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Nitrous oxide is an anesthetic medication which can also be recreationally abused in the form of whippet canisters. Its prolonged abuse can interfere with Vitamin B12 metabolism and lead to its functional deficiency. We report a case of a 30-year-old male who presented with generalized weakness and was found to have subacute combined degeneration (SCD) of the spinal cord. His laboratory workup showed low Vitamin B12 with elevated homocysteine and methylmalonic Co-A levels, and further questioning revealed prolonged nitrous oxide abuse. Nitrous oxide causes functional inactivation of methylcobalamin by rendering it unable to function as a coenzyme for methionine synthase enzyme. This leads to the decreased production of methionine and subsequent production of myelin. This case describes nitrous oxide abuse as an important etiology to be considered in patients presenting with weakness and myeloneuropathy and describes important imaging findings.

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The anatomical structure of the medulla oblongata is complex, its nerve fibers are dense, and its blood vessels are complex. Clinical manifestations of ischemic damage to the medulla oblongata are therefore relatively diverse, and include vertigo, dysphagia, and dysarthria. Although facial paralysis may also occur, medullary infarction with facial paralysis as the first and only symptom is rare. Herein, we report a case of medullary infarction with ipsilateral central facial paralysis as the only symptom.

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BACKGROUND: Moebius syndrome (MoS), a rare congenital condition caused by the underdevelopment of the sixth and seventh cranial nerves, presents with uni- or bilateral facial paralysis and lateral gaze palsy. Those with MoS often have incomplete eyelid closure (lagophthalmos). This study aimed to investigate the experiences of individuals living with incomplete eyelid closure due to MoS. METHODS: Participants shared their experiences in semi-structured open-ended focus groups during the 2023 MoS Foundation Conference. Data were analyzed thematically using Nvivo. The Terzis and Bruno scoring system was used to grade participant eyelid closure (range: 1 being no eyelid closure with full scleral show to 5 being complete eyelid closure with no scleral show) and blink (from 1 being no blink to 5 being synchronous and complete blink present). Marginal reflex distances 1 and 2 (MRD1 and MRD2) were measured to grade for ptosis and lid retraction, respectively. RESULTS: Fifteen participants participated in two focus groups, comprising adults (n = 12) and adolescents (n = 3). All participants had lagophthalmos with some scleral show, ptosis, and lid retraction. The median eyelid closure score was 3 (incomplete eye closure with 1/3 scleral showing). Five key themes were identified: social stigma and misunderstanding, daily life impacts, seasonal exacerbations, different attitudes toward surgical intervention between adults and adolescents, and a prevailing sense of self-acceptance regarding their condition. CONCLUSION: Incomplete eyelid closure poses significant social challenges for individuals with MoS, especially around social encounters. Our findings show the importance of developing tailored communication tools to support those living with this facial difference.

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Despite tracheotomy being a routine procedure, it is not rare to encounter anatomic irregularities that can compromise its success. In this report, we describe a case in which a high riding innominate artery was identified within the surgical trajectory moments before incision, which ultimately necessitated airway securement using an alternative laryngological procedure. Laryngoscope, 2024.

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Unilateral vocal cord paralysis (UVCP) is a growing area of research in pediatrics as it spans across many specialties including otolaryngology, cardiology, general surgery, respirology, and speech language pathology. Iatrogenic injury is the most common cause of UVCP, however there is a wide range of data reporting the prevalence, symptom burden, and best treatment practice for this condition. The literature included systematic reviews and meta-analyses, retrospective studies and limited prospective studies. Overall, the literature lacked consistency in the diagnosis, treatment, and long-term outcomes of patients with UVCP. Many articles conflated bilateral vocal cord paralysis (BVCP) with UVCP and had limited data on the natural history of the condition. There was no consensus on objective and subjective measurements to evaluate the condition or best indications for requiring surgical intervention. Thyroplasty, injection medialization (IM) and recurrent laryngeal nerve reinnervation (RLNR) were the reported surgical interventions used to treat UVCP, however there was limited data on short and long-term surgical outcomes in children. More research is needed to determine the true prevalence, natural history, indications for surgical intervention and long-term outcomes for pediatric patients with this condition.

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BACKGROUND: YouTube has emerged as an important source for obtaining information regarding health issues. OBJECTIVE: The study aimed to assess the reliability and quality of facial paralysis exercise videos that are accessible on the YouTube platform. METHODS: The investigation was carried out on Youtube, utilizing the keyword "facial paralysis exercises". We listed the first 100 videos based on relevancy. The quality and reliability of the videos were assessed using DISCERN, the Journal of the American Medical Association (JAMA) Benchmark Criteria, the Global Quality Scale (GQS), and the Video Power Index (VPI). RESULTS: Out of 100 studies, we excluded 52 and included the remaining 48. The scores we obtained for the videos were as follows: DISCERN Quality (2.92±0.91), DISCERN Total (39.16±6.75), JAMA (2.09±0.55), and GQS (3.00±0.89). Our study also revealed that videos uploaded by healthcare professionals had significantly higher DISCERN total, JAMA and VPI scores compared to those uploaded by non-healthcare professionals (p = 0.018, 0.001 and 0.023, respectively). Additionally, we observed a positive and statistically significant correlation between the DISCERN quality score, total score, JAMA, and video features. CONCLUSION: The facial paralysis exercise videos were determined to be of medium to low quality. Higher-quality videos need to be produced.

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Propionic acidemia (PA) is a rare metabolic disorder stemming from genetic mutations, often causing hyperammonemia, acidosis, and basal ganglia issues. Its symptoms range from vomiting to neurological abnormalities, with severe cases presenting in neonates. Neurological complications including stroke-like episodes are common, requiring immediate attention. An eight-month-old boy with PA presented to the emergency department with respiratory distress, cough, and lethargy. Initial evaluation showed acidemia and elevated ammonia levels. He tested positive for rhinovirus and was diagnosed with acute viral bronchiolitis. While his respiratory symptoms improved, he developed neurological deficits, including hypotonia and weakness. Neurology consultations explored possible diagnoses such as botulism or acute inflammatory demyelinating polyneuropathy (AIDP). Imaging revealed basal ganglia abnormalities consistent with PA progression. Due to aspiration risk, he was transferred to the pediatric intensive care unit for supportive care. Despite unremarkable lumbar puncture and MRI results, new metabolic brain changes were noted, particularly in the basal ganglia. He was managed for weakness and feeding difficulties due to a metabolic stroke. After adjusting nutritional support and discussing long-term feeding options, he was discharged on day 29 with a nasogastric tube due to his inability to meet caloric goals orally. Neurological complications in PA, such as basal ganglia abnormalities and stroke-like episodes, are well-documented. Our case illustrates how an acute respiratory illness can obscure underlying neurological deficits, leading to delayed diagnosis. Symptoms resembling other conditions, such as descending hypotonia in our case, broaden the differential diagnosis to include botulism toxicity and AIDP. This report demonstrates the variety of clinical features patients with PA can present with and the importance of working up a metabolic crisis in addition to conditions with overlapping symptoms.

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Miller Fisher syndrome (MFS) typically presents with acute development of ataxia, ophthalmoplegia, and areflexia. Bilateral vocal cord palsy (BVCP) is a rare manifestation of MFS. We present a case of a 66-year-old male diagnosed with MFS complicated by an unusually delayed onset of BVCP while undergoing inpatient rehabilitation. We also describe the inpatient rehabilitation course, including the use of a patient-guided suspension system (PGSS) as a therapeutic adjunct to aid gait training, resulting in significant functional improvement in ambulation and activities of daily living. Given the rarity of BVCP in MFS, this case highlights the importance of healthcare professionals being aware of this phenomenon so that prompt treatment can be initiated to reduce significant morbidity. Innovative treatment approaches such as the use of a PGSS may also prove beneficial in the rehabilitation of patients with MFS with significant ataxia.

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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.

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PURPOSE OF REVIEW: Patients with various neurological disorders often present with or manifest during their disease process laryngologic pathology that can lead to significant morbidity. Recognizing and treating this aspect of their disease may be crucial in optimizing patient outcome. RECENT FINDINGS: We discuss updated information and management regarding various neurological disorders that affect the larynx and how these sequelae are diagnosed and treated. An understanding of the laryngologic manifestations of neurological disorders will facilitate management of these patient populations. Preventing and minimizing complications arising from these sequelae will improve quality of life and optimize patient outcomes.

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Objective: Proteus syndrome, a rare disorder with an incidence of one in a million, is characterized by connective tissue nevi, asymmetric limb overgrowth, and abnormal subcutaneous adipose tissue distribution. Limited awareness of this condition often hinders accurate clinical diagnosis. We report a case of Proteus syndrome with concurrent progressive paralysis in the unilateral lower limb, aiming to enhance understanding of the disease and its associated complications. Methods: The patient, an 11-year-old male, has been conclusively diagnosed with Proteus Syndrome. This diagnosis was established by analyzing clinical manifestations, imaging studies, and laboratory tests. In addition, a literature review was conducted to systematically elucidate the etiology, diagnosis, treatment, and prognosis of this condition. Results: According to the clinical manifestations, we confirmed a case of Proteus syndrome. This example exhibits the general characteristics of patients with severe hemihypertrophy of the bilateral lower limbs, anomalies in hypodermic and adipose distribution, and unilateral lower limb progressive paralysis. Pathological biopsy confirmed the right chest wall mass as a lipoma. Notably, the patient experiences lower limb movement disorders caused by intraspinal disease. At the same time, the gene sequencing results of this Proteus syndrome patient showed mutations in the IDUS gene and SPECC1L gene, which have not been reported before. Conclusion: We diagnosed Proteus Syndrome with lower limb sensorimotor abnormalities, which may be caused by mutations in the IDUS gene or SPECC1L gene. This is the first report of these kinds of gene mutations in association with Proteus Syndrome.

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Facial paralysis significantly impacts the form and function of patients. Assessment of the face in zones is important to ensure no functional area of the face is neglected. Nasal valve compromise in patients with facial paralysis, for example, is often overlooked yet should be addressed to correct nasal obstruction. In flaccid facial paralysis, inferomedial displacement of the alar base and lateral nasal sidewall insufficiency contribute to nasal valve compromise. For surgical candidates, static suspension of the nasal valve in a superolateral vector is an ideal technique to address the etiology of nasal obstruction in patients with facial paralysis.

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Ramsay Hunt syndrome is a rare viral condition that develops from the varicella zoster virus that affects the face's geniculate ganglion. It has been defined by a herpes zoster oticus, which can be associated with further cranial nerve lesions and acute peripheral facial nerve palsies. In this case, we present a 73-year-old female patient who presented to the outpatient department (OPD) with unbearable pain in the lower left cheek that she had been experiencing for the last four days. The reported pain was continuous and could be described as deep-aching and burning. Facial swelling was observed in relation to the lower lip, especially in the vermillion area; there was ulceration, paralysis seen on the left face, and swelling on the submandibular region. Intravenous corticosteroids and antiviral drugs were administered to her for seven days as an association therapy. In this report, the authors want to stress the necessity of using adequate clinical examination and early intervention in the case of the Ramsay Hunt syndrome.

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BACKGROUND: Intestinal dysfunction plays an important role in the clinical progress and prognosis of severe acute pancreatitis (SAP). Qingyi decoction (QYD) has shown beneficial effects on intestinal function recovery, but the prevention actions of the QYD on intestinal paralysis and its mechanism have not been fully explored. METHODS: The possible molecular mechanism was unraveled by network pharmacology, including active ingredients and potential target prediction, as well as GO, KEGG, and REATCOME pathway enrichment analyses. The potential interactions between the main active ingredients of the QYD and core genes were explored by molecular docking. A retrospective cohort study on 137 patients with SAP from Tianjin Nankai Hospital was conducted to evaluate the preventive effect of QYD on intestinal paralysis. RESULTS: A total of 110 active ingredients in QYD were screened out, and 37 key targets were predicted by network pharmacology. GO, KEGG, and REATCOME enrichment analyses showed that bioinformatics annotation of the hub genes was mainly involved in intestinal epithelial functions and inflammatory response pathways. The main components of QYD possessed good affinity with IL-6, TNF, CASP3, CXCL8, and CRP by molecular docking. Patients who used QYD plus usual care seemed to have fewer intestinal paralysis rates, lower risk of renal insufficiency, ARDS and blood purification therapy, and shorter hospital and ICU stays. The multivariable regression analyses indicated that the mode of nasogastric and enemas administration of QYD (P = 0.010) and timely intervention with QYD (P = 0.045) were the independent protective factors for intestinal paralysis prevention in patients with SAP. CONCLUSION: In conclusion, QYD can be used as an effective adjuvant procedure to prevent the occurrence and development of intestinal paralysis in patients with SAP. The mechanisms may be involved in the anti-inflammatory response and maintenance of intestinal epithelial function.

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INTRODUCTION: Airway management including endotracheal intubation (ETI) is a key skill for emergency clinicians. Therefore, it is important for emergency clinicians to be aware of the current evidence regarding the identification and management of patients requiring ETI. OBJECTIVE: This paper evaluates key evidence-based updates concerning ETI for the emergency clinician. DISCUSSION: ETI is commonly performed in the emergency department (ED) setting but has many nuanced components. There are several tools that have been used to predict a difficult airway which incorporate anatomic and physiologic features. While helpful, these tools should not be used in isolation. Preoxygenation and apneic oxygenation are recommended to reduce the risk of desaturation and patient decompensation, particularly with noninvasive ventilation in critically ill patients. Induction and neuromuscular blocking medications should be tailored to the clinical scenario. Video laryngoscopy is superior to direct laryngoscopy among novice users, while both techniques are reasonable among more experienced clinicians. Recent literature suggests using a bougie during the first attempt. Point-of-care ultrasound is helpful for confirming correct placement and depth of the endotracheal tube. CONCLUSIONS: An understanding of literature updates can improve the ED care of patients requiring emergent intubation.

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Objective To analyze the muscle trophism and expression of interleukin-6 in the biceps brachii muscle of rats with incomplete cervical spinal cord injury treated with neuromuscular electrical stimulation (NMES). Methods Adult rats underwent C5-C7 spinal cord hemisection and a 5-week NMES protocol. Trophism of the biceps brachii was assessed using muscle weight/body weight ratio and histological analysis. Interleukin-6 expression from biceps brachii was measured using the enzyme-linked immunosorbent assay technique. Results Preservation of the biceps brachii muscle trophism was found in the NMES treated group, along with prevention of the reduction of interleukin-6 levels. Conclusion Spinal cord injury causes muscle atrophy and decreases interleukin-6 levels. These alterations are partially prevented by NMES. The results suggest a possible NMES action mechanism and underscore the clinical use of this therapeutic tool.

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The main characteristic of Moebius syndrome is a lack of facial expressions, which involves stigmatization in many social contexts. We examined whether an applicant with this syndrome would be rated lower in personnel selection despite having equal qualifications. In two experiments, participants rated two applicants. Ratings of an applicant with Moebius syndrome were significantly lower when videos of job interviews had been watched without giving information about the syndrome. However, ratings did not differ when still images had been presented accompanied by an audio track or when participants were informed about Moebius syndrome ahead of the video. Discriminatory decisions in personnel selection could be reduced by educating about stigma, here, a neurologically caused lack of facial expressions.

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Hereditary congenital facial palsy (HCFP) is a medical condition caused by dysfunction of the seventh cranial nerve. HCFP is characterized by feeding difficulties and dysmorphic features in the orofacial region. In some cases hearing loss, strabismus, limb malformations, and musculoskeletal defects may be associated. There are three types of HCFP: HCFP3 (OMIM 614744) results from autosomal recessive pathogenic variants in the HOXB1 gene, while HCFP1 and 2 (OMIM 601471, 604185) are autosomal dominant, genetically less defined conditions. We report on a case of congenital bilateral facial palsy due to two novel compound heterozygous variants in the HOXB1 gene, found by exome sequencing (ES), in a child with facial nerve axonal neuropathy without evidence of nerve hypoplasia on neuroimaging. The results of this report suggest that in individuals with congenital facial paralysis and preserved ocular motor skills, with or without facial nerve hypoplasia and with confirmed facial nerve axonal neuropathy, HOXB1 variants and therefore a diagnosis of HCFP3 should be primarily considered.

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OBJECTIVES: Injection laryngoplasty (IL) has been widely used as an initial treatment option for unilateral vocal fold paralysis (UVFP). An additional (second) IL is considered a salvage treatment for unsatisfactory outcomes of initial IL resulting from inadequate injection or early resorption of the injection material. This study aims to evaluate the efficacy of additional IL, distinguishing between "salvage" (within 4 months) and "repeated" injections (beyond 4 months), and to analyze prognostic factors for successful outcomes. METHODS: This retrospective study involved patients who received IL at Asan Medical Center from January 2014 to December 2020. Voice parameters were collected after each procedure, and those who conducted the statistical analysis were blinded to the study subjects. Among the 65 patients who underwent additional IL, 51 patients were enrolled in this study. Postinjection grade, roughness, breathiness, asthenia, strain (GRBAS) scales were used to determine satisfactory treatment outcomes. Success of the additional IL was defined as a postinjection grade of dysphonia score of 0 or 1, with a reduction in grade compared with the preinjection grade. RESULTS: The mean age of the patients was 61.6 years. Out of a total of 51 patients, 37 were men participating in the study. The odds ratio represents the likelihood of success in the second IL. Improved voice outcome after the additional IL was maintained in 23 (45%) patients. Compared with the failure group, the success group had a longer injection time interval between the initial and additional injection (9.1 vs. 7.4 months, respectively, p = 0.010). The success group had a higher proportion of patients with injection intervals >6 months (73.9% vs. 42.9%, p = 0.026). Logistic regression analysis revealed an injection interval >6 months had an odds ratio of 0.265 (confidence interval: 0.080-0.874, p = 0.029). CONCLUSIONS: Additional injections would benefit the patients whose voice outcomes are maintained for a longer period (>6 months) after the first injection.

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Background: Poliomyelitis is always a great concern in Pakistan and is a public health emergency. COVID-19 and recent floods have increased the challenge. Aim: This article highlights the situation of polio in Pakistan and also recommends several steps to eradicate the disease as early as possible. Methodology: Selected articles were selected from electronic databases such as PubMed, Google Scholar, and Scopus using keywords such as Wild Poliovirus, Vaccine Derived Polio Infection, Acute Flaccid Paralysis, Vaccine, and Pakistan. Result: Polio infection has two types, wild poliovirus and vaccine-derived polio infection. 2019 and 2020 were a challenging time as cases were increased at that time in Pakistan. Acute flaccid paralysis is the most common complication of this disease. The maximum cases of polio are being reported from Khyber Pakhtunkhwa. Conclusion: A special public health importance should be given in this province. Regular vaccination and strict surveillance are important. It is also important to spread awareness among the people. Early identification and timely diagnosis are very important. Early diagnosis, proper timely treatment, vaccination, awareness, and community-based research will help Pakistan to eradicate this disease as early as possible.