Subacute Horizontal Diplopia, Jaw Dystonia, and Laryngospasm.

Jaw dystonia and laryngospasm in the context of subacute brainstem dysfunction have been described in a small number of diseases, including antineuronal nuclear antibody type 2 (ANNA-2, also known as anti-Ri) paraneoplastic neurologic syndrome. Severe episodes of laryngospasms causing cyanosis are potentially fatal. Jaw dystonia can also cause eating difficulty, resulting in severe weight loss and malnutrition. In this report, we highlight the multidisciplinary management of this syndrome associated with ANNA-2/anti-Ri paraneoplastic neurologic syndrome and discuss its pathogenesis.

New phenotype of severe neonatal episodic laryngospasm due to a missense mutation in SCN4A: A case report and literature review. / 由SCN4AåŸºå› é”™ä¹‰çªå˜å¯¼è‡´çš„ä¸¥é‡æ–°ç”Ÿå„¿å‘ä½œæ€§å–‰ç—‰æŒ›1ä¾‹åŠæ–‡çŒ®å¤ä¹ .

Severe neonatal episodic laryngospasm (SNEL) is an ion channel disease characterized by recurrent life-threatening myotonia of respiratory muscle due to mutations in the voltage-gated sodium channel genes. Here we reported a newborn manifested as paroxysmal cyanosis and limb myotonia after birth. The neonate also developed muscle hypertrophy and stunted growth during the follow-up. Whole exome sequencing confirmed c.2395G>A, p.Ala799Thr heterozygous mutation of SCN4A. Carbamazepine was found to be effective on treating the disease. This case expands our understanding of the phenotype resulting from SCN4Amutations. By summarizing the characteristics of reported 16 cases in SNEL,we found they were mainly in the p.G1306E mutation. The common symptoms were upper airway muscle stiffness and feeding difficulties during neonates.When grow up, most patients have different degrees of recurrent attacks of myotonia and progressed muscle hypertrophy. Some of them have athlete-like special faces but all showed myotonic discharge in eletromyogram.

Management of Refractory Laryngospasm.

Laryngospasm has been well described in patients emerging from general anesthesia (GA) and is routinely managed with intermittent positive-pressure mask ventilation, a temporary increase in the depth of anesthesia, or small, titrated amounts of succinylcholine. Patients with severe laryngospasm require reintubation to maintain adequate oxygenation and ventilation. However, reintubation may be only a temporary solution because laryngospasm may recur during re-emergence and re-extubation; thus, anesthesiologists need a comprehensive plan that addresses potential causes of laryngospasm and incorporates continuous positive airway pressure (CPAP) for patients with difficulty emerging from GA. Transnasal humidified rapid-insufflation ventilatory exchange (THRIVE) is a noninvasive ventilation and oxygenation technique used to provide apneic oxygenation, which also generates CPAP. THRIVE uses a high-flow nasal cannula and is more easily tolerated than CPAP with a tight-fitting mask. To our knowledge, we present the first case of refractory laryngospasm during emergence from GA that was successfully managed with THRIVE.

Negative Pressure Pulmonary Edema: A Case Report.

Negative pressure pulmonary edema is an uncommon complication of the extubation of the endotracheal tube. An increase in intrathoracic pressure and negative pressure of the lung caused by acute laryngeal spasm results from acute upper respiratory obstruction causing life-threatening pulmonary edema by alveolar-capillary damage is called negative pressure pulmonary edema. We here describe 28-years old female case the preoperative diagnosis of pelvic inflammatory disease undergoing exploratory laporoscopy caused negative pressure pulmonary edema while extubation. With the immediate treatment, the patient was discharged without any abnormalities.

Paroxysmal Laryngospasm: A Rare Condition That Respiratory Physicians Must Distinguish from Other Diseases with a Chief Complaint of Dyspnea.

Background: In recent years, we have observed respiratory difficulty manifested as paroxysmal laryngospasm in a few outpatients, most of whom were first encountered in a respiratory clinic. We therefore explored how to identify and address paroxysmal laryngospasm from the perspective of respiratory physicians. Methods: The symptoms, characteristics, auxiliary examination results, treatment, and prognosis of 12 patients with paroxysmal laryngospasm treated in our hospital from June 2017 to October 2019 were analyzed. Results: Five males (42%) and 7 females (58%) were among the 12 Han patients sampled. The average age of the patients was 49.25 ± 13.02 years. The disease course ranged from 14 days to 8 years and was characterized by sudden dyspnea, an inability to inhale and exhale, a sense of asphyxia, and voice loss during an attack. Eight patients with gastroesophageal reflux were cured after antacid treatment. One case of upper respiratory tract infection (URI) was completely relieved after symptomatic treatment. One patient with left vocal cord paralysis experienced complete relief after specialist treatment by an otorhinolaryngologist. Episodes in 1 patient were significantly reduced after lifestyle improvement. One patient experienced spontaneous relief after rejecting treatment. Conclusions: Paroxysmal laryngospasm is a rare laryngeal disease that generally occurs secondary to gastroesophageal reflux disease (GERD), and antireflux therapy is frequently effective for its treatment. A respiratory physician should master and identify the symptoms and differentiate this condition from hysterical stridor, reflux-related laryngospasm, and asthma. Timely referral to otolaryngologists, gastroenterologists, and other specialists for standardized examination and regular treatment should be provided when necessary.

Hemi-laryngopharyngeal Spasm (HeLPS): Defining a New Clinical Entity.

OBJECTIVE: Hemi-laryngopharyngeal spasm (HeLPS) has recently been described in the neurosurgical literature as a cause of intermittent laryngopharyngeal spasm and cough due to vascular compression of the vagus nerve at the cerebellopontine angle. We present the diagnostic criteria for this syndrome. METHODS: A retrospective chart review of six patients with HeLPS and three patients misdiagnosed with this condition are presented. All patients were diagnosed and treated at a tertiary care academic centre from July 2013 to July 2017. RESULTS: Patients with HeLPS had five defining characteristics: 1) All patients had symptoms of episodic laryngopharyngeal spasm and coughing. Patients were asymptomatic between episodes and were refractory to speech therapy and reflux management. 2) Laryngoscopy showed hyperactive twitching of the ipsilateral vocal fold in two of the six patients. No other inter-episodic abnormalities were seen. 3) Botulinum toxin A injections into the thyroarytenoid muscle on the affected ipsilateral side reduced laryngopharyngeal spasms. Botulinum toxin injection in the contralateral thyroarytenoid muscle did not improve laryngopharyngeal spasm. 4) Magnetic resonance imaging revealed ipsilateral neurovascular compression of the vagus nerve rootlets by the posterior inferior cerebellar artery. 5) Microvascular decompression (MVD) surgery of the ipsilateral vagus nerve resolved all symptoms (follow-up 2-4 years). CONCLUSION: The diagnostic criteria for hemi-laryngopharyngeal spasm (HeLPS) are proposed. Otolaryngology recognition of this new clinical entity may lead to a surgical cure and avoid the unnecessary therapies associated with misdiagnosis. LEVEL OF EVIDENCE: 4.

Repeated Postanesthetic Laryngospasm in a Male Adult.

Laryngospasm is a potential complication after general anesthesia that is universally dreaded because the failure to act swiftly and effectively could be fatal for the patient. This case report involves a morbidly obese male patient who received his first general anesthetic and experienced 4 episodes of laryngospasm within an hour after emergence. Laryngospasm occurs when the vocal cords adduct, closing the glottis, thereby preventing gas exchange in a spontaneously ventilating individual. This patient was able to physically indicate his impending glottic closure, thus warning his caregivers of the imminent airway emergency. He was safely intubated during the fourth episode and admitted to the intensive care unit for monitoring. After 2 days, the patient was extubated and recovered otherwise uneventfully. This case demonstrates the high level of vigilance required during all phases of anesthesia care.

Vibratory Onset of Adductor Spasmodic Dysphonia and Muscle Tension Dysphonia: A High-Speed Video Study✰.

OBJECTIVE: Adductor spasmodic dysphonia (AdSD) is a challenging voice disorder to diagnose, often erroneously diagnosed as muscle tension dysphonia (MTD) or vocal tremor, due to its similarity in auditory and perceptual presentation. Assessments using laryngoscopy or strobolaryngoscopy procedures have demonstrated limited utility in the diagnosis of spasmodic dysphonia. High-speed videoendoscopy (HSV) provides visualization of the precise vibratory pattern of phonatory onset and thus, offers an alternative to previous diagnostic strategies for visual diagnosis of AdSD. The purpose of this study was to examine vibratory onset of patients with AdSD and patients with MTD using HSV methodology. METHODS: HSV of six adults with AdSD and five adults with MTD were captured during sustained phonation. Digital kymography was used to obtain precise vibrogram data at the mid-membranous region of the vocal fold prior to and at the onset of phonation. Voice onset delay was examined by (1) quantifying timing of prephonatory delay and steady state delay and (2) describing vocal fold onset movements qualitatively in each diagnosis. RESULTS: HSV adequately captured the phonatory onset of the vocal folds. Voice onset delay was not significantly different between AdSD and MTD. However, there were distinct differences in voice onset gestures. Both AdSD and MTD patients presented with vocal hyperfunction during the onset of phonation. In a subset of AdSD patients, a rapid sustained adduction occurred following the initial vibratory motion. Vocal fold vibration then continued until steady phonation was achieved. This oscillatory break pattern was not observed in patients with MTD. Therefore, there appears to be differences in vocal fold vibratory onset motion between MTD and AdSD. CONCLUSION: HSV captures the precise vibratory onset in patients with MTD and AdSD. Differences were most notable after vibratory onset as opposed to vibratory delay measurements. Examination of voice onset gesture may offer an additional laryngeal parameter to assist in the differential diagnosis of spasmodic dysphonia.

Negative-pressure pulmonary edema after laparoscopic appendectomy.

Negative-pressure pulmonary edema (NPPE) is acute-onset bilateral pulmonary interstitial edema. This condition can be caused by significant negative intrathoracic pressure generated by large inspiratory effort against acute upper airway obstruction. Postoperative NPPE is rare but potentially life-threatening if not recognized and treated promptly. This article describes a patient who developed postoperative NPPE following a laparoscopic appendectomy.

Paroxysmal laryngospasm: Episodic closure of the upper airway.

Paroxysmal laryngospasm is an episodic event, resulting in complete closure of the larynx caused by hypersensitization of laryngeal tissue and protective laryngeal reflexes. This condition most often occurs secondary to laryngopharyngeal reflux. Prognosis generally is good after treatment is initiated; however, often because of a misunderstanding of the clinical course and potential causes, patients with paroxysmal laryngospasm can go untreated. This article describes paroxysmal laryngospasm, similarly presenting diseases, and treatment strategies.

Edema pulmonar por presión negativa con máscara laríngea clásica. Presentación de un caso pediátrico / Pulmonary edema due to negative pressure with classical laryngeal mask. Presentation of a pediatric case

RESUMEN El edema pulmonar por presión negativa es una complicación rara y dramática en la anestesia general. Habitualmente ocurre como consecuencia de un laringoespasmo u otra causa de obstrucción de la vía aérea. Se presentó un caso con el objetivo de mostrar los elementos usados para el diagnóstico y tratamiento del edema pulmonar por presión negativa. Paciente de 7 años de edad, femenina, ASA I, operada de metatarso varo funcional con anestesia general balanceada y máscara laríngea clásica # 3. Desarrolló dicho evento adverso durante la recuperación anestésica. El diagnóstico se basó en la disociación toraco abdominal al restablecer la ventilación espontanea, crepitantes en ambos hemitórax, cianosis central, hipoxemia y la presencia de infiltrado difuso bilateral alveolar. Se intubó la tráquea, se controló la ventilación con presión positiva al final de la espiración y se administró furosemida. La paciente fue trasladada a la Unidad de Cuidados Intensivos donde evolucionó satisfactoriamente. Este es un síndrome cuya verdadera incidencia se desconoce debido a la escasa familiarización con el mismo. La evolución de los pacientes es favorable siempre que se establezca el diagnóstico y el tratamiento oportuno (AU).

ABSTRACT Pulmonary edema due to negative pressure is a rare and dramatic complication in general anesthetic. It usually occurs as a consequence of a laryngeal spasm or another cause of respiratory tract obstruction.A case was presented with the aim of showing the elements used for the diagnosis and treatment of the pulmonary edema due to negative pressure. An ASA I, 7-year-old female patient, was operated on a functional metatarsus varus with balanced general anesthetic and classical laryngeal mask number 3. She developed this adverse event during the anesthetic recovery. The diagnosis was based on the thoracoabdominal dissociation when recovering spontaneous ventilation, crepitation in hemithoraxes, central cyanosis, hypoxemia, and alveolar bilateral diffused infiltrate. The trachea was intubated, ventilation was controlled with positive pressure at the end of the expiration and furosemide was administered. The patient was transferred to the Intensive Care Unit where she evolved satisfactorily. This is a syndrome whose true incidence is unknown as a result of the lack of familiarization with it. Patients' evolution is favorable whenever the right diagnosis and treatment are timely established (AU).

[Laryngospasm in amyotrophic lateral sclerosis : A case report]. / Laryngospasmus bei Amyotropher Lateralsklerose : Ein Fallbericht.

Laryngospasm in amyotrophic lateral sclerosis is a symptom seldom described in neurological literature. Despite the self limiting character it means a life threatening condition for the patient himself. This case report and the following discussion tries to look upon underlying causes and options of therapy.

[Negative pressure pulmonary edema following a cholecystectomy - A case report]. / Œdème pulmonaire à pression négative après une cholécystectomie ­ à propos d'un cas.

Negative pressure pulmonary edema (NPPE) or post-obstructive pulmonary edema (POPE) is a rare but a life threatening respiratory situation. The most frequent cause of NPPE is a post anesthetic laryngospasm. Edema is developed after relief of upper airway obstruction. Its incidence is 0.1 % of general anesthesia with tracheal intubation. We report this clinical case of a 28-year-old woman, who developed dyspnea followed by acute respiratory distress with unilateral pulmonary edema after extubation following general anesthesia for cholecystectomy under celioscopy. After elimination of any other cause, context, clinical and radiological characteristics, and the favorable evolution under oxygen and furosemide made it possible for us to link this acute respiratory distress to a NPPE whose pathophysiological causes and mechanisms are discussed.

Dyspnea in Children: What is driving it and how to approach it.

Dyspnea in children has important physical and psychosocial impact. It is useful to define the quality of the dyspnea and quantify its magnitude in a child-friendly manner. Through careful history taking and physical examination, a targeted investigation can lead to identification of the cause and potential treatment. This article provides a framework for the clinical approach to dyspnea in children, including important information to gather during the history, physical assessment, how to quantify dyspnea, and choice and use of laboratory measurements.

Detection of Respiratory Adverse Events in Pediatric Dental Patients Sedated With 0.75mg/Kg of Midazolam and Oxygen by Continuous Pretracheal Auscultation: A Prospective Randomized Controlled Trial.

PURPOSE: Sedation is becoming more commonplace for pediatric patients undergoing minor procedures. Fortunately, electronic monitors have contributed to a reduction in the associated respiratory adverse events (RAEs). To test the hypothesis that adding the pretracheal stethoscope (PTS) to standard monitoring methods (SMMs) may improve RAE detection in sedated pediatric dental patients, the frequency of RAEs detected by SMMs (i.e. visual observation, capnography, and pulse oximetry) was compared to that detected by SMMs alongside continuous PTS auscultation. STUDY DESIGN: A prospective, randomised, controlled trial was performed with 100 pediatric patient participants of ASA≤2, who were scheduled to receive dental treatment under 0.75 mg/kg and oxygen. Patients were randomised into Groups A (n=50; SMMs) and B (n=50; SMMs+PTS). Inclusion criteria were behavioral management problems and intolerance to dental treatment despite behavioral management techniques or nitrous oxide administration. Exclusion criteria were high-risk conditions for RAEs, altered mental status, gastrointestinal disorders, parental refusal of conscious sedation and failure of previous conscious sedation. An anesthesist was present throughout the dental treatments. RESULTS: RAEs were detected in 10 (20%) and 22(44%) Group A and B patients respectively (p=0.01). The majority of RAEs within Group B were detected by PTS auscultation (n=19). Capnography produced 13 and 15 false-positive results in Groups A and B respectively, whereas the PTS produced 4(8%) false-positive results in Group B (p=0.009). CONCLUSIONS: PTS was found to be useful for detecting RAEs during pediatric dental sedation with 0.75mg/kg midazolam and oxygen, in the presence of an anesthesist.

Episodic hemilaryngopharyngeal spasm (HELPS) syndrome: case report of a surgically treatable novel neuropathy.

The authors describe a novel cranial neuropathy manifesting with life-threatening episodic hemilaryngopharyngeal spasm (HELPS). A 50-year-old woman presented with a 4-year history of intermittent throat contractions, escalating to life-threatening respiratory distress. Botulinum toxin injections into her right vocal cord reduced the severity of her spasms, but the episodes continued to occur. MRI demonstrated a possible neurovascular conflict involving the cranial nerve IX-X complex and the posterior inferior cerebellar artery. Microvascular decompression of the upper rootlets of the vagal nerve eliminated her HELPS without complication. The authors propose a mechanism of HELPS implicating isolated involvement of the upper motor rootlets of the vagus nerve.

Inhaled Mannitol as a Laryngeal and Bronchial Provocation Test.

OBJECTIVES: Timely diagnosis of vocal cord dysfunction (VCD), more recently termed "inducible laryngeal obstruction," is important because VCD is often misdiagnosed as asthma, resulting in delayed diagnosis and inappropriate treatment. Visualization of paradoxical vocal cord movement on laryngoscopy is the gold standard for diagnosis, but is limited by poor test sensitivity. Provocation tests may improve the diagnosis of VCD, but the diagnostic performance of current tests is less than ideal. Alternative provocation tests are required. This pilot study demonstrates the feasibility of using inhaled mannitol for concurrent investigation of laryngeal and bronchial hyperresponsiveness. METHODS: Consecutive patients with suspected VCD seen at our institution's asthma clinic underwent flexible laryngoscopy at baseline and following mannitol challenge. VCD was diagnosed on laryngoscopy based on inspiratory adduction, or >50% expiratory adduction of the vocal cords. Bronchial hyperresponsiveness after mannitol challenge was also assessed. We evaluated the interrater agreement of postmannitol laryngoscopy between respiratory specialists and laryngologists. RESULTS: Fourteen patients with suspected VCD in the context of asthma evaluation were included in the study. Mannitol provocation demonstrated VCD in three of the seven patients with normal baseline laryngoscopy (42.9%). Only two patients had bronchial hyperresponsiveness. There was substantial interrater agreement between respiratory specialists and laryngologists, kappa = 0.696 (95% confidence interval: 0.324-1) (P = 0.006). CONCLUSION: Inhaled mannitol can be used to induce VCD. It is well tolerated and can evaluate laryngeal and bronchial hyperresponsiveness at the same setting.