Takotsubo Cardiomyopathy in a Young Patient Presenting as Cardiac Arrest and Cardiogenic Shock.

Takotsubo cardiomyopathy (TC) is a reversible cardiac disorder that rarely results in serious morbidity and mortality. Cardiogenic shock and cardiac arrest can occur in patients with TC. In this case report, we present the case of a 31-year-old woman with no significant comorbidities who suffered cardiac arrest due to TC and subsequent cardiogenic shock that required inotropic and vasopressor support. The patient's condition progressively improved, and her cardiac function recovered within a few weeks. This case illustrates the importance of recognizing TC as a significant cause of otherwise unexplained cardiac arrest and highlights the need for evidence-based guidelines for treating cardiogenic shock in this setting.

Systolic function in dogs with pulmonic stenosis of different severities and the effect of balloon valvuloplasty.

INTRODUCTION/OBJECTIVES: Pulmonic stenosis (PS) is one of the most common congenital heart diseases in dogs leading to right ventricular (RV) pressure overload, myocardial remodeling, and potential RV dysfunction. Our objectives were to investigate the extent of RV systolic dysfunction in canine PS and to examine the immediate influence of balloon valvuloplasty (BV) on systolic function. ANIMALS, MATERIALS AND METHODS: This prospective study evaluated 72 dogs with PS and 86 healthy dogs. Echocardiographic parameters of systolic function included normalized tricuspid annular plane systolic excursion (N-TAPSE), normalized systolic myocardial tissue Doppler velocity of the lateral tricuspid annulus (N-RVFW-S'), fractional area change, and speckle-tracking longitudinal endocardial RV strain. Forty-four dogs underwent BV and were re-examined after surgery. RESULTS: Systolic function at the basal segment of the RV was significantly lower in the PS group when compared to healthy dogs (mean N-TAPSE 4.29 ± standard deviation 1.18 mm/kg0.285 vs. 5.60 ± 1.29 mm/kg0.285; median N-RVFW-S' 5.28 [lower-upper 25% quantile 4.35-6.43 cm/s/kg0.186] vs. 7.82 [6.73-8.79 cm/s/kg0.186]; all P<0.001). Global longitudinal RV endocardial strain showed no significant difference between the two groups (-28.50 ± 6.23% vs. 28.61 ± 4.64%; P=0.886), but segmental strain analyses revealed basal hypo- and potential compensatory hyperkinesis of the apical RVFW. Furthermore, BV affected most parameters of systolic function, but not the segmental strain values and N-TAPSE. CONCLUSIONS: Right ventricular basal longitudinal systolic function is decreased in dogs with PS in comparison to a healthy cohort. Regional and global function does not necessarily coincide.

[Encephalopathy GNAO1]. / Entsefalopatiya GNAO1.

OBJECTIVE: To study the clinical picture of all patients with GNAO1 encephalopathy detected in the Russian Federation. This publication is a multicenter study combining data from epileptological centers in Moscow, Novosibirsk, St. Petersburg, Nizhny Novgorod, Tyumen. MATERIAL AND METHODS: Nine patients were included, aged 2 to 19 years, with 4 mutations. Male to female sex ratio = 5:4. RESULTS: 8 patients (5 with mutation c.607G>A (p.Gly203Arg), 1 - c.155A>G (Gln52Arg), 1 - c.485G>A (p.Arg162Gln)) had a variant of epileptic encephalopathy, developmental encephalopathy, 1 patient had torsion dystonia without epilepsy (mutation c.713A>G (p.Asp238Gly)). Epileptic seizures in 8 children with epileptic encephalopathy GNAO1 in 100% debuted at 1 month of life, becoming the earliest symptom of the disease. Motor development delayed in 100% of cases. Mental development was not affected only in the case of the dystonic variant. Hyperkinesis (dystonia, choreoathetosis, ballism) followed later, from 2 to 8 months. They were more severe than epilepsy. 4 patients with the c.607G>A (p.Gly203Arg) mutation developed repeated dystonic storms that were resistant to most drugs. CONCLUSION: Epilepsy in GNAO1 is difficult to treat, but temporary or complete remission is possible. Effective drug strategies for the treatment of hyperkinesis have not yet been developed. Expansion of indications for surgical therapy (DBS) of hyperkinesis in this syndrome is desirable.

The Impact of Revascularization in a Patient with Atypical Manifestations of Hypoperfusion.

Background and Objectives: Carotid revascularization is one of the most effective treatment options in patients with severe carotid artery stenosis causing hypoperfusion in basal ganglia. Atypical manifestations include hyperkinetic movements, noted as extremely rare. We report a case about a patient with 2-months-long complaints of Uncontrollable movements in his right side of the body subsided after carotid revascularization. Case presentation: A 71-year-old male was admitted to Pauls Stradins Clinical University Hospital with the main complaints of 2-months-long uncontrollable movements in his right hand and his right leg. When performing coordination tasks, slight inaccuracy was noted with the right-side extremities. Hyperkinetic movements-choreoathetosis in the right side of the patient's face, arm, and leg-were seen. Computed tomography angiography revealed subocclusion in the proximal segment of the left internal carotid artery and 30% stenosis in the proximal segment of the right internal carotid artery. The patient was consulted by a vascular surgeon. Eversion endarterectomy of the left internal carotid artery was performed. The early postoperative period occurred without complications. The patient was discharged from the hospital 2 days after the surgery in good overall health condition. Two months later, choreoathetotic movements in his right side of the body had markedly decreased. No focal neurologic deficits were noted. Conclusions: Revascularization may be effective by eliminating emboli and stenosis, leading to hypoperfusion in watershed territories. A case of a 71-year-old male patient with the main complaints of 2-months-long uncontrollable movements in his right side of the body subsiding after carotid revascularization was demonstrated. It is vital to recognize atypical manifestations of hypoperfusion, associated with stenosis in internal carotid arteries, to early make a diagnosis, to perform an appropriate treatment, and to reduce the risk of cerebral infarction in the future, resulting in a longer high-quality life for the patient.

Facial synkinesis: A distressing sequela of facial palsy.

INTRODUCTION: Synkinesis refers to abnormal involuntary facial movements that accompany volitional facial movements. Despite a 55% incidence of synkinesis reported in patients with enduring facial paralysis, there is still a lack of complete understanding of this debilitating condition, leading to functional limitations and decreased quality of life.1 This article reviews the diagnostic assessment, etiology, pathophysiology, rehabilitation, and nonsurgical and surgical treatments for facial synkinesis. METHODS: A PubMed and Cochrane search was done with no date restrictions for English-language literature on facial synkinesis. The search terms used were "facial," "synkinesis," "palsy," and various combinations of the terms. RESULTS: The resultant inability to control the full extent of one's facial movements has functional and psychosocial consequences and may result in social withdrawal with a significant decrease in quality of life. An understanding of facial mimetic musculature is imperative in guiding appropriate intervention. While chemodenervation with botulinum toxin and neurorehabilitation have continued to be the primary treatment strategy for facial synkinesis, novel techniques such as selective myectomy, selective neurolysis, free-functioning muscle transfer, and nerve grafting techniques are becoming increasingly utilized in treatment regimens. Facial rehabilitation, including neuromuscular retraining, soft tissue massage, and relaxation therapy in addition to chemodenervation with botulinum toxin, remains the cornerstone of treatment. In cases of severe, intractable synkinesis and non-flaccid facial paralysis, surgical interventions, including selective neurectomy, selective myectomy, nerve grafting, or free muscle transfer, may play a more significant role in alleviating symptoms. DISCUSSION: A multidisciplinary approach involving therapists, clinicians, and surgeons is necessary to develop a comprehensive treatment regimen that will result in optimal outcomes. Ultimately, therapy should be tailored to the severity and pattern of synkinesis, and each patient approached on a case-by-case basis. A multidisciplinary approach involving therapists, clinicians, and surgeons is necessary to develop a comprehensive treatment regimen that will result in optimal outcomes.

Biliary hyperkinesia in adolescents-it isn't all hype!

BACKGROUND: Biliary dyskinesia generally refers to a hypofunctioning gallbladder with an ejection fraction (EF) of <35% on hepatobiliary iminodiacetic acid scan with cholecystokinin stimulation (CCK-HIDA testing). In adults, biliary hyperkinesia has a defined association with biliary colic symptoms and can be relieved with surgical intervention. This clinical entity has not been well described in children or adolescents. In fact, only recently have we seen biliary hyperkinesia on HIDA at our centers. To that end, we reviewed our recent experience with adolescents who have presented and been treated for this unusual clinical entity. METHODS: With IRB approval, we retrospectively reviewed the records of all patients with abnormally high HIDA EFs (>80%) cared for by the pediatric surgery services at two tertiary care centers over the span of a three-year period. Age, sex, BMI, CCK-HIDA results, and preoperative testing and post-operative pathology were noted. Resolution of symptoms was determined by subjective patient self-reporting at postoperative visit. RESULTS: Eighteen patients met inclusion criteria. Average age 15.7 (range, 10-17 years), median BMI 27.3 (±8.2). Fifteen patients were female and 3 were male. Average CCK-HIDA EF was 91.6% (±5.2), 82.4% of the patients had evidence of chronic cholecystitis and/or cholesterolosis on pathology. Postoperatively, 82.4% of the patients available for follow up (n=17) reported complete or near complete resolution of symptoms. CONCLUSIONS: Biliary hyperkinesia is an emerging clinical entity in children and adolescents and has a similar presentation to biliary hypokinesia. While the pathophysiologic mechanism of pain is not fully elucidated, laparoscopic cholecystectomy appears to provide a surgical cure for these patients and should be considered in the differential for the patient with an unremarkable workup and history suggestive of biliary colic.

Targeted chemodenervation of the posterior belly of the digastric muscle for the management of jaw discomfort in facial synkinesis.

BACKGROUND: Botulinum toxin (BT-A) chemodenervation has been proved to significantly improve the physical and psychological well-being of patients suffering from facial synkinesis. Despite this, a cohort of patients has persistent tightness and discomfort around the angle of the jaw, which may be caused by synkinesis within the posterior belly of digastric (PBD) muscle. This study was designed to evaluate the benefits of ultrasound-guided BT-A injections into the PBD. METHODS: Thirty-three patients with recalcitrant tightness and discomfort around the angle of the jaw, despite maximal facial therapy and platysmal chemodenervation were selected for inclusion. Patients underwent ultrasound-guided BT-A injection into the ipsilateral PBD muscle (skin puncture site 1 cm inferior and posterior to the angle of mandible). Outcomes consisted of the Facial Disability Index (FDI), Synkinesis Assessment Questionnaire (SAQ), and a visual analogue scale (VAS) designed to assess tightness and pain around the PBD when moving the jaw, swallowing, and masticating. Questionnaires were completed two weeks before and postinjection. Statistical analysis was performed using a paired t-test. RESULTS: Nineteen patients completed the post-treatment outcome questionnaire. A statistically significant improvement was noted in the physical and social function aspects of the FDI and all aspects of the patient-reported VAS scores apart from tightness and pain on jaw retrusion and swallowing. There was no significant difference in the SAQ. CONCLUSION: This study has demonstrated the patient-perceived benefit of ultrasound-targeted BT-A chemodenervation of PBD. This represents a low-risk treatment option that can be easily added to the repertoire of treatments offered to patients with post paralysis facial synkinesis.

Dynamic left ventricular outflow tract obstruction in a patient with acute coronary syndrome and without the apical akinesia: Potential alternative mechanisms causing a dynamic left ventricular outflow tract obstruction other than a compensatory basal hyperkinesis.

The mechanism for dynamic left ventricular outflow tract obstruction (LVOTO) after acute coronary syndromes (ACS) is thought to be apical infarction with compensatory hyperkinesia of the residual normally perfused basal segments of the myocardium. However, herein, we report a patient with ACS and dynamic LVOTO (peak gradient of 250 mm Hg at rest) that could not be secondary to apical akinesia. We propose a potential alternative mechanism leading to dynamic LVOTO in ACS, namely, the interplay between sigmoid septum, basal hyperkinesis, and outflow tract narrowing induced by afterload reduction due to acute myocardial ischemia itself.

Video electroencephalogram combined with electromyography in the diagnosis of hyperkinetic movement disorders with an unknown cause.

OBJECTIVES: Diagnosis of hyperkinetic movement disorders with an unknown cause is usually challenging. The objective of this study is to learn about video electroencephalogram (VEEG) combined with electromyography (EMG) in the diagnosis of hyperkinetic movement disorders with an unknown cause. METHODS: We performed an observational cohort study by recruiting consecutive patients with hyperkinetic movements as the main manifestation with an unknown cause for VEEG combined with EMG evaluations. RESULTS: A total of 77 consecutive patients were enrolled for VEEG-EMG examination. Among them, 57 patients changed their diagnosis after VEEG-EMG assessment, with a mean final diagnosis age of 35.4 ± 20.3 years (range, 4-74 years). The mean duration between initial and final diagnosis was 54.8 ± 71.3 months (range 0.5-300 months). The most common misdiagnosed hyperkinetic movement disorders were myoclonus (40.4%), followed by tremors (24.6%), dystonia (15.8%), psychogenic movement disorders (10.5%), and periodic leg movement syndrome (PLMS) (7.0%). Outcomes of therapy were significantly improved after VEEG-EMG examination (p = 0.000). CONCLUSIONS: Simultaneous VEEG and EMG are important in the diagnosis of hyperkinetic movement disorders with an unknown cause.

The role of pruriceptors in enhancing sensitivity to pruritogens in a murine chronic compression model of dorsal root ganglion.

Chronic pruritus is a symptom that commonly observed in neurological diseases. It has been hypothesized that the chronic pruritus may result from sensitization of itch-signaling pathways but the mechanisms remain obscure. In this study, we established a mouse model of chronic compression of dorsal root ganglion (CCD) and injected various pruritogenic and algogenic agents intradermally to the calf skin ipsilateral to the compressed dorsal root ganglion (DRG). Compared to the naïve mice, a significant increase in itch-related behaviors was observed in the CCD mice after the injection of pruritogens including histamine and BAM8-22, but not after the injection of capsaicin, although all the above agents evoked enhanced pain-related behaviors toward the injected site. In addition, we investigated if pruritogen-evoked activities of DRG neurons were enhanced in this model. In vivo calcium imaging revealed that compressed DRG neurons exhibited enhanced responses to histamine and BAM8-22. Immunoflorescent staining also showed that the histamine receptor H1 and the capsaicin receptor TRPV1 were significantly upregulated in DRG neurons. Our findings indicated that the sensitization of primary pruriceptive neurons may underlie the enhanced itch sensation after chronic compression of DRG in the mice, and may play a role in chronic pruritus in neurological diseases.

The Spectrum of Movement Disorders in Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis Both in Children and Adults: An Experience From a Single Tertiary Care Center.

Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is a form of autoimmune encephalitis. The characteristic clinical features include seizure, psychosis-like symptoms, abnormal movements, and autonomic disturbances. Patients with anti-NMDAR encephalitis can present with various types of movement disorders. Typically, the movement disorders start following intervals of psychiatric and prodromal manifestations in young adults; however, in children, these might be an early presentation of anti-NMDAR encephalitis. The disease is under-recognized and underdiagnosed in Pakistan. Early recognition of the disease is important to commence timely treatment leading to a better prognosis. Here we present a collection of anti-NMDAR encephalitis patients, specifically focussing on the different types of movement disorders and the differences in clinical manifestations between children and adults.

[Electromyography in childhood patients with tic hyperkinesis and Tourette's syndrome]. / Elektromiografiya u bol'nykh detskogo vozrasta s tikoznymi giperkinezami i sindromom Turetta.

OBJECTIVE: To determine the electromyographic patterns of motor tics in children. MATERIAL AND METHODS: 130 patients were examined, which were divided into 3 groups: the 1st group included 21 patients with motor tics; the 2nd group included 87 patients with motor-vocal tics; the 3rd group included 22 patients with Tourette's syndrome (ST). The control group consisted of 35 healthy children. Clinical methods for assessing the severity and semiotics of tics were used, including the YGTSS scale, hyperkinesis counting in 20 minutes, cutaneous electromyography (EMG) in rest and exercise modes with turn-amplitude analysis. RESULTS: The following EMG patterns of tic hyperkinesis in the resting mode were found: 1) the amplitude of the background activity exceeds the values in the control group (p<0.05); 2) in case of clonic tics, a high-amplitude volley activity (VA) arises simultaneously in the form of spindles with a duration of 10-30 ms with an amplitude of 500-2500 µV; 3) with dystonic tics, volleys in the form of rhombuses with a duration of 100-1000 ms and an amplitude of 1000-3000 µV; 4) volley-slow wave had a duration of 307.2±109.6 ms and an amplitude of 540.3±238.6 µV. In the mode of physical activity, VA increases, which is maximally expressed in amplitude on the circular muscle of the eye and the superficial flexor of the fingers in patients with CT (p<0.05). Analysis of correlations established an inverse relationship between the amplitude of VA and the number of hyperkinesis in 20 minutes. CONCLUSION: Qualitative and amplitude-time analysis of EMG makes it possible to objectify the disease, to isolate patients with CT from chronic motor-vocal tics and to model the prognosis. Probably, the tic can act as a compensatory manifestation of excessive impulsation of cortical-subcortical genesis.

A Case of Neuromuscular Electrical Stimulation for Childhood Stroke Hyperkinesis: A Brief Report.

AIM: Some conditions within specific populations are so rare rigorous evidence is unavailable. Childhood hyperkinesis is one example, yet presents an opportunity to examine sensation's contribution to motor function. METHODS: The patient experienced functional difficulty from hyperkinesis as a result of childhood stroke. Home-based passive neuromuscular electrical stimulation (NMES) was implemented an hour/day, six days/week, over 6 weeks (36 hours). Clinical and robotic measures (Assisting Hand Assessment, Box and Block Test, Jebsen Taylor Test of Hand Function, Kinarm) were administered before and after the intervention and at 9 months. RESULTS: NMES was feasible and well tolerated. Clinically important gains of arm function were maintained at 9 months. Robotic measures showed improved hyperkinesis, namely reduced movement segmentation and improved target approximation, in addition to improved proprioceptive function after NMES. CONCLUSION: This case study illustrates the use of NMES within a previously unexplored population and highlights the potential importance of sensory systems to motor gains.

Cardiac Mechanoenergetics in Patients with Acute Myocardial Infarction: From Pressure-Volume Loop Diagram Related to Cardiac Oxygen Consumption.

Acute myocardial infarction (AMI) results in significant changes in cardiac structure and functions, leading to left ventricular remodeling and subsequent systolic and diastolic dysfunction. To improve current approaches in diagnoses, treatments, and prevention of cardiovascular diseases, a better understanding of cardiac mechanoenergetics, including systolic performance and energy demand, becomes paramount. In this review, we summarize cardiac mechanics, cardiac energetics, and their relationship in complications related to AMI using 2 important physiologic frameworks, pressure-volume loops and the Vo2-pressure-volume area relationship diagram, as they are powerful tools for understanding physiologic behavior and mechanoenergetics of the left ventricle.

A Case of Hyperekplexia That Started From Childhood: Clinical Diagnosis With Negative Genetic Investigations.

Here, we report the case of a 63-year-old woman affected by abnormal, excessive, and involuntary reactions to harmless and unexpected sensory stimuli, compatible with the diagnosis of hyperekplexia. It is a pathology that involves the glycinergic system on a hereditary basis, and even if genetic proof compatible with the diagnosis is not present in this case, the fact that an aunt on her father's side suffered from the same disorders supports the clinical suspicion. From an early age, clinical history shows anomalous motor manifestations, initially framed as a form of focal epilepsy or ordinary disorders of the mood sphere, later excluded by the lack of effectiveness of a targeted therapy. Despite this, intellectual, psychological, and socio-emotional development was regular. The manifestations, present throughout childhood, adolescence, and early adulthood in moderate entity, worsened after the age of 50, perhaps due to hormonal changes. The presence of consequent anxiety and depression has compromised her quality of life, and in order to improve it, therapies were resorted, which, however, produced cognitive-attention deficits. No diagnostic exam has confirmed the diagnosis, although some scars in some brain areas involved in the control of reactions are elements favorable to this condition in genetically predisposed subjects. Therapies currently in use attenuate the motor symptomatology without resolving it and cause side effects in the psychological and cognitive sphere. In this case, we want to highlight the difficulty of diagnosing a very rare genetic condition, still not well-known, which presents symptoms easily mistaken for other more common diseases, because there are no specific clinical-diagnostic tools for the time being. In this particular case, we describe a female patient with an atypical onset age and negative genetic investigations compared with what is known in literature regarding this rare disorder. That is why it has been thought she was affected by epilepsy or anxiety-related disorders for several years.

Disautonomías neurovegetativas: un caso clínico interesante / Neurovegetative dysautonomies: an interesting clinical case

RESUMEN Fundamento: la hiperactividad simpática paroxística consiste en episodios autolimitados de hipertensión arterial, taquicardia, taquipnea, hiperhidrosis, disminución del nivel de conciencia, aumento del tono muscular con postura en extensión, hipertermia, sialorrea y midriasis. Con frecuencia se retrasa su reconocimiento lo que incrementa la morbilidad y mortalidad. Objetivo: conocer la importancia de un diagnóstico y tratamiento precoz de la enfermedad para mayor supervivencia del paciente afectado. Presentación del caso: paciente de 33 años de edad, femenina, que desarrolló una hiperactividad simpática paroxística asociada con hidrocefalia obstructiva. Conclusiones: debe sospecharse la enfermedad en pacientes con daño cerebral agudo de diversas causas. El diagnóstico temprano es vital para evitar estudios diagnósticos e intervenciones innecesarias e iniciar un tratamiento rápido y apropiado que modifique la evolución del síndrome.

ABSTRACT Background: paroxysmal sympathetic hyperactivity consists of self-limited episodes of arterial hypertension, tachycardia, tachypnea, hyperhidrosis, decreased level of consciousness, increased muscle tone with extension posture, hyperthermia, sialorrhea and mydriasis. Frequently their recognition is delayed, which increases morbidity and mortality. Objective: to make known the importance of an early diagnosis and treatment of the entity for greater survival of the affected patient. Case report: a 33-years-old female patient who developed a paroxysmal sympathetic hyperactivity associated with obstructive hydrocephalus. Conclusions: the entity should be suspected in patients with acute brain damage of various etiologies. Early diagnosis is vital to avoid unnecessary diagnostic studies and interventions and to initiate a rapid and appropriate treatment that modifies the evolution of the syndrome.

Effect of ixeris sonchifolia pretreatment on expression of hypoxia-inducible factor-1α following myocardial injury induced by exhausting exercise in rats / 中华麻醉学杂志

Objective@#To evaluate the effect of ixeris sonchifolia pretreatment on the expression of hypoxia-inducible factor-1α (HIF-1α) following myocardial injury induced by exhausting exercise in rats.@*Methods@#Fifty-six healthy clean-grade male Wistar rats, weighing 200-220 g, were divided into 3 groups using a random number table method: control group (group C, n=8), exhausting exercise group (group E, n=24) and ixeris sonchifolia pretreatment group (group IS, n=24). In E and IS groups, the model of myocardial injury was established by exhausting swimming.In group IS, the rats were subjected to exhausting swimming after intraperitoneal ixeris sonehifolia 20 ml/kg.In E and IS groups, blood samples were taken from the inferior vena cava at 0, 6 and 24 h after exhaustion (T1-3) for determination of serum cardiac troponin I (cTnI) concentrations by enzyme-linked immunosorbent assay.The animals were sacrificed after anesthesia, and myocardial specimens were obtained for determination of the cell apoptosis index (by TUNEL) and expression of HIF-1α, Bax and Bcl-2 (by immunohistochemistry), and Bax/Bcl-2 ratio was calculated.The area of myocardial injury was observed using HBFP assay.@*Results@#Compared with group C, the area of myocardial injury, concentration of serum cTnl, Bax/Bcl-2 ratio and apoptosis index were significantly increased, and the expression of HIF-1α in myocardial tissues was up-regulated at each time point in E and IS groups (P<0.05). Compared with group E, the area of myocardial injury, concentration of serum cTnl, Bax/Bcl-2 ratio and apoptosis index were significantly decreased, and the expression of HIF-1α in myocardial tissues was down-regulated at each time point in group IS(P<0.05).@*Conclusion@#The mechanism by which ixeris sonchifolia pretreatment mitigates myocardial injury induced by exhausting exercise is related to inhibiting up-regulated expression of HIF-1α in myocardial tissues and reducing cell apoptosis in rats.