Transient Weakness Research Articles

Efficacy and safety of ultrasound guided stellate ganglion block: developing new clinical indications beyond electrical storm

Abstract Background Percutaneous stellate ganglion block (PSGB) is recommended by the latest European guidelines for patients with drug and ventricular tachycardia (VT) ablation refractory electrical storm (ES). Yet, due to the strong antiarrhythmic potential, combined with the good safety profile, we’ve recently used it for to the prophylaxis of ventricular arrhythmias (VAs) in high-risk patients and the prophylaxis/treatment of atrial arrhythmias in patients with acute heart failure (HF). Purpose To describe our single-center experience with PSGB usage outside the conventional indication of ES. Methods We hereby describe our single center experience with PSGB from 2/2021 to 2/2024. Results 56 patients (87% male, mean age 64 ± 12 years) received a total of 91 PSGB performed with the lateral, ultrasound (US) guided technique. Most of the procedures consisted of a single bolus anesthetic injection of lidocaine plus ropivacaine, 27% in an additional continuous infusion, mainly with ropivacaine. All procedures except for 3 in a single patient who had previously received left cardiac sympathetic denervation, were performed on the left side. Most of the patients (61%) suffered ischemic cardiomyopathy (CMP), including 11 with an acute coronary syndrome; the rest had non-ischemic CMP. 63 PSGB (69%) were performed in patients with impending or manifested cardiogenic shock (SCAI classification B or more). Mean LVEF was 24 ± 12%. Most of the procedures (n=80, 88%) were performed due to ongoing refractory VAs, yet 6 (6%) aimed to prevent major VAs in high-risk patients, mostly in the setting of recent ES (within 1 month) and need for Levosimendan to support cardiac output, in one case due to recent stereotactic VT ablation, to prevent early VAs in the phase of acute radiation induced microvascular damage. All 6 were effective in preventing clinically significant VAs. Additionally, 5 single bolus PSGBs (5%) were performed due to atrial arrhythmias with high ventricular rate despite intravenous drugs in the setting of acute HF. Specifically, 3 patients had atrial fibrillation (AF), 1 patient runs of ectopic atrial tachycardia (AT) and 1 patient 2:1 atrial flutter. Left-sided PSGB significantly reduced (≥25% reduction) ventricular rate during AF and AT but not during the single case of 2:1 atrial flutter, in 2/3 AF cases a cardioversion into sinus rhythm occurred within 40 minutes from PSGB. Only 1 (1%) major complication occurred (respiratory arrest), that was quickly and effectively treated with lipid emulsion, while minor complications were observed in 11% of PSGBs (mostly transient left arm weakness). Conclusions Our data suggest that US-guided PSGB usage, thanks to its feasibility at bed-side and good safety profile, may expand, beyond ES, to not only VAs prevention in high-risk settings, but also to prophylaxis/treatment of atrial arrhythmias in critically ill patients with acute heart failure requiring concomitant inotropic support.

The Role of Cage Placement Angle in Optimizing Short-Term Clinical Outcomes in Lateral Lumbar Interbody Fusion

OBJECTIVEThe purpose is to investigate the impact of cage angle on clinical outcomes and indirect decompression efficacy in patients undergoing lateral lumbar interbody fusion (LLIF). METHODSA retrospective review was conducted on 87 patients with single-level lumbar degenerative disease (LDD) who underwent LLIF. Patients were grouped based on the angle of cage placement: minimal (0°-5°), mild (>5° ≤ 15°), and severe (>15°). Clinical outcomes assessed included pain intensity, functional improvement, and complication rates. Magnetic resonance imaging (MRI) evaluations included measurements of canal diameter (CD) and central canal area (CCA) pre- and postoperatively. Patient-reported outcomes were also analyzed using the Japanese Orthopaedic Association Back Pain Evaluation Questionnaire (JOABPEQ). RESULTSClinical and radiographic outcomes were significantly improved across all cage angle groups. Reductions in low back pain, leg pain, and numbness were significant in all groups, with no significant differences. MRI evaluations revealed significant increases in CD and CCA postoperatively, confirming effective indirect decompression. JOABPEQ scores showed significant improvements in all domains, including low back pain, lumbar function, walking ability, social life function, and mental health. However, the severe angle group had higher rates of delayed cage subsidence (DCS). Complications such as transient motor weakness, thigh pain, numbness, and the need for revision surgery were consistent across groups, with no significant differences. CONCLUSIONSLLIF effectively treats LDD patients, providing significant short-term clinical and radiographic improvements regardless of cage angle. However, oblique cage placement increases the risk of cage subsidence, requiring careful surgical planning and postoperative following.

Diagnostic Challenges in Identifying Atrial Myxoma as a Cause of Transient Ischemic Attacks: A Case Report

Abstract Background A 67-year-old woman presented to hospital on multiple occasions with transient neurological symptoms over a period of 20 months. Initial diagnostics, including computed tomography (CT), carotid Dopplers, and electrocardiogram, were unremarkable. Magnetic resonance imaging (MRI) was arranged following the third neurological event which revealed multi-territory acute ischemic events, suggestive of a cardioembolic source. Transoesophageal echocardiography identified a large mobile atrial myxoma, histologically confirmed. Atrial myxoma, though rare, is an important cause of thromboembolic stroke, underscoring the need for comprehensive cardiac evaluation in cerebrovascular incidents. Methods The patient's case was reviewed, focusing on initial presentations, diagnostic workup, and subsequent management. Initial presentations included transient visual disturbances and transient leg weakness. Routine tests were conducted, including CT brain, carotid Dopplers, and blood tests. MRI and transoesophageal echocardiography were performed which ultimately identified the underlying cause of the patient’s neurological events. Results Initial evaluations (CT, carotid Dopplers) were normal. An MRI performed 20 months after her initial presentation revealed small acute infarcts in the right cerebellar hemisphere and left superior parietal lobe. Transoesophageal echocardiography subsequently detected a large atrial myxoma. The patient's transient symptoms and negative initial workup delayed the diagnosis, highlighting the necessity of including cardiac imaging early in the stroke workup, especially in cases of suspected cardioembolic events. Conclusion This case illustrates the critical role of thorough cardiac evaluation, including echocardiography, in diagnosing the cause of transient ischemic attacks (TIA) and strokes. The patient’s initial negative findings delayed the identification of a cardioembolic source, emphasizing the importance of comprehensive diagnostic protocols for suspected cerebrovascular events. Urgent surgical resection is essential for atrial myxoma to prevent recurrent embolic events. This report highlights the diagnostic and management challenges of cardiac myxomas, advocating for a more rigorous approach to stroke evaluation and timely intervention.

Use of Botulinum Toxin in Upper-Limb Tremor: Systematic Review and Perspectives.

Tremor is the most common movement disorder, with significant functional and psychosocial consequences. Oral medications have been disappointing or limited by side effects. Surgical techniques are effective but associated with risks and adverse events. Botulinum toxin (BT) represents a promising avenue but there is still no double-blind evidence of efficacy on upper limb function. A systematic review on the effects of BT in upper-limb tremor was conducted. A systematic search of the literature was conducted up to July 2023, including the keywords "botulinum toxin" and "tremor". All randomized controlled trials (RCTs) and open-label studies were analyzed. Independent reviewers assessed their methodological quality. There were only eight published RCTs and seven published open-label studies, with relatively small sample sizes. This review suggests that BT is more effective when injections are patient-tailored, with analyses based on clinical judgement or kinematics. Subjective and objective measures frequently improve but transient weakness may occur after injections, especially if wrist or fingers extensors are targeted. A number of studies had methodological limitations. The authors discuss how to optimize tremor assessments and effects of BT injection. Controlled evidence is still lacking but it is suggested that distal "asymmetric" BT injections (targeting flexors/pronators while sparing extensors/supinators) and proximal injections, involving shoulder rotators when indicated, may avoid excessive weakness while optimizing functional benefit.

Bilateral Todd's paralysis in a patient with left fronto-opercular epilepsy.

Postictal paresis ("Todd's paralysis") is commonly observed as a unilateral, transient motor weakness, lasting minutes to hours, after focal or focal to bilateral tonic-clonic seizures, contralateral to the epileptogenic zone. Bilateral postictal paresis is exceedingly rare and could be misinterpreted, especially if the preceding convulsive phase was not witnessed. An 18-year-old right-handed male patient with refractory focal epilepsy with seizure onset at age 3 years, was admitted for presurgical video-EEG monitoring. His seizures were predominantly nocturnal, consisting of a laryngeal somatosensory aura, occasionally evolving to bilateral tonic or tonic-clonic seizures with occasional asymmetrical limb extension during the tonic phase (right arm extension). Postictally, consciousness recovery was fast, if ever lost. At that stage, we documented severe dysarthria and bilateral symmetrical arm paresis lasting several minutes. The ictal pattern and interictal epileptiform activity were projected on the fronto-central midline. Brain MRI was highly suggestive of a bottom-of-sulcus dysplasia with underlying transmantle sign on the left premotor, fronto-opercular region and an FDG-PET-CT showed a concordant left fronto-operculo-insular hypometabolism. A complete lesionectomy was performed, with the additional guidance of intraoperative electrocorticography, resulting in sustained seizure freedom. Anatomo-pathology confirmed a type 2b focal cortical dysplasia. We speculate that, in our patient, a left fronto-opercular ictal onset with an early spread to both primary motor cortices and relative sparing of consciousness networks allowed the emergence of a clinically detectable postictal bilateral paresis.

Frontal Disconnection for Treating Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Epilepsy (MOGHE) in the Frontal Lobe.

Malformation of cortical development is an important cause of drug-resistant epilepsy in young children. Mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy (MOGHE) has been added to the last focal cortical dysplasia (FCD) classification and commonly involves the frontal lobe. The semiology at the onset of epilepsy is dominated by non-lateralizing infantile spasm; the boundaries of the malformation are usually difficult to determine by magnetic resonance imaging (MRI) and positron emission tomography (PET), and electroencephalography (EEG) findings are often widespread. Therefore, the traditional concept and strategy of preoperative evaluation to determine the extent of the epileptogenic zone by comprehensive anatomo-electro-clinical methods are difficult to implement. Frontal disconnection is an effective surgical method for the treatment of epilepsy, but there are few related reports. A total of 8 children with histo-pathologically confirmed MOGHE were retrospectively studied. MOGHE was located in the frontal lobe in all patients, and frontal disconnection was performed. The periinsular approach was used in the disconnective procedures, divided into several surgical steps: the partial inferior frontal gyrus resection, the frontobasal and intrafrontal disconnection, and the anterior corpus callosotomy. One patient presented with a short-term postoperative speech disorder, while another patient exhibited transient postoperative limb weakness. No long-term postoperative complications were observed. At 2 years after surgery, 75% of patients were seizure-free, with cognitive improvement in half of them. This finding suggested that frontal disconnection is an effective and safe surgical procedure for the treatment of MOGHE instead of extensive resection in the frontal lobe.

Imaging of the Pediatric Knee.

During normal development, imaging findings in the immature knee joint may mimic pathology or indicate transient sites of weakness, prone to injury. This article reviews the development of the knee joint, age- and maturation-dependent imaging considerations, and various developmental variants that can be encountered, subdivided into those that involve the tibiofemoral and patellofemoral compartments, soft tissues, and osseous components. The tibiofemoral compartment section reviews the focal periphyseal edema zone (FOPE), ossification variants of the femoral condyles, distal femoral metaphyseal cortical irregularity from periosteal traction, and the metaphyseal subperiosteal stripe, which should be distinguished from pathologic mimickers such as endochondral ossification dysfunction, osteochondritis dissecans (OCD), fibroosseous lesion, periosteal and subcortical pathologies. The patellofemoral compartment section includes a review of partite patella, dorsolateral defect, variant trochlear morphology, and maturation-dependent sites of transient weakness that are prone to injury from repetitive overuse (Sinding-Larsen-Johansson syndrome and Osgood-Schlatter disease) and avulsion fractures (patellar sleeve and tibial tubercle avulsions). Finally, soft tissue (discoid lateral meniscus, meniscal flounce, anterior cruciate ligament variants) and osseous components (meniscal ossicle, fabella, and cyamella) are reviewed.

Parotidectomy for deep lobe pleomorphic adenomas is associated with higher rates of complications and recurrence.

Pleomorphic adenoma (PA) is a common parotid tumor, yet due to the relative rarity of deep lobe PA (DLPA), there is a paucity of information about its clinical presentation and surgical outcomes. We reviewed the charts of patients with previously untreated parotid PA between the years 1990 and 2015. Clinical parameters and surgical outcomes were compared between superficial lobe PA (SLPA) and DLPA. The cohort comprised 147 cases of DLPA and 222 cases of SLPA. DLPA were larger (median 2.6 cm vs. 2.0 cm, p < 0.001), more often discovered incidentally on imaging (33%, n = 48) and had unique presentations (pharyngeal mass, dysphagia, otalgia). Postsurgical complications were more frequently observed in DLPA (41% vs. 30% in SLPA, p = 0.025), mainly transient facial nerve weakness. DLPA also showed higher recurrence rates (n = 6, 4.1% vs. n = 1, 0.4%, p = 0.016). Parotidectomy for DLPA carries a higher risk of complications and recurrence compared to SLPA.

Surgical treatment of brain arteriovenous malformations in patients with hereditary hemorrhagic telangiectasia: a single-center experience

OBJECTIVE Hereditary hemorrhagic telangiectasia (HHT) is an autosomal-dominant disorder characterized by multiple vascular malformations. Brain arteriovenous malformations (bAVMs) are a significant manifestation of HHT. The surgical management of these lesions in patients with HHT remains debated, with limited literature on postoperative outcomes. The goal of this study was to evaluate the safety and efficacy of surgical treatment for bAVMs in patients with HHT and propose a treatment rationale based on a single-center experience. METHODS This retrospective review included 20 patients diagnosed with HHT who underwent resection of 23 bAVMs at the Stanford University Medical Center between January 2007 and September 2023. Data were also collected on bAVMs treated conservatively, with embolization, or with radiosurgery at the authors’ institution, for comparison. RESULTS There were 16 Spetzler-Martin (SM) grade I, 6 SM grade II, and 1 SM grade IV bAVM. Six of the bAVMs presented with neurological symptoms (3 with hemorrhage and 3 with focal neurological deficits), while the rest were detected on routine screening. Complete excision was angiographically confirmed in all patients, with a mean overall hospital stay of 2.1 days and a mean follow-up of 36 months. Postoperative complications were limited to transient mild weakness in 2 patients, 1 of whom also had transient speech deficits, and visual field deficits in 3 patients, 2 of whom improved on long-term follow-up. CONCLUSIONS In this most extensive surgical series published to date, resection of bAVMs in patients with HHT showed favorable outcomes with a low complication rate, suggesting that the benefits of surgery outweigh the risks, especially considering the potential cumulative lifetime risk of hemorrhage. MR arterial spin labeling was found to be the most sensitive noninvasive measure of detecting bAVMs in patients with HHT.

PAROTID TUMORS; PRESENTATION, SURGICAL MANAGEMENT AND COMPLICATIONS

Background: The parotid gland is the most common salivary gland affected by tumors, 80% of them affects the parotid glands and 80% affects the superficial lobe. Pleomorphic adenoma is the commonest salivary tumor. Other types of tumors occur less frequently. Most tumors are diagnosed with ultrasound; MRI shows more anatomical details. Superficial parotidectomy is done for benign tumors, more extensive resection is done for malignant tumors. Results: This a cross sectional study which included 69 patients who underwent various types of parotid surgeries over a period of 4 years. The mean age of our patients was 45.29 years, and 52.2% were males. The tumor was present in the right parotid gland in 49.3%, in the left side in 43.5%, and was bilateral in 2.9%. The mean duration of symptoms was 29.67 months, all patients were sent for ultrasound and 42 patients were sent for the FNAC, 91.3% of patients underwent superficial parotidectomy. Pleomorphic adenoma was the commonest type of tumors which was diagnosed in 69.6%, followed by Warthin’s tumors in 13%, Non-Hodgkin's lymphoma in 4.3%, and the rest comprises some rare types of tumors. Complications were reported in 30.43% of patients and the commonest complications were transient facial nerve weakness followed by salivary leak (11.6 and 8.7 %) respectively. Conclusion: Parotid tumors are rare types of tumors and pleomorphic adenoma is the commonest type, identifying the anatomy during surgery especially the course of the facial nerve is the most important step, complications are common but fortunately most are transient.

An in vitro model for postoperative cranial nerve dysfunction and a proposed method of rehabilitation with N-acetylcysteine microparticles

PurposeWhen operating near cranial motor nerves, transient postoperative weakness of target muscles lasting weeks to months is often observed. As nerves are typically intact at a procedure’s completion, paresis is hypothesized to result from a combination of neurapraxia and axonotmesis. As both neurapraxia and axonotmesis involve Schwann cell injury and require remyelination, we developed an in vitro RSC96 Schwann cell model of injury using hydrogen peroxide (H2O2) to induce oxidative stress and investigated the efficacy of candidate therapeutic agents to promote RSC96 viability. As a first step in developing a long-term local administration strategy, the most promising of these agents was incorporated into sustained-release microparticles and investigated for bioactivity using this assay.MethodsThe concentration of H2O2 which reduced viability by 50% was determined to establish a standard for inducing oxidative stress in RSC96 cultures. Fresh cultures were then co-dosed with H2O2 and the potential therapeutics melatonin, N-acetylcysteine, resveratrol, and 4-aminopyridine. Schwann cell viability was evaluated and the most efficacious agent, N-acetylcysteine, was encapsulated into microparticles. Eluted samples of N-acetylcysteine from microparticles was evaluated for retained bioactivity.Results100 µM N-acetylcysteine improved the viability of Schwann cells dosed with H2O2. 100 µM Microparticle-eluted N-acetylcysteine also enhanced Schwann cell viability.ConclusionWe developed a Schwann cell culture model of iatrogenic nerve injury and used this to identify N-acetylcysteine as an agent to promote recovery. N-acetylcysteine was packaged into microparticles and demonstrated promise as a locally administrable agent to reduce oxidative stress in Schwann cells.

A surgical resection case of myxoma arising from the posterior wall of the left atrium complicated with complete atrioventricular block

An 80-year-old female was referred to our institution due to transient right upper limb weakness. Transthoracic and transesophageal echocardiography revealed a tumor in the left atrium. The tumor was attached to the posterior wall of the left atrium near the atrioventricular node. Intraoperative pathological examination revealed that the tumor was a myxoma, and complete resection was successfully performed. However, she experienced persistent complete atrioventricular block postoperatively and required pacemaker implantation.

Quantifying Satisfaction of Degenerative Lumbar Spinal Stenosis Patients in Post-lateral Lumbar Interbody Fusion Surgery: Establishing Critical JOABPEQ Cutoff Scores

This retrospective study aimed to determine the Japanese Orthopedic Association Back Pain Evaluation Questionnaire (JOABPEQ) cutoff scores for assessing patient satisfaction post-lateral lumbar interbody fusion (LLIF) in degenerative lumbar spinal stenosis (DLSS) patients. Analyzing 136 DLSS patients (83 males, 53 females), the study evaluated demographics, pain (Numeric Rating Scale), and JOABPEQ outcomes (low back pain, lumbar function, walking ability, social life, mental health). Patient satisfaction was surveyed, and based on their responses, patients were categorized into "Beneficial" and "Non-Beneficial" groups. Statistical analysis encompassed the Kolmogorov-Smirnov test, t-tests, Mann-Whitney U test, and Receiver Operating Characteristic (ROC) curve analysis for JOABPEQ cutoff determination. Postoperative improvements in JOABPEQ scores, especially in walking ability, social life function, and mental health, were significant. Pain intensity, assessed using the Numeric Rating Scale, also showed notable reductions. The Δwalking ability cutoff was set at 25.00, indicating substantial mobility improvement. This domain's area under the curve (AUC) was 0.815 (95% CI: 0.726-0.903), demonstrating high effectiveness in assessing patient satisfaction post-surgery. The study also found no significant differences in complication rates between groups for conditions like transient motor weakness, thigh pain/numbness, and revision surgery. This study underscores the value of patient-centered outcomes in evaluating LLIF surgery success for DLSS. The identified JOABPEQ cutoff values provide a quantitative tool for assessing patient satisfaction, emphasizing the necessity of comprehensive postoperative evaluations beyond traditional clinical metrics for improved patient care and life quality.

True Incidence of Marginal Mandibular Nerve Palsy Following Neonatal Mandibular Distraction Osteogenesis.

In children with PRS, MDO is routinely performed to alleviate airway obstruction; however, it involves risk of injury to the MMN. We hypothesize that MMN palsy incidence following MDO, reported at 1-15%, is underestimated. This study investigates the true incidence of MMN palsy after MDO to better guide follow-up care and improve treatment of this complication. A retrospective review of PRS patients who underwent MDO at a single, tertiary pediatric hospital between September 2007 and March 2021 was conducted. Patients who underwent MDO under one year of age and had postoperative clinical evaluations detailing MMN function were included. Logistic regression analysis was performed to investigate predictors of MMN injury. Of 93 patients who underwent MDO, 59.1% met inclusion criteria. 56.4% were female, 43.6% were syndromic, and average age at MDO was 1.52 ± 2.04 months. The average length of mandibular distraction was 17.3 ± 4.36mm, average duration of intubation was 6.57 ± 2.37 days, and average time until hardware removal was 111.1 ± 23.6 days. Sixteen patients (29.1%) presented with permanent MMN dysfunction, comprised of 8 patients with bilateral weakness and 8 with unilateral weakness. An additional five patients (9.1%) presented with transient MMN weakness that resolved within a year. Average length of follow-up postoperatively was 6.02 years, and no significant predictors of nerve injury were found. In this 14-year review of patients with PRS who underwent MDO, 38.2% demonstrated evidence of MMN palsy (29.1% permanent, 9.1% transient), which is much greater than previously described.

Ligasure-assisted submandibular gland excision in deep plane neck lift: review of 83 patients.

In performing a neck lift, the size and location of the submandibular gland (SMG) must be evaluated to achieve the ideal neck contour. Many surgeons, however, avoid SMG excision because of the technical difficulty, risks of hematoma, and nerve damage. LigaSure™ (Valleylab, Boulder, CO) is a bipolar energy-based instrument that permanently seals vessels and connective tissue which can make the SMG excision step easy and safe by reducing surgical risks. A total of 83 patients who underwent SMG excision between 2018 and 2022 were included in the study. All patients' SMGs were partially excised by LigaSure™ (LS), and the complication rates were documented. Patients were followed up for an average of 21 months (9 months-2.4 years). No perioperative intraglandular bleeding or postoperative hematoma was observed in any of the patients. No sialoma was observed in any of the patients postoperatively. Only three patients underwent repeated seroma aspirations. Transient lower lip weakness was observed in 7 patients which all recovered in the first 6 months postoperatively. LS-assisted SMG excision in deep neck lift surgery was found to be technically easier, time-saving, safe, and highly effective in preventing hematoma and intraoperative bleeding.

Does the use of tranexamic acid intraoperatively reduce postoperative blood loss and complications following biportal endoscopic lumbosacral decompression?

Biportal endoscopic spine surgery is an effective minimally invasive technique for treating common lumbar pathologies. We aim to evaluate the impact of intraoperative tranexamic acid (TXA) use on postoperative blood loss in biportal endoscopic decompression surgery. Patients undergoing biportal endoscopic lumbar discectomies and decompressions either by same day surgery or overnight stay at a single institution beginning in October 2021 were prospectively enrolled. This study was non-randomized, non-blinded with the first cohort of consecutive patients receiving 1 g of intravenous TXA intra-operatively before closure and the second cohort of consecutive patients receiving no TXA. Exclusion criteria included any revision surgery, any surgery for the diagnosis of spinal instability, infection, tumor, or trauma, any contraindication for TXA. Eighty-four patients were included in the study, with 45 (54%) receiving TXA and 39 (46%) not receiving TXA. Median follow-up was 168 days [interquartile range (IQR), 85-368 days]. There were no differences in patient or surgical characteristics between cohorts. Estimated blood loss (EBL) was similar (P=0.20), while post-operative drain output was significantly lower in the TXA cohort (P=0.0028). Single level discectomies had significantly less drain output as compared to 2 level unilateral laminotomy, bilateral decompression (ULBD) cases (P<0.005). Post-operative complications were similar, with low rates of wound complication (1.2%) and transient postoperative weakness (2.4%, P>0.99 for both). Oswestry disability index (ODI), visual analog scale (VAS) back and VAS leg scores decreased significantly; the absolute decrease in scores did not differ between groups (P=0.71, 0.22, 0.86, respectively). Systemic intraoperative TXA administration is associated with a significant decrease in post-operative blood loss in biportal spinal endoscopy, with no impact on the improvement in patient-reported outcomes (PROs) or rate of post-operative complications. Single level biportal discectomies had significantly less postoperative drainage with TXA and may not need drains postoperatively. Larger, randomized studies are necessary to evaluate the cost-effectiveness of TXA use in biportal spinal endoscopy.

Neuromyelitis Optica with Negative AQP-4 Antibody

Abstract&#x0D; Introduction : Neuromyelitis Optica (NMO) or neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease of the central nervous system that causes demyelination of the optic nerve and spinal cord. Approximately 75% of cases have antibodies against aquaporin 4 (AQP-4 Antibody).&#x0D; Case Illustration : A 42 years old male presented with progressing blurred vision in his right eye for 3 weeks. He described there was a black spot in his central vision which gradually enlarged to his peripheral vision, headache for 4 months, and transient tingling sensation and weakness on the left arm for 1 week. Positive RAPD in the right eye and signs of optic neuritis on funduscopy in Opthalmology examination. Campimetry examination revealed generalized depression in the right eye. The visual evoked potential (VEP) was consistent with right optic nerve demyelination. Spine MRI showed extensive transverse myelitis lesions &gt;3 spinal cord hyperintensity on the level of C2-C6. However, the serum AQP-4 antibody was negative. The patient was treated with corticosteroid, and neuroprotector and was also being treated alongside the neurology department.&#x0D; Discussion : NMO is a rare autoimmune disease. NMO is characterized by segmented demyelination and inflammation of the spinal cord and the optic nerves which gives many ocular signs and symptoms like optic neuritis. A negative test result does not rule out the diagnosis of NMO. Brain and spine MRI plays an important role in diagnosing NMO. Treatment of NMO consists of corticosteroid and plasmapheresis or intravenous immunoglobulin.&#x0D; Conclusion : This case describes a diagnosis of NMO with a Negative AQP-4 antibody.

The complex treatment paradigms for concomitant tethered cord and scoliosis: illustrative case.

Scoliosis associated with tethered cord syndrome is one of the most challenging spinal deformities to manage. Multiple surgical approaches have been developed, including traditional staged and concomitant procedures, spine-shortening osteotomies, and individual vertebral column resections. A 10-year-old female presented with congenital kyphoscoliosis with worsening curve progression, tethered spinal cord, and a history of enuresis. The scoliosis had progressed to a 26° coronal curve and 55° thoracolumbar kyphosis. Preoperative magnetic resonance imaging of the spine revealed a tethered cord between the levels of L3-4 and a large kyphotic deformity at L1. The patient underwent laminectomy, during which intraoperative motor signals were lost. A planned hemivertebrectomy at L1 was performed prior to an L4 laminectomy, untethering of the filum terminale, and posterior spinal fusion from T11 to L2. After surgery, the patient experienced transient lower-extremity weakness, with her neurological function improving from baseline over the next 2 months. Ultimately, the goal of this surgery was to halt the progressive decline in motor function, which was successfully achieved. Much remains to be learned about the treatment of this complicated disease, especially in the setting of concomitant scoliosis. This case serves to exemplify the complex treatment paradigms that exist when attempting to manage this clinical syndrome and that more remains to be learned.

A pedigree of myotonia congenita with a novel mutation p.F343C of the CLCN1 gene

A Japanese woman experienced slowness of movement in her early teens and difficulty in opening her hands during pregnancy. On admission to our hospital at 42 years of age, she showed grip myotonia with warm-up phenomenon. However, she had neither muscle weakness, muscle atrophy, cold-induced symptomatic worsening nor episodes of transient weakness of the extremities. Needle electromyography of the first dorsal interosseous and anterior tibial muscles demonstrated myotonic discharges. Whole exome sequencing of the patient revealed a heterozygous single-base substitution in the CLCN1 gene (c.1028T>G, p.F343C). The same substitution was identified in affected members of her family (mother and brother) by Sanger sequencing, but not in healthy family members (father and a different brother). We diagnosed myotonia congenita (Thomsen disease) with a novel CLCN1 mutation in this pedigree. This mutation causes a single amino acid substitution in the I-J extracellular loop region of CLCN1. Amino acid changes in the I-J loop region are rare in an autosomal-dominantly inherited form of myotonia congenita. We think that this pedigree is precious to understand the pathogenesis of myotonia congenita.

Andersen-Tawil syndrome.

Andersen-Tawil syndrome (ATS) is one of the periodic paralyses, a set of skeletal muscle disorders that cause transient weakness of the arms and legs lasting minutes to many hours. Distinguishing features of ATS include facial and limb dysmorphisms, cardiac arrhythmia, difficulties with executive function, and association with dominant mutations in the potassium channel, KCNJ2. In this review, we discuss the key features of ATS, diagnostic testing, pathophysiology and treatment of ATS, and compare them with other periodic paralyses.