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Objective: To assess whether access to smartphone video capture of infantile spasms at initial presentation is associated with improved time to diagnosis and treatment., Methods: We conducted a collaborative retrospective cohort study of 80 consecutive infants with confirmed infantile epileptic spasms syndrome initially presenting from 2015 to 2021 at 2 US pediatric centers. Statistical methods used included Mann-Whitney U test to assess the difference in lead times to electroencephalogram (EEG), diagnosis, and treatment between groups with and without video capture. A χ 2 analysis was used to assess differences in demographics, clinical characteristics, and treatment outcomes between groups. Multivariate regression analysis was used to account for etiology types and infantile spasms capture on EEG., Results: Patients with smartphone video infantile spasms capture initially presented a median of 9 days earlier (P = .02), had their first EEG 16 days earlier (P = .007), and were diagnosed and started treatment 17 days earlier (P = .006 and P = .008, respectively) compared with the nonvideo group. The video group had a 25% greater response to initial standard treatment (P = .02) and a 21% greater freedom from infantile spasms at long-term follow-up (P = .03), although this long-term outcome lost statistical significance after adjustment for etiology type (P = .07) and EEG capture of infantile spasms (P = .059)., Conclusion: Our findings suggest a benefit of smartphone video capture of infantile spasms in reduced time to diagnosis and initial standard treatment, which are associated with improved treatment response rates. Substantial differences in lead times and treatment response highlight the clinical importance of pediatricians recommending caregivers to obtain smartphone video of events concerning for infantile spasms., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Injection of botulinum toxin (Botox) into the laryngeal muscles has become the treatment of choice for controlling the symptoms of spasmodic dysphonia (SD). Currently, no specific battery of objective tests to assess the outcome is universally accepted. The purpose of this study was to investigate demographic, clinical, and treatment factors with voice outcome following Botox injection. Sixty-eight patients with adductor SD who underwent at least one Botox injection during a 5-year period were studied. Voice outcome measures were made from patient self-reporting scales and included overall vocal quality, length of response, and duration of breathiness. Vocal quality was significantly correlated with the underlying severity of vocal symptoms prior to treatment, incidence of breathiness and unilateral versus bilateral injection. The length of response was greater in males and following bilateral injections. An increased period of breathiness significantly correlated with bilateral injections.

We analyzed frequency and duration parameters of voice and speech in two men with adductor spasmodic dysphonia (SD). One was treated with botulinum toxin injection; the other received acupuncture therapy. Improvement after acupuncture therapy in terms of standard deviation of fundamental frequency, acoustic perturbation measurements, durational measurements of voice and speech, and spectrographic analysis was comparable to the results achieved with botulinum toxin injection. Voice and speech parameters were stable 1 year after acupuncture therapy.

Description and quantification of the symptoms of adductor spasmodic dysphonia often reflect the clinician's knowledge of the disorder, ideas about the cause of the disorder, and personal experience. No reliable instrument that identifies and quantifies the spectrum of perceptual symptoms has been available. Therefore, we developed a standardized measure called the Unified Spasmodic Dysphonia Rating Scale (USDRS) in cooperation with a team of 118 experienced voice judges. Consensual validations of content validity guided the incremental development of the scale. Using the USDRS allows more consistent and complete data collection, both clinically and in research clinical trials.

Heterogeneity in the quality and task sensitivity of vocal symptoms in the spasmodic dysphonia (SD) population contributes to controversy as to whether this is a single disorder or two disorders with different etiologies (neurogenic versus psychogenic). Perceptual and acoustic assessments of vocal symptoms are inadequate to resolve this controversy. However, myoelectric events are intimately proximal to the source of vocal disruption and may be informative. The present report employs statistical modeling of quantitative amplitude measures of electromyographic activity recorded from thyroarytenoid to examine neuromotor bases of vocal symptoms in SD. Consideration of perceptual ratings of the quality and task sensitivity of vocal symptoms in the context of statistical models provides support for the conclusion that the range of vocal symptoms identified as SD represents a single, neurogenic disorder.

A 45-year-old Caucasian female patient with a clinical rehistory and ECG's conforming to the syndrome of variant angina as characterized by Prinzmetal is presented. ECG's recorded during spontaneous pain demonstrated ST-segment elevation and symmetrical peaking of the T-waves in the lateral precordial leads and short runs of ventricular tachycardia. Similar ECG changes were recorded during treadmill exercise- and hand-grip exercise-induced chest pain. An echocardiogram recorded during angina induced by hand-grip exercise demonstrated progressive flattening of septal motion. Multiple views of the coronary system by selective coronary cineangiography were normal with the patient at rest. Angina was then induced by hand-grip exercise and a repeat right anterior oblique view of the left coronary system revealed marked spasm of the left anterior descending artery proximal to the first septal perforator.

Catheter-induced coronary artery spasm has been observed frequently. It is usually transient, reacts to the administration of nitroglycerin, and its distribution is generally confined to an area in proximity ot the intubated catheter. A 43-year-old woman with recurrent chest pain was found to have a rather long segment of tight proximal obstruction of the right coronary artery and experienced a myocardial infarction during coronary catheterization. Because of recurrent attacks of severe chest pain, coronary artery bypass surgery was performed which failed to result in significant improvement of her symptoms. Two repeat coronary cineangiograms seven weeks and three years after surgery revealed the proximal right coronary artery to be free of stenotic lesions or of luminal irregularities. After considering possible mechanisms of myocardial necrosis in the presence of normal coronary arteries it is concluded that myocardial necrosis can result from catheter-induced coronary artery spasm in spite of administration of nitroglycerin.

The nonrandom association of congenital ichthyosis with neurologic impairment, ectodermal dysplasia, dwarfism, hypogonadism, and renal disease has prompted the review of numerous syndromes. The difficulties in characterization of syndromes in the absence of pathognomonic signs is discussed in relation to three siblings presented herein. Despite extensive investigation, underlying metabolic defects remain obscure.

There has been increasing interest in coronary artery spasm as etiopathogenic mechanism for various syndromes associated with myocardial ischemia. A case with documented organic coronary artery disease is presented, in which coronary artery spasm was the probable cause of intraoperative and early postoperative cardiac arrest. We recommend that coronary spasm be considered in the differential diagnosis of perioperative cardiac arrest.