharged from our hospital and able to independently walk without unsteadiness.Lesson:This is a rare case of transverse myelitis following COVID-19 vaccination. Positive OCB in CSF in the present case highlights the possibility of autoimmune processes, including polyclonal activation of B lymphocytes, following vaccination.     

Refractory hyponatremia in neuromyelitis optica in a pediatric patient: A case report

Rationale:Neuromyelitis optica spectrum disorders (NMOSD) is a rare autoimmune disease predominantly involving optic nerves and spinal cord, and possible comorbidities including syndrome of inappropriate antidiuretic hormone secretion or urinary complication. We reported a young girl diagnosed with NMOSD presented with refractory hyponatremia, acute urine retention, and general weakness. Clinical symptoms improved gradually after receiving intravenous immunoglobulin, high-dose methylprednisolone, and plasmapheresis. NMOSD should be kept in mind in adolescence with acute urine retention, intermittent fever, and hyponatremia.Patient concerns:A 15-year-old girl admitted to our hospital due to no urination for 2 days.Diagnosis:Aquaporin-4 antibodies were detected showing positive both in serum and cerebrospinal fluid. Long transverse myelitis in cervical and thoracic spinal cord and optic neuritis was revealed in magnetic resonance imaging.Interventions:Intravenous immunoglobulin 2 g/kg was infused totally in 4 days, and methylprednisolone pulse therapy was subsequently followed in 5 days; followed by 5 courses of plasmapheresis a week later.Outcomes:Her muscle power, syndrome of inappropriate antidiuretic hormone secretion condition, and urinary function were all improved after immune-modulated treatment course; NMOSD relapsed twice within the first year after diagnosis, however no relapse of NMOSD in the subsequent 1 year.Lessons:To the best of our knowledge, this was the first childhood case of NMO accompanied by refractory hyponatremia in the reported literature. In childhood cases presenting with refractory hyponatremia and limb weakness, NMO or NMOSD should be considered possible diagnoses despite their rarity in pediatric cases.     

Impact of 2 different posterior screw fixation techniques on primary stability in a cervical translational injury model: A biomechanical evaluation

Background:In case of injuries to the subaxial cervical spine, especially in osteoporotic bone, the question of the most stable operative technique arises. There are several techniques of screw fixation available regarding dorsal stabilization. This study investigates 2 techniques (lateral mass screws (LMS) vs cervical pedicle screws (CPS)) in the subaxial cervical spine regarding primary stability in a biomechanical testing using a translational injury model.Methods:A total of 10 human formalin fixed and 10 human fresh-frozen specimens (C 4 - T 1) were investigated. Specimens were randomized in 2 groups. Fracture generation of a luxation injury between C 5 and C 6 was created by a transection of all ligamentous structures as well as the intervertebral disc and a resection of the facet joints.Dorsal stabilization of C 4/C 5 to C 6/C 7 was performed in group A by lateral mass screws, in group B by pedicle screws. In the biomechanical testing, the specimens were loaded at 2 N/s in translation direction until implant failure.Results:Formalin fixed specimen: Mean load failure was 513.8 (±86.74) Newton (N) for group A (LMS) and 570.4 (±156.5) N for group B (CPS). There was no significant difference (P = .6905).Fresh frozen specimen: Mean load failure was 402.3 (±96.4) N for group A (LMS) and 500.7 (±190.3) N for group B (CPS). There was no significant difference (P = .4206).Conclusion:In our loading model respecting the translational injury pattern and a flexion movement we could not verify statistically significant differences between lateral mass screws and cervical pedicle screws. Mean loading failure was slightly higher in the CPS group though.     

A pediatric case report and literature review of facioscapulohumeral muscular dystrophy type1

Rationale:Early-onset facioscapulohumeral muscular dystrophy (FSHD) is defined as facial weakness before the age of 5 and shoulder weakness before the age of 10. Early-onset facioscapulohumeral muscular dystrophy is relatively rare in the clinic. This onset is relatively early, the symptoms are serious, and it is likely to be accompanied by retinal vascular disease, sensorineural deafness, epilepsy and other extramuscular multisystem diseases. We report the clinical characteristics of 2 patients with early-onset facial and shoulder brachial muscular dystrophy to improve clinicians’ understanding of this particular condition.Patient concerns:We report 2 pediatric patients with FSHD type 1. Patient 1 is an 11-year-old boy with reduced facial expression for 9 years and proximal muscle weakness for 6 years. Patient 2 is a 4-year and 6-month-old girl with developmental delay for 3 years and facial weakness for 1 year.Diagnosis:According to the clinical manifestations and molecular genetic testing (such as Southern blot analysis), the patients were diagnosed with early-onset FSHD1.Interventions:The patients received cocktail therapy (vitamin B1 tablets, vitamin B2 tablets, vitamin B6 tablets, vitamin C tablets, vitamin E tablets, idebenone tablets, etc.) to improve their muscle metabolism.Outcomes:Both patients’ condition did not improve after being given cocktail treatment. According to a recent follow-up, the symptoms of facial weakness and proximal muscle weakness were aggravated.Lessons:Early-onset FSHD presents early and has frequent systemic features, and it is a severe subtype of FSHD. Early identification and genetic diagnosis should be performed to improve patient prognosis.     

Beneficial effects of preoperative oral nutrition supplements on postoperative outcomes in geriatric hip fracture patients: A PRISMA-compliant systematic review and meta-analysis of randomized controlled studies

Background:Geriatric hip fracture patients often present malnutrition during admission, which leads to higher morbidity and mortality. Protein-based oral nutrition supplements may improve nutritional status. We conducted this systematic review and meta-analysis of randomized controlled trials (RCTs) according to the PRISMA guidelines to elucidate whether preoperative nutrition supplements can improve postoperative outcomes in geriatric hip fracture patients.Methods:Only RCTs conducted to compare postoperative outcomes between geriatric hip fracture patients (>60 years old) receiving preoperative oral protein-based nutrition supplement (ONS group) and those who receiving regular diet (Control group) were included. PubMed, Embase, and Cochrane Central Register of Controlled Trials were searched from inception until August, 2021. Postoperative outcomes, including complications, length of hospital stay, and in-hospital mortality, were assessed.Results:A total of 5 RCTs with 654 geriatric hip fracture patients (ONS group: 320 subjects; Control group 334 subjects) were included. Our data revealed that postoperative complications risk in the ONS group was significantly lower than in the Control group (odd''s ratio: 0.48, 95% confidence intervals [CI]: 0.26–0.89, P = .02, I² = 64%). However, no significant differences in the length of hospital stay (standardized mean difference: −0.35 days, 95% CI: −1.68 to 0.98 days, P = .61, I² = 0%) and the risk of having postoperative in-hospital mortality (odd''s ratio: 1.07, 95% CI: 0.43–2.63, P = .89, I² = 54%) between these 2 groups were observed. Quality assessment revealed high risk of bias and significant data heterogeneity (I²>50%) in most included RCTs.Conclusion:Preoperative protein-based oral nutrition supplements exert beneficial, but limited, effects on postoperative outcomes in geriatric patients with hip fracture undergoing surgery.     

Effects of progressive neuromuscular stabilization exercise on the support surface on patients with high obesity with lumbar instability: A double-blinded randomized controlled trial

Background:Intensive neuromuscular stabilization exercise on highly obese patients with low back pain results in positive effects of body fat decline and prevention of complications. The purpose of this study is to investigate the effects of progressive neuromuscular stabilization exercise on unstable surface on pain, motor function, psychosocial factors, balance, and abdominal contraction with highly obese patients with lumbar instability.Methods:This study is a double-blinded randomized controlled trial. A total of 46 highly obese patients (body mass index [BMI] ≥ 30 kg/m²) with lumbar instability were assigned randomly to experimental group (n = 23) and control group (n = 23). The control group performed the intensive progressive exercise on a stable surface and the experimental group on an unstable surface.Result:Significant differences were shown for BMI, QVAS, K-ODI, FABQ, and balance ability for both groups before and after the intervention (P < .05), and only the experimental group showed significant difference for transverse abdominis muscle thickness in contraction and contraction rate (P < .05). Compared to the control group, the experimental group showed significant difference (P < .05) in the amount of changes for QVAS, K-ODI, balance ability, transverse abdominis muscle thickness in contraction, and contraction rate.Conclusion:Progressive neuromuscular stabilization exercise program on unstable surfaces demonstrated to be an effective and clinically useful method to decrease pain level, increase motor function, balance, and transverse abdominis muscle thickness in contraction and contraction rate for highly obese patients with lumbar instability.     

Spontaneous complete uterine rupture with protrusion of foetal limbs at the third trimester following laparoscopic cornuostomy: A case report

Rationale:Spontaneous complete uterine rupture during gestation is rare and has no specific symptoms; however, it is a life-threatening event for both the fetus and mother. The rupture typically happens in labor and is uncommon before labor. Herein, we present the case of a woman, encountering complete rupture at third trimester followed by laparoscopic cornuostomy.Patient concerns:A 26-year-old woman presented with acute right lower abdominal pain at 33 weeks and 5 days of gestation.Diagnoses:We made a diagnosis of threatened uterine rupture.Intervention:Urgent cesarean section performed. Exploration of the uterine dehiscence wound demonstrated that the myometrium was completely ruptured at the primary laparoscopic surgical scar with a defect of 40 mm, and live birth and preservation of the uterus was achieved.Outcome:On the third day of operation, she had a good recovery and was discharged. After a 6-week postpartum follow-up, she displayed a good level of rehabilitation.Lessons:Pregnancy after laparoscopic cornuostomy should be treated as high-risk gestation and the rupture during gestation of the uterine scar should be suspected once lower abdominal pain occurred. Swift diagnosis and prompt intervention play a crucial role in saving the lives of the fetus and the mother.     

Clinical use of platelet-rich fibrin in the repair of non-healing incision wounds after fibular fracture surgery: A case report

Rationale:This report describes rehabilitation for a 53-year-old female recovering from non-healing skin and soft tissue defect after distal tibial open fracture by using platelet-rich fibrin topical repair following an automobile accident.Patient concerns:The patient was admitted to a rehabilitation specialty hospital approximately 1 year post a fracture of the distal left tibiofibula and separate surgical tibiofibular fracture incision and internal fixation + bone grafting.Diagnoses:Clinical presentation included the left ankle incision was interrupted for about 3 cm with poor healing, a small amount of muscle necrosis, fat liquefaction, a large amount of yellow purulent secretion overflow and necrotic material was seen in the local wound.Interventions:Platelet rich fibrin (PRF) gel was injected into the wounds and submerged sites to make full contact with the wounds and close the wounds with the autologous platelet-rich fibrin prepared by mixing, and then covered with oil gauze to keep the wounds moist and promote granulation growth. The outermost layer was covered with cotton pads.Outcomes:After 30 days of 2 PRF treatments, the skin defect was healed and no significant abnormality was observed at 6 months follow-up.Lessons:Treatment with topical autologous platelet-rich plasma gel Significantly accelerates the healing of wounds, shortens healing time, improves healing quality and reduces scar formation without significant adverse effects.     

The association of OPG polymorphisms with risk of osteoporotic fractures: A systematic review and meta-analysis

Background:Subjects with low bone mineral density and osteoporosis are more likely to suffer osteoporotic fractures during their lifetime. Polymorphisms in osteoprotegerin (OPG) gene are found to be associated with low bone mineral density and osteoporosis risk but their association with fracture risk is inconclusive. Here, we performed a meta-analysis to investigate the relationship between OPG polymorphisms with susceptibility to osteoporotic fractures.Methods:Eligible studies investigating the association between common OPG polymorphisms (A164G, T245G, T950C, and G1181C) and risk of osteoporotic fracture were retrieved from PubMed, EMBASE, Web of Science, and the Cochrane Library. Odds ratio (OR) and the 95% confidence interval (CI) were calculated in the allelic, dominant, recessive, and homozygous model. Subgroup analyses of vertebral fractures, Caucasians, and postmenopausal women were also performed.Results:A total of 14 studies comprising 5459 fracture cases and 9860 non-fracture controls were included. A163G was associated with fracture risk in dominant (OR = 1.29, 95%CI 1.11–1.50), recessive (OR = 1.64, 95%CI 1.10–2.44), and homozygous model (OR = 1.73, 95%CI 1.16–2.59). T245G was significantly correlated with susceptibility to fractures in all genetic models. Subjects with CC genotype of T950C had a reduced risk of fracture compared to those with CT or TT genotypes (OR = 0.81, 95%CI 0.70–0.94, P = .004). Subgroup analysis showed that A163G and T245G but not T950C and G1181C were associated with vertebral fracture risk.Conclusion:OPG A163G and T245G polymorphisms were risk factors of osteoporotic fractures while T950C had a protective role. These polymorphisms can be used as predictive markers of fractures.     

Two cases of rt-PA with dual antiplatelet therapies with capsular warning syndrome

Rationale:Capsular warning syndrome (CWS) is a term to describe stereotyped lacunar transient ischemic attacks (TIAs). Patients with CWS are at high risk of developing completed stroke. However, the exact pathophysiology of CWS is still unclear, and there is no conclusive clinical strategy for CWS patients.Patient symptoms:Two cases of middle-aged men with hypertension, hyperlipidemia, and diabetes mellitus presented with fluctuating right-sided weakness, numbness, and dysarthria.Diagnoses:These two patients were diagnosed with CWS.Interventions:Recombinant tissue plasminogen activator (rt-PA) intravenous thrombolysis (0.9 mg/kg) was administered first and treated with aspirin (100 mg) and clopidogrel (75 mg) after 24 h of rt-PA for 21 days following by aspirin (100 mg) alone.Outcomes:Both cases got favorable clinical outcomes of somatic symptoms. In addition, diffusion-weighted imaging (DWI or DW-MRI) showed that ischemic injury disappeared in case 1 while maintained within a reasonable range in case 2.Lessons:Early recognition and rt-PA/dual antiplatelet treatment may be an effective strategy for patients with CWS.     

Spontaneous rupture of pyogenic liver abscess with subcapsular hemorrhage mimicking ruptured hepatocellular carcinoma: A case report

Rationale:Spontaneous rupture of PLA (pyogenic liver abscess) is an extremely rare and life-threatening event. Ruptured PLA is very difficult to distinguish from malignant HCC (hepatocellular cancer) rupture or cholangiocarcinoma rupture on CT (computed tomography) scan.Patient concerns:We describe the case of a 71-year-old man with fever, right upper abdominal pain, nausea with intermittent vomiting, and general fatigue. He had no medical or surgical history.Diagnosis:CT scan showed a hypodense mass in right hepatic lobe and MRI (magnetic resonance imaging) revealed a heterogenous mass of ∼6 cm in segment VI of the liver and heterogenous fluid in the subcapsular region. We made a tentative diagnosis of HCC rupture with subcapsular hemorrhage based on these findings.Intervention:After improving the patient''s condition by administering empirical therapy consisting of intravenous antibiotics and fluids, we performed surgical exploration. Gross examination of the abdomen showed that almost the entire right hepatic lobe was hemorrhagic and affected by peritonitis. Therefore, we performed right hepatectomy. The intraoperative frozen biopsy revealed suspicious PLA with marked necrosis, neutrophil infiltration, and hemorrhagic rupture, although no malignant tissue or fungus was observed. The postoperative secondary pathology report confirmed the diagnosis of PLA with hemorrhagic rupture.Outcomes:The patient was discharged 13 days after the operation. Follow-up CT was performed 5 months after discharge and revealed no abnormal findings.Lessons:A high index of suspicion is key to preventing misdiagnosis of ruptured PLA and improving prognosis. Furthermore, even if rupture of the PLA is initially localized, delayed peritonitis may occur during medical treatment. Therefore, vigilant monitoring is essential.     

Delayed transient obstructive hydrocephalus after cerebral aneurysm rupture: A case report

Rationale:Obstructive hydrocephalus (OH) frequently occurs in patients with a ruptured cerebral aneurysm (CA), and it may lead to severe neurological deficits, including life-threatening brain herniation. OH generally occurs in the early stage of CA rupture, rather than in the late stage, and rarely resolves without therapy.Patient concerns:A 64-year-old woman with a ruptured anterior communicating artery aneurysm was treated with coil embolization. Nineteen days after her CA rupture, because of the delayed transient OH, she experienced a dramatic cycle in consciousness over 9 hours: wakefulness–drowsiness–coma–drowsiness–wakefulness.Diagnosis:The patient was diagnosed with delayed transient obstructive hydrocephalus, which is a very rare condition.Interventions:Mannitol was administered to reduce intracranial pressure.Outcomes:The patient was discharged from the hospital 30 days after admission, with a final GCS score of 15 and without weaknesses. At follow-up 2 months after discharge, brain CT revealed non-recurrence of hydrocephalus.Lessons:A blood clot of any size in the ventricle is likely to lead to obstructive hydrocephalus. Prolonged bed rest for IVH patients may help to reduce the incidence of delayed OH.     

Comparison of small symptomatic and asymptomatic abdominal aortic aneurysms based on computational fluid dynamics analysis

Background:The purpose of this study was to compare the hemodynamic parameters of symptomatic and asymptomatic abdominal aortic aneurysms (AAAs) to explore the risk factors for AAA rupture.Methods:We conducted a retrospective analysis of 26 patients with symptomatic small AAAs and 60 patients with asymptomatic small AAAs. Computational fluid dynamics methods were used to compare hemodynamic characteristics between the symptomatic and asymptomatic groups and to evaluate risk factors for the occurrence of symptomatic AAAs.Results:The maximum diameters in the symptomatic and asymptomatic groups were 49.7 ± 4.94 mm and 48.4 ± 4.55 mm, respectively. Wall shear stress values at turbulent flow regions in the symptomatic and asymptomatic groups were 0.0098 ± 0.0084 Pa versus 0.0174 ± 0.0068 Pa, respectively. Shear stress values at the site with maximal blood flow impact force in the symptomatic and asymptomatic groups were 1.13 ± 0.466 Pa and 2.04 ± 0.42 Pa, respectively. The areas of the intra-luminal thrombus in the section with the maximum diameter in the symptomatic and asymptomatic groups were 952.19 ± 413.53 mm² versus 646.63 ± 296.88 mm², respectively.Conclusion:The wall shear stress in the symptomatic group was lower than that in the asymptomatic group.     

Meningioma preceding CASPR2 antibody limbic encephalitis with a stroke mimic: A case report

Rationale:Todd paralysis (a stroke-like presentation in some patients with epilepsy) caused by limbic encephalitis (LE) is not easily distinguished from acute ischemic stroke by clinicians in the emergency room.Patient concerns:We report a contactin-associated protein-like 2-antibody (CASPR2-Ab)-positive patient who presented with atypical LE.Diagnoses:CASPR2-Ab-positive LE was the presumed diagnosis. Re-evaluation of cerebrospinal fluid (CSF) samples revealed autoantibodies targeting CASPR2 at an immunoglobulin G titer of 1:1. The clinical presentation of subacute onset seizures, abnormal electroencephalography, hypermetabolism on positron emission tomography, good immunotherapy response, and the presence of specific antibodies in serum supports a diagnosis of autoimmune LE.Intervention:The patient received glucocorticoids (1 g for 3 days and 500 mg for 3 days), immunoglobulin (25 g for 3 days), sodium valproate (1 g for 3 days), and clonazepam (1 mg for 3 days).Outcomes:Remission of temporal lobe epilepsy symptoms and cognitive dysfunction was observed. Follow-up analysis of CSF and serological examination were not approved by the patient. His Mini-Mental State Examination score improved to 21/30. Stable remission of symptoms was achieved throughout the follow-up period of 50 days.Lessons:Autoimmune encephalitis (AE) should be considered in cases of late-onset epilepsy following meningioma peritumoral brain edema and resection. A diagnosis of AE should be considered in patients presenting with stroke-like symptoms if the magnetic resonance imaging abnormality does not match a known vascular territory. Early and correct diagnosis is crucial because immunotherapy is usually effective for this disease.     

Risk factors for anterior communicating artery aneurysm rupture: A protocol for systematic review and meta-analysis

Background:Although the research on the risk factors of anterior communicating artery (AComA) aneurysm has made great progress, the independent effect of each risk factor on the rupture of AComA aneurysm is controversial among different studies. We will perform a protocol for systematic review and meta-analysis to investigate risk factors for AComA aneurysm rupture and quantify their independent effects.Methods:A systematic search according to Preferred Reporting Items for Systematic Reviews and Meta-Analysis Protocols guidelines in PubMed, Embase, and the Cochrane Library databases was conducted from inception to August 31, 2021 for published studies concerning risk factors for AComA aneurysm rupture. In the absence of statistical heterogeneity (ie, P > .10 and I² < 50%), we will use a fixed-effects model to pool the results across sufficient studies. Otherwise, we will present the results employing the random-effects model. Quality assessment of the included studies will be evaluated using the Newcastle–Ottawa Scale. Statistical analyses will be performed using Stata16 (Stata Corporation, College Station, TX, USA) software.Results:The findings of this study will be submitted to peer-reviewed journals for publication.Conclusion:This systematic review will provide evidence to determine the risk factors that affect the rupture of the AComA aneurysm and quantify their independent effects.Ethics and dissemination:Since the proposed study uses pre-published data, ethical approval is not required.Review registration number:CRD42021284262. (https://www.crd.york.ac.uk/PROSPERO/).     

Electromyographic study assessing swallowing function in subacute stroke patients with respiratory muscle weakness

Background:Dysphagia has been reported to be associated with the descent of the hyolaryngeal complex. Further, suprahyoid muscles play a greater role than infrahyoid muscles in elevation of the hyolarngeal complex. Respiratory muscle training (RMT) can improve lung function, and expiratory muscle strength training can facilitate elevation of the hyoid bone and increase the motor unit recruitment of submental muscles during normal swallowing. This study aimed to investigate the surface electromyography (sEMG) of the swallowing muscles, bilaterally, and the effect of RMT on swallowing muscles in stroke patients with respiratory muscle weakness.Methods:Forty patients with first episode of unilateral stroke were included in this retrospective controlled trial. After exclusion of 11 patients with respiratory muscle strength stronger than 70% of the predicted value, 15 were allocated to the RMT group and 14 to the control group. However, eventually, 11 patients in RMT group and 11 patients in control group completed the study. The sEMG of the orbicularis oris, masseter, submental, and infrahyoid muscles were recorded during dry swallowing, water swallowing (2 mL), and forced exhalation against a threshold breathing trainer set at different intensities, at baseline and after 6-week RMT.Results:Regarding the sEMG of submental muscles, there were significant between-group differences on the latency of the unaffected side (P = .048), significant change from baseline force on the unaffected side (P = .035), and significant between-side difference (P = .011) in the RMT group during dry swallowing. Significant change in the duration from baseline was observed on the affected side of the RMT group when blowing was set at 50% maximal expiratory pressure (MEP; P = .015), and on the unaffected side of the control group when blowing set at 15% MEP (P = .005). Significant difference was observed in the duration between 50% MEP and 15% MEP after 6-week program in the control group (P = .049).Conclusions:A 6-week RMT can improve the electric signal of the affected swallowing muscles with more effect on the unaffected side than on the affected side during dry swallowing. Furthermore, RMT with 50% MEP rather than 15% MEP can facilitate greater submental muscle activity on the affected side in stroke patients with respiratory muscle weakness.     

Sporadic amyotrophic lateral sclerosis with seropositive neuromyelitis optica spectrum disorder: A case report

Rationale:Neuromyelitis optica spectrum disorder (NMOSD) is a severe inflammatory disorder of the central nervous system with an autoantibody against aquaporin-4 protein (AQP4), and amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. We report a female patient with ALS who had asymptomatic AQP4 antibody at the diagnosis of ALS, and NMOSD occurred 4 years later after the diagnosis of ALS.Patient concerns:She was already bedridden and had tracheostomy because of ALS which was diagnosed at her age of 55. At the time of her ALS diagnosis, she had no brain or spinal cord lesions, but was seropositive for AQP4 antibody. At her age of 59, new-onset complete paralysis of all extremities and severe pain on the posterior neck and both shoulders occurred and visited the hospital.Diagnosis:Longitudinally extensive transverse myelitis was diagnosed, which was the onset attack of seropositive NMOSD. The diagnosis was confirmed based on the international consensus diagnostic criteria for NMOSD with MR imaging, cerebrospinal fluid exam and laboratory work-ups with AQP4 antibody test.Interventions:High dose methylprednisolone was administered for 5 days. Plasma exchange as a further treatment was recommended, but she and her family refused.Outcomes:Her pain was relieved after steroid treatment, but there was no improvement of her leg weakness.Lessons:This case is a rare combination of neuroinflammatory and neurodegenerative diseases. Considering the alterations of blood-brain barrier along with the progression of ALS, it highlights that the consequence of ALS pathogenesis might affect the development of NMOSD. And the careful follow-up is recommended even in patients with profound weakness, especially if those who were at risk of developing certain neurological disorders.     

Effect of Nusinersen in a late onset spinal muscular atrophy patient for 14 months: A case report

Rationale:Spinal muscular atrophy (SMA) is a genetic disorder caused by genetic defect of SMN1 gene. SMA was an untreatable disease until 2016, when Nusinersen an antisense oligonucleotide therapy was approved for treatment. We report the effect of Nusinersen in a late onset SMA for 14 months.Patient concerns:A 13-year-old boy who was diagnosed as SMA with progressive proximal limb weakness was treated with intrathecal injection of Nusinersen.Diagnosis:The patient had progressive proximal limb weakness after 2 years of age. The patient had elevated creatine kinase level and shoed neurogenic changes in the needle electromyography study. After genetic analysis, homozygous deletion in Exon 7 and 8 of SMN1 protein was found and he was diagnosed as late onset SMA.Interventions:Intrathecal Nusinersen was administered per protocol.Outcomes:After 14 months of treatment, the patient showed significant clinical improvement in the revised Hammersmith functional rating scale and 6-minute walk test.Lessons:Although there is limited data on the effect of Nusinersen in late onset SMA patients, our case adds on the effectiveness even in late onset SMA. More studies are needed to consolidate the effects and adverse events of Nusinersen in late onset SMA.