RESUMEN
Although epilepsy is the most common comorbidity of brain tumors, epileptic spasms rarely occur. Brain tumors associated with epileptic spasms are mostly low-grade gliomas. To date, few studies in the literature have reported on malignant (Grades 3-4) brain tumors associated with epileptic spasms. Thus, we aimed to investigate the characteristics of malignant brain tumor-associated epileptic spasms. We retrospectively reviewed patients with malignant brain tumors and epileptic spasms in our institution. Data on demographics, tumor histology, magnetic resonance imaging, epileptic spasm characteristics, electroencephalography, and treatment responsiveness were also collected. Six patients were included. In all cases, the brain tumors occurred in infancy in the supratentorial region and epileptic spasm onset occurred after the completion of brain tumor treatment. Anti-seizure medication did not control epileptic spasms; two patients were seizure-free after epileptic surgery. Although all patients had developmental delays caused by malignant brain tumors and their treatment, developmental regression proceeded after epileptic spasm onset. Two patients who achieved seizure-free status showed improved developmental outcomes after cessation of epileptic spasms. This is the first report of the characteristics of malignant brain tumor-associated epileptic spasms. Our report highlights a difficulties of seizure control and possibillity of efficacy of epileptic surgery in this condition. In malignant brain tumor-associated epileptic spasms, it is important to proceed with presurgical evaluation from an early stage, bearing in mind that epileptic spasms may become drug-resistant.
Asunto(s)
Neoplasias Encefálicas , Electroencefalografía , Humanos , Masculino , Femenino , Neoplasias Encefálicas/complicaciones , Estudios Retrospectivos , Lactante , Preescolar , Epilepsia/etiología , Epilepsia/fisiopatología , Imagen por Resonancia Magnética , Glioma/complicaciones , Glioma/fisiopatología , Espasmo/etiología , Espasmo/fisiopatología , Anticonvulsivantes/uso terapéutico , NiñoRESUMEN
Paroxysmal neurological symptoms in patients with multiple sclerosis (MS) have long been acknowledged. However, consideration of whether such symptoms are a clinical attack and sufficient for fulfillment of MS diagnostic criteria has varied as criteria have evolved over time. Previous studies and anecdotal reports indicate that some patients with MS first present with syndromes such as trigeminal neuralgia, Lhermitte's phenomenon, tonic spasm, and seizure years before an attack typical of MS such as optic neuritis or myelitis. We discuss four patients with presumed MS who initially presented with these syndromes with evidence of a corresponding central nervous system (CNS) lesion who, were these symptoms considered an attack, could have been diagnosed with relapsing remitting MS or clinically isolated syndrome. This case series aims to highlight the unmet need for data for such patient presentations and for clinical guidance from future MS diagnostic criteria to optimize care.
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Esclerosis Múltiple , Humanos , Femenino , Adulto , Esclerosis Múltiple/diagnóstico , Esclerosis Múltiple/complicaciones , Esclerosis Múltiple/fisiopatología , Persona de Mediana Edad , Masculino , Esclerosis Múltiple Recurrente-Remitente/diagnóstico , Esclerosis Múltiple Recurrente-Remitente/fisiopatología , Esclerosis Múltiple Recurrente-Remitente/complicaciones , Imagen por Resonancia Magnética , Neuralgia del Trigémino/diagnóstico , Neuralgia del Trigémino/etiología , Diagnóstico Diferencial , Convulsiones/etiología , Convulsiones/diagnóstico , Espasmo/diagnóstico , Espasmo/etiología , Espasmo/fisiopatología , Neuritis Óptica/diagnóstico , Neuritis Óptica/fisiopatologíaRESUMEN
OBJECTIVES: To investigate the effect of electroacupuncture (EA) on Rho/Rho-associated coiled-coil-forming kinases (ROCK) signaling pathway of uterus tissue in rats with dysmenorrhea, so as to explore the underlying mechanism of EA treating primary dysmenorrhea (PD) and uterine smooth muscle spasm, and to observe whether there is a difference in the effect of meridian acupoints in Conception Vessel (CV) and Governer Vessel (GV). METHODS: Sixty female SD rats were randomly divided into saline, model, CV, GV, and non-acupoint groups, with 12 rats in each group. The dysmenorrhea model was established by subcutaneous injection of estradiol diphenhydrate combined with intraperitoneal injection of oxytocin (OT). EA (2 Hz) was applied to "Qihai" (CV6) and "Zhongji" (CV3) for CV group, "Mingmen" (GV4) and "Yaoshu" (GV2) for GV group, "non-acupoint 1" and "non-acupoint 3" on the left side for non-acupoint group, and manual acupuncture was applied to "Guanyuan" (CV4) for CV group, "Yaoyangguan" (GV3) for GV group, "non-acupoint 2" on the left side for non-acupoint group. The treatment was conducted for 20 min each time, once daily for 10 days. The writhing score was evaluated. The smooth myoelectric signals of rats' uterus in vivo were recorded by multi-channel physiological recorder. The uterine histopathological changes were observed by HE staining. The contents of prostaglandin F2α (PGF2α), OT and calcium ion (Ca2+) in uterine tissue of rats were detected by ELISA. The protein and mRNA expression levels of smooth muscle 22-α (SM22-α), RhoA and ROCKâ ¡ in uterine tissue were detected by Western blot and fluorescence quantitative PCR, respectively. RESULTS: Compared with the saline group, the writhing score of rats in the model group was increased (P<0.01), the amplitude voltage of uterine smooth muscle in vivo was elevated (P<0.01), the contents of PGF2α, OT and Ca2+, the protein and mRNA expression of SM22-α, RhoA and ROCK â ¡ in uterine tissue were all increased (P<0.01). Compared with the model and the non-acupoint groups, the writhing scores of the CV and the GV groups were decreased (P<0.01, P<0.05), the amplitude voltage of uterine smooth muscle was decreased (P<0.01), the contents of PGF2α, OT and Ca2+ in uterine tissue were decreased (P<0.01, P<0.05), and the protein expression and mRNA expression of SM22-α, RhoA and ROCKâ ¡ in uterine tissue were decreased (P<0.01, P<0.05). HE staining showed extensive exfoliation of uterine intima with severe edema and increased glandular secretion in the model group, which was alleviated in the CV and GV groups. CONCLUSIONS: EA at acupoints of CV and GV can significantly reduce the writhing score, uterine smooth muscle amplitude voltage, pathological injury degree of uterus, and relieve spasm of uterine smooth muscle in dysmenorrhea rats, which may be related to its effect in regulating PGF2α and OT contents, inhibiting the Rho/ROCK signaling pathway, and reducing the SM22-α, RhoA, ROCKâ ¡ protein and mRNA expression, and Ca2+ content in uterine tissue.
Asunto(s)
Puntos de Acupuntura , Dismenorrea , Electroacupuntura , Ratas Sprague-Dawley , Transducción de Señal , Útero , Quinasas Asociadas a rho , Animales , Femenino , Dismenorrea/terapia , Dismenorrea/metabolismo , Dismenorrea/genética , Quinasas Asociadas a rho/metabolismo , Quinasas Asociadas a rho/genética , Ratas , Humanos , Útero/metabolismo , Músculo Liso/metabolismo , Espasmo/terapia , Espasmo/genética , Espasmo/metabolismo , Espasmo/fisiopatologíaAsunto(s)
Enfermedades del Nervio Facial/fisiopatología , Síndrome Hipereosinofílico/fisiopatología , Espasmo/fisiopatología , Enfermedades del Nervio Facial/etiología , Humanos , Síndrome Hipereosinofílico/complicaciones , Masculino , Músculos Masticadores/fisiopatología , Persona de Mediana Edad , Espasmo/etiologíaRESUMEN
We investigated the sensitivity, specificity and safety of ergonovine provocation test of radial artery in the diagnosis of coronary artery spasm (CAS). The patients who came to our hospital for chest pain from January to June 2020 as well as had coronary stenosis < 50% and no radial artery stenosis, were enrolled in this study. These patients were divided into CAS group and control group after intracoronary ergonovine provocation test. All patients underwent ergonovine provocation test of radial artery, the inner diameter (D0 and D1) and the peak systolic velocities (PSV0 and PSV1) of the radial artery were measured by ultrasound before and after ergonovine provocation. The predictive value of ergonovine provocation test of radial artery for the diagnosis of CAS was analyzed using receiver operator characteristic (ROC) curve. There were 19 patients in the CAS group and 28 patients in the control group. Low density lipoprotein cholesterol and smoking rate were significantly higher in the CAS group than in the control group (all P < 0.05), but there were no significant differences in other items (P > 0.05) between the two groups. In the ergonovine provocation test of radial artery, degree of radial artery stenosis was significantly higher in the CAS group [41.50% (35.60%, 50.00%)] than in the control group [11.25% (5.15%, 23.00%)] (P = 0.000), but there were no siginificant differences in D0, PSV0 and PSV1 between the two groups (P > 0.05). The area under ROC curve of ergonovine (120 µg) provocation test of radial artery for the diagnosis of CAS was 0.912 with 95%CI: 0.792-0.975, P = 0.001, cut-off of 31%, specificity of 92.86% and sensitivity of 84.21%. The ergonovine (120 µg) provocation test of radial artery did not cause any adverse reactions. We concluded that the ergonovine provocation test of radial artery has high sensitivity, specificity and safety in the diagnosis of CAS.
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Vasoespasmo Coronario/diagnóstico , Vasos Coronarios/efectos de los fármacos , Ergonovina/farmacología , Área Bajo la Curva , Dolor en el Pecho/fisiopatología , Angiografía Coronaria/métodos , Vasos Coronarios/metabolismo , Electrocardiografía/métodos , Femenino , Humanos , Masculino , Persona de Mediana Edad , Arteria Radial/efectos de los fármacos , Arteria Radial/metabolismo , Sensibilidad y Especificidad , Espasmo/diagnóstico , Espasmo/fisiopatologíaRESUMEN
INTRODUCTION: Peripheral nerve hyperexcitability syndrome (PNHS) is characterized by muscle fasciculations and spasms. Nerve hyperexcitability and after-discharges can be observed in electrophysiological studies. Autoimmune mechanisms play a major role in the pathophysiology of primary PNHS. METHODS: We retrospectively conducted a case-control study recruiting patients with clinical and electrophysiological features of PNHS. Control patients were diagnosed with other neuronal or muscular diseases. Contactin-associated protein2 (CASPR2) and leucine-rich glioma-inactivated1 (LGI1) antibodies were examined. RESULTS: A total of 19 primary PNHS patients and 39 control patients were analyzed. The most common symptoms for the case group were fasciculations (11/19) and muscle spasms (13/19). Case group patients were likely to demonstrate electrodiagnostic findings of nerve hyperexcitability (17/19) and after-discharges in the tibial nerve (19/19). We found high prevalence of CASPR2 (9/19) and LGI1 (6/19) antibodies in the case group. DISCUSSION: Primary PNHS patients were likely to show after-discharges in the tibial nerve. The pathogenesis of PNHS is autoimmune CASPR2 and LGI1 antibodies are possible pathogenic antibodies for primary PNHS.
Asunto(s)
Autoanticuerpos/inmunología , Fasciculación/diagnóstico , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Espasmo/diagnóstico , Adulto , Anciano , Estudios de Casos y Controles , Moléculas de Adhesión Celular Neuronal/inmunología , Electrodiagnóstico , Fasciculación/inmunología , Fasciculación/fisiopatología , Femenino , Humanos , Péptidos y Proteínas de Señalización Intracelular/inmunología , Masculino , Persona de Mediana Edad , Enfermedades del Sistema Nervioso Periférico/inmunología , Enfermedades del Sistema Nervioso Periférico/fisiopatología , Estudios Retrospectivos , Espasmo/inmunología , Espasmo/fisiopatología , Adulto JovenRESUMEN
PURPOSE: Eating epilepsy was previously known as a kind of focal reflex epilepsy. However, the development of eating-induced multiple generalized seizures and the associated EEG changes were rarely reported. Herein, we present a 13-year-old generalized epilepsy patient with eating-induced generalized seizures since the age of 5. CASE PRESENTATION: The 13-year-old male patient had suffered from late-onset eating-induced epileptic spasms during the meal since the age of 5. Meanwhile, he also experienced spontaneous epileptic spasms during the period of sleep. The seizure frequency and type gradually increased from 7 years of age. In addition to epileptic spasms, he started experiencing atypical absence with myoclonic jerks during the meal. Ictal EEG presented as the appearance of an irregular slow-wave mixed with generalized polyspike wave with the intake of food, and gradually evolved to bursts of generalized polyspike wave complexes. At the end of the meal, the EEG returned to normal. Nevertheless, at the age of 13, his seizure frequency increased and appeared new seizure type, and besides epileptic spasm and atypical absence, he began to experience myoclonic seizure during sleep and awaking-generalized tonic-clonic seizure in the morning. In this period he started taking valproic acid, topiramate and clonazepam, and his seizure frequency was reduced. CONCLUSION: In conclusion, this case demonstrated the variability of eating induced multiple generalized seizure types, and eight years follow-up also indicates that generalized epilepsy progressed with age. The EEG and clinical changes of our patient contribute to a better understanding of the electro-clinical features of eating-induced multiple generalized seizures and the course of generalized epilepsy with such seizures.
Asunto(s)
Epilepsia Generalizada/fisiopatología , Convulsiones/etiología , Adolescente , China , Clonazepam/uso terapéutico , Ingestión de Alimentos/fisiología , Epilepsia Generalizada/metabolismo , Estudios de Seguimiento , Humanos , Masculino , Mioclonía/fisiopatología , Sueño/fisiología , Espasmo/fisiopatología , Topiramato/uso terapéutico , Ácido Valproico/uso terapéuticoRESUMEN
OBJECTIVE: Epileptic spasms are a hallmark of severe seizure disorders. The neurophysiological mechanisms and the neuronal circuit(s) that generate these seizures are unresolved and are the focus of studies reported here. METHODS: In the tetrodotoxin model, we used 16-channel microarrays and microwires to record electrophysiological activity in neocortex and thalamus during spasms. Chemogenetic activation was used to examine the role of neocortical pyramidal cells in generating spasms. Comparisons were made to recordings from infantile spasm patients. RESULTS: Current source density and simultaneous multiunit activity analyses indicate that the ictal events of spasms are initiated in infragranular cortical layers. A dramatic pause of neuronal activity was recorded immediately prior to the onset of spasms. This preictal pause is shown to share many features with the down states of slow wave sleep. In addition, the ensuing interictal up states of slow wave rhythms are more intense in epileptic than control animals and occasionally appear sufficient to initiate spasms. Chemogenetic activation of neocortical pyramidal cells supported these observations, as it increased slow oscillations and spasm numbers and clustering. Recordings also revealed a ramp-up in the number of neocortical slow oscillations preceding spasms, which was also observed in infantile spasm patients. INTERPRETATION: Our findings provide evidence that epileptic spasms can arise from the neocortex and reveal a previously unappreciated interplay between brain state physiology and spasm generation. The identification of neocortical up states as a mechanism capable of initiating epileptic spasms will likely provide new targets for interventional therapies. ANN NEUROL 2021;89:226-241.
Asunto(s)
Ondas Encefálicas/fisiología , Neocórtex/fisiopatología , Células Piramidales/fisiología , Espasmos Infantiles/fisiopatología , Tálamo/fisiopatología , Animales , Modelos Animales de Enfermedad , Electrocorticografía , Femenino , Humanos , Lactante , Masculino , Neocórtex/efectos de los fármacos , Células Piramidales/efectos de los fármacos , Ratas , Ratas Wistar , Convulsiones/inducido químicamente , Convulsiones/fisiopatología , Bloqueadores de los Canales de Sodio/toxicidad , Espasmo/inducido químicamente , Espasmo/fisiopatología , Espasmos Infantiles/inducido químicamente , Tetrodotoxina/toxicidad , Tálamo/efectos de los fármacosRESUMEN
BACKGROUND: Peppermint oil is well known to inhibit smooth muscle contractions, and its topical administration during colonoscopy is reported to reduce colonic spasms. AIMS: We aimed to assess whether oral administration of IBGard™, a sustained-release peppermint oil formulation, before colonoscopy reduces spasms and improves adenoma detection rate (ADR).⯠METHODS: We performed a single-center randomized, double-blinded, placebo-controlled trial. Patients undergoing screening or surveillance colonoscopies were randomized to receive IBGard™ or placebo. The endoscopist graded spasms during insertion, inspection, and polypectomy. Bowel preparation, procedure time, and time of drug administration were documented. Statistical analysis was performed using the Student's t test and Wilcoxon rank-sum test. RESULTS: There was no significant difference in baseline characteristics or dose-timing distribution between IBGard™ and placebo groups. Similarly, there was no difference in ADR (IBGard™ = 47.8%, placebo = 43.1%, p = 0.51), intubation spasm score (1.23 vs 1.2, p = 0.9), withdrawal spasm score (1.3 vs 1.23, p = 0.72), or polypectomy spasm score (0.52 vs 0.46, p = 0.69). Limiting the analysis to patients who received the drug more than 60 min prior to the start of the procedure did not produce any significant differences in these endpoints. CONCLUSIONS: This randomized controlled trial failed to show benefit of orally administered IBGard™ prior to colonoscopy on the presence of colonic spasms or ADR. Because of its low barrier to widespread adoption, the use of appropriately formulated and timed oral peppermint oil warrants further study to determine its efficacy in reducing colonic spasms and improving colonoscopy quality.
Asunto(s)
Pólipos Adenomatosos/patología , Neoplasias del Colon/patología , Pólipos del Colon/patología , Colonoscopía , Parasimpatolíticos/administración & dosificación , Aceites de Plantas/administración & dosificación , Espasmo/prevención & control , Administración Oral , Anciano , California , Colonoscopía/efectos adversos , Preparaciones de Acción Retardada , Método Doble Ciego , Femenino , Humanos , Masculino , Mentha piperita , Persona de Mediana Edad , Parasimpatolíticos/efectos adversos , Aceites de Plantas/efectos adversos , Valor Predictivo de las Pruebas , Espasmo/etiología , Espasmo/fisiopatologíaRESUMEN
A 30-year-old healthy man presents after a fall with diffuse weakness and pain and recent onset left eye ptosis in the setting of three weeks of progressive bilateral leg weakness, gait instability, and difficulty washing his hair due to upper extremity weakness. He had also developed stiffness in his neck and shoulders and uncontrollable muscle spasms. Exam was notable for ptosis, fatiguing weakness in extremities, increased tone, hyperreflexia with clonus, and spastic gait. A mediastinal mass was found on chest CT (Fig. 1), and biopsy confirmed an invasive thymoma with positive nodes. Serum testing was positive for antibodies to acetylcholine receptors as well as glutamic acid decarboxylase.
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Miastenia Gravis/etiología , Síndrome de la Persona Rígida/etiología , Timoma/complicaciones , Neoplasias del Timo/complicaciones , Adulto , Trastornos Neurológicos de la Marcha/diagnóstico , Trastornos Neurológicos de la Marcha/etiología , Trastornos Neurológicos de la Marcha/fisiopatología , Humanos , Masculino , Debilidad Muscular/diagnóstico , Debilidad Muscular/etiología , Debilidad Muscular/fisiopatología , Miastenia Gravis/diagnóstico , Miastenia Gravis/fisiopatología , Metástasis de la Neoplasia , Espasmo/diagnóstico , Espasmo/etiología , Espasmo/fisiopatología , Síndrome de la Persona Rígida/diagnóstico , Síndrome de la Persona Rígida/fisiopatología , Timoma/diagnóstico , Timoma/patología , Neoplasias del Timo/diagnóstico , Neoplasias del Timo/patologíaRESUMEN
We present a case in which a movement disorder neurologist developed two different hyperkinetic movements due to corticospinal tract hyperexcitability, which resolved completely with surgical removal of an epidural spinal canal tumor. The first-hand description of these movements creates a unique opportunity for enhanced insight into these complex movement phenomena.
Asunto(s)
Neoplasias Epidurales/complicaciones , Hipercinesia/etiología , Linfoma de Células B Grandes Difuso/complicaciones , Mioclonía/etiología , Espasmo/etiología , Compresión de la Médula Espinal/etiología , Descompresión Quirúrgica , Neoplasias Epidurales/diagnóstico por imagen , Neoplasias Epidurales/patología , Neoplasias Epidurales/cirugía , Humanos , Hipercinesia/fisiopatología , Linfoma de Células B Grandes Difuso/diagnóstico por imagen , Linfoma de Células B Grandes Difuso/patología , Linfoma de Células B Grandes Difuso/cirugía , Masculino , Persona de Mediana Edad , Mioclonía/fisiopatología , Procedimientos Neuroquirúrgicos , Reflejo Anormal , Reflejo de Sobresalto , Espasmo/fisiopatología , Compresión de la Médula Espinal/diagnóstico por imagen , Compresión de la Médula Espinal/fisiopatología , Compresión de la Médula Espinal/cirugía , Vértebras TorácicasRESUMEN
BACKGROUND: The modern study of the traditional Chinese medicine (TCM) compound recipes is a complex issue because of the large number of components in the recipes that would produce several metabolites after entering the body. The TCM compound recipes are known to have the advantage of synergistic treatment by multiple targets due to diverse components. Therefore, a research method that can reflect the overall effect of compounds with multi-components is essential. The pharmacological studies of the classic TCM compound recipes mainly use the sero-pharmacological method. It is a semi-in vivo study method, and the drug to be tested in the in vitro experiment is the drug-containing serum from the model animals (the drug to be tested is a mixed drug system containing the prototype drugs in animal bodies that have pharmacodynamic effects and the metabolites). Herein, a safe and effective external TCM recipe was used to develop another semi-in vivo experimental method to reflect the overall effects of TCM. AIM: To observe the effects of in-vitro intestinal absorption liquid of aqueous extracts of the TCM compound recipe-Guangtongxiao foam aerosol (Guangtongxiao)-on the tension of isolated rectal rings of mouse and investigate the underlying mechanisms of antispasmodic and amelioration of muscular spasm-induced pain. METHODS: Intestinal absorption liquid of the Guangtongxiao aqueous extract at the five time points (30, 45, 75, 105, and 120 min) was prepared using a non-everted gut sac method. The isolated rectal rings of mice were prepared by pre-contraction using potassium chloride (KCl) or acetylcholine chloride (ACh) to make steady contraction. The intestinal absorption liquid were added cumulatively to the sink with the constricted rectal rings. The effects of the five groups of the intestinal absorption liquid with different drug concentration were observed on the tension of the isolated rectal rings. Then the ex vivo perfusion of the mouse rectal ring was performed as same as Guangtongxiao intestinal absorption liquid experiments, and the effects of two major components of Guangtongxiao, paeoniflorin (Pae) and tetrahydropalmatine (THP), on the rectal ring pre-treated with high concentration of KCl and ACh to induce contraction were studied. RESULTS: The relaxation rate of the five groups of the intestinal canals increased significantly with 3200 µL cumulative sample volume as compared to the blank group (P < 0.01). It suggested that the relaxation activity of the intestinal absorption liquid enhanced significantly with the prolongation of the interaction between isolated rectal rings and intestinal absorption liquid in a time-dependent manner. Also, significant differences were detected while comparing between the 120-min intestinal absorption liquid group and the blank group with respect to various cumulative sampling volumes (P < 0.01). In addition, the intestinal relaxation rate elevated gradually with the increase in sampling volume, indicating that the concentrations of active substances in the intestinal absorption liquid prepared by the non-everted gut sac model increased and the intestinal relaxation activity was enhanced with the prolongation of the absorption time in a dose-dependent manner. And Pae and THP in a concentration-dependent manner caused relaxation of the rectal ring, which is pretreated with high K+(KCl) and ACh to induce contraction. The EC50 of Pae and THP was 8.67 × 10-5 M (6.68 × 10-5-1.13 × 10-4) and 1.41 × 10-4 M (1.24 × 10-4-1.61 × 10-4) in the contraction model induced by KCl, and was 6.15 × 10-5 M (4.47 × 10-5-8.45 × 10-5), and 1.31 × 10-4 M(1.22 × 10-4-1.42 × 10-4) in the model induced by ACh, respectively. CONCLUSION: The intestinal absorption liquid of Guangtongxiao exerted a remarkable relaxation activity for the rectal rings, and relaxation of the smooth muscle tension might be one of the antispasmodic mechanisms of Guangtongxiao compound recipe. Also, adopting a semi-in-vivo experimental method to study the efficacy of topical external TCM recipe medicine is optimal.
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Medicamentos Herbarios Chinos/farmacología , Fármacos Gastrointestinales/farmacología , Relajación Muscular/efectos de los fármacos , Músculo Liso/efectos de los fármacos , Recto/efectos de los fármacos , Espasmo/prevención & control , Animales , Medicamentos Herbarios Chinos/metabolismo , Fármacos Gastrointestinales/metabolismo , Técnicas In Vitro , Absorción Intestinal , Mucosa Intestinal/metabolismo , Masculino , Ratones , Músculo Liso/fisiopatología , Ratas Sprague-Dawley , Recto/metabolismo , Recto/fisiopatología , Espasmo/fisiopatologíaRESUMEN
CDKL5 deficiency disorder (CDD) is a devastating neurodevelopmental disorder characterized by early-onset epilepsy, severe intellectual disability, cortical visual impairment and motor disabilities. Epilepsy is a central feature of CDD, with most patients having intractable seizures, but seizure frequency and severity can vary. Clinical reports demonstrate a diversity in seizure semiology and electrographic features, with no pattern diagnostic of CDD. Although animal models of CDD have shown evidence of hyperexcitability, spontaneous seizures have not been previously reported. Here, we present the first systematic study of spontaneous seizures in mouse models of CDD. Epileptic spasms, the most frequent and persistent seizure type in CDD patients, were recapitulated in two mouse models of CDD carrying heterozygous mutations, Cdkl5R59X and Cdkl5KO. Spasm-like events were present in a significant proportion of aged heterozygous female mice carrying either of the two Cdkl5 mutations with significant variability in seizure burden. Electrographically, spasms were most frequently associated with generalized slow-wave activity and tended to occur in clusters during sleep. CDD mice also showed interictal and background abnormalities, characterized by high-amplitude spiking and altered power in multiple frequency bands. These data demonstrate that aged female heterozygous Cdkl5 mice recapitulate multiple features of epilepsy in CDD and can serve to complement existing models of epileptic spasms in future mechanistic and translational studies.
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Envejecimiento/patología , Epilepsia/genética , Epilepsia/fisiopatología , Síndromes Epilépticos/genética , Síndromes Epilépticos/fisiopatología , Proteínas Serina-Treonina Quinasas/genética , Convulsiones/genética , Convulsiones/fisiopatología , Espasmos Infantiles/genética , Espasmos Infantiles/fisiopatología , Animales , Electroencefalografía , Femenino , Heterocigoto , Ratones , Ratones Noqueados , Mutación , Sueño de Onda Lenta , Espasmo/genética , Espasmo/fisiopatologíaRESUMEN
OBJECTIVES: Due to its rarity and similar sound, typewriter tinnitus (TT) can be misdiagnosed as middle ear myoclonic tinnitus (MEMT). We aim to clarify the characteristics of TT compared to MEMT, and the long-term therapeutic response to carbamazepine. METHODS: Fourteen patients with TT and 28 patients with MEMT were enrolled. RESULTS: TT patients were older than MEMT patients, and their tinnitus symptoms were mostly unilateral. Tinnitus symptoms, which is associated with dizziness, facial spasm, and head motion, were more common in TT, whereas MEMT were more related to noise. Acoustic reflex decay perturbation and low loudness discomfort level were diagnostic signs in MEMT patients, while decreased level of wave II in ABR was the most reliable sign in TT patients. All TT patients exhibited partial or complete response to carbamazepine, but there was a relapse rate after withdrawal of the drug was 60%. Increase in age and longer duration of symptoms were the risk factors of relapse of TT. CONCLUSION: The different characteristics observed in this study will be helpful to diagnose TT and MENT. Duration of tinnitus was the most important long-term prognostic factor of the carbamazepine trial, which indicates the importance of its earlier diagnosis.
Asunto(s)
Oído Medio/fisiopatología , Mioclonía/fisiopatología , Acúfeno/diagnóstico , Adulto , Anciano , Anticonvulsivantes/uso terapéutico , Audiometría de Tonos Puros , Carbamazepina/uso terapéutico , Mareo/fisiopatología , Intervención Médica Temprana , Potenciales Evocados Auditivos del Tronco Encefálico/fisiología , Músculos Faciales , Femenino , Humanos , Masculino , Persona de Mediana Edad , Movimiento , Ruido , Recurrencia , Reflejo Acústico/fisiología , Espasmo/fisiopatología , Factores de Tiempo , Acúfeno/clasificación , Acúfeno/tratamiento farmacológico , Acúfeno/fisiopatología , Adulto JovenRESUMEN
The loss of descending serotonin (5-HT) to the spinal cord contributes to muscle spasms in chronic spinal cord injury (SCI). Hyperexcitable motoneurons receive long-lasting excitatory postsynaptic potentials (EPSPs), which activate their persistent inward currents to drive muscle spasms. Deep dorsal horn (DDH) neurons with bursting behavior could be involved in triggering the EPSPs due to loss of inhibition in the chronically 5-HT-deprived spinal cord. Previously, in an acutely transected preparation, we found that bursting DDH neurons were affected by administration of the 5-HT1B/1D receptor agonist zolmitriptan, which suppressed their bursts, and by N-methyl-d-aspartate (NMDA), which enhanced their bursting behavior. Nonbursting DDH neurons were not influenced by these agents. In the present study, we investigate the firing characteristics of bursting DDH neurons following chronic spinal transection at T10 level in adult mice and examine the effects of replacing lost endogenous 5-HT with zolmitriptan. Terminal experiments using our in vitro preparation of the sacral cord were carried out ~10 wk postransection. Compared with the acute spinal stage of our previous study, DDH neurons in the chronic stage became more responsive to dorsal root stimulation, with burst duration doubling with chronic injury. The suppressive effects of zolmitriptan were stronger overall, but the facilitative effects of NMDA were weaker. In addition, the onset of DDH neuron activity preceded ventral root output and the firing rates of DDH interneurons correlated with the integrated long-lasting ventral root output. These results support a contribution of the bursting DDH neurons to muscle spasms following SCI and inhibition by 5-HT.NEW & NOTEWORTHY We investigate the firing characteristics of bursting deep dorsal horn (DDH) neurons following chronic spinal transection. DDH neurons in the chronic stage are different from those in the acute stage as noted by their increase in excitability overall and their differing responses serotonin (5-HT) and N-methyl-d-aspartate (NMDA) receptor agonists. Also, there is a strong relationship between DDH neuron activity and ventral root output. These results support a contribution of the bursting DDH neurons to muscle spasms following chronic spinal cord injury (SCI).
Asunto(s)
Potenciales de Acción/fisiología , Potenciales Postsinápticos Excitadores/fisiología , Interneuronas/fisiología , Neuronas Motoras/fisiología , Células del Asta Posterior/fisiología , Agonistas del Receptor de Serotonina 5-HT1/farmacología , Serotonina/metabolismo , Espasmo , Traumatismos de la Médula Espinal , Raíces Nerviosas Espinales , Potenciales de Acción/efectos de los fármacos , Animales , Enfermedad Crónica , Modelos Animales de Enfermedad , Potenciales Postsinápticos Excitadores/efectos de los fármacos , Interneuronas/efectos de los fármacos , Interneuronas/metabolismo , Ratones , Ratones Endogámicos C57BL , Neuronas Motoras/efectos de los fármacos , Neuronas Motoras/metabolismo , N-Metilaspartato/farmacología , Oxazolidinonas/farmacología , Células del Asta Posterior/efectos de los fármacos , Células del Asta Posterior/metabolismo , Espasmo/metabolismo , Espasmo/fisiopatología , Traumatismos de la Médula Espinal/metabolismo , Traumatismos de la Médula Espinal/fisiopatología , Raíces Nerviosas Espinales/efectos de los fármacos , Raíces Nerviosas Espinales/metabolismo , Raíces Nerviosas Espinales/fisiopatología , Triptaminas/farmacologíaRESUMEN
Elective and emergent coronary interventions via transradial access (TRA) are been used increasingly as they carry a reduced risk of complications. Percutaneous coronary intervention via TRA may lead to radial artery spasms (RAS) that may result in prolonged procedure time, cross-over of access site, and other complications. This review article discusses the recent definitions, incidences, pathophysiology, predictive score calculator, efficacy, the safety of the radial cocktails, and treatment of the RAS.
Asunto(s)
Arteria Radial/fisiopatología , Espasmo/etiología , Femenino , Humanos , Masculino , Factores de Riesgo , Espasmo/fisiopatología , Resultado del TratamientoRESUMEN
La rigidez cadavérica (rigor mortis) es un proceso no muy bien comprendido por la mayoría de los médicos. El conocimiento de la intimidad del proceso de la rigidez cadavérica es de vital importancia ya que es una de las variables que junto con las livideces (livor mortis) y la temperatura (algor mortis) del cadáver ayudan a determinar el cronotanatodiagnóstico, tanatocronodiagnóstico o intervalo postmortal del período inmediato de la muerte. Para entender el mecanismo de la rigidez y el espasmo cadavérico es preciso hacer un repaso de la contracción muscular fisiológica en el vivo. Hay que tener presente que el tipo de fibra muscular predominante modificará las características de la contracción muscular fisiológica en el vivo, y también la rigidez y el espasmo cadavérico. (AU)
The cadaveric rigidity (rigor mortis) is a process which is not very well understood by the majority of the doctors. The knowledge of the intimacy of the cadaveric stiffness process is of vital importance since it is one of the variables that, as well as the postmortem lividity (livor mortis) and the body temperature post mortem (algor mortis) help determine the chronotanatodiagnostic, tanatochronodiagnostic or postmortal interval of the immediate period of death. In order to understand the mechanism of stiffness and cadaveric spasm, it is necessary to review the physiological muscle contraction in vivo. We should keep in mind that the predominant type of muscle fiber will modify the characteristics of physiological muscle contraction in vivo, as well as stiffness and cadaveric spasm. (AU)
Asunto(s)
Humanos , Rigor Mortis/fisiopatología , Espasmo/fisiopatología , Fibras Musculares Esqueléticas/clasificación , Fibras Musculares Esqueléticas/fisiología , Factores de Tiempo , Contracción Muscular/fisiología , Relajación Muscular/fisiologíaRESUMEN
During the perinatal period in mammals when active sleep predominates, skeletal muscles twitch throughout the body. We have hypothesized that myoclonic twitches provide unique insight into the functional status of the human infant's nervous system. However, assessments of the rate and patterning of twitching have largely been restricted to infant rodents. Thus, here we analyze twitching in human infants over the first seven postnatal months. Using videography and behavioral measures of twitching during bouts of daytime sleep, we find at all ages that twitching across the body occurs predominantly in bursts at intervals of 10 s or less. We also find that twitching is expressed differentially across the body and with age. For example, twitching of the face and head is most prevalent shortly after birth and decreases over the first several months. In addition, twitching of the hands and feet occurs at a consistently higher rate than does twitching elsewhere in the body. Finally, the patterning of twitching becomes more structured with age, with twitches of the left and right hands and feet exhibiting the strongest coupling. Altogether, these findings support the notion that twitches can provide a unique source of information about typical and atypical sensorimotor development.
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Desarrollo Infantil/fisiología , Músculo Esquelético/fisiología , Sueño/fisiología , Espasmo/fisiopatología , Animales , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Sueño REM/fisiología , Análisis Espacio-Temporal , Grabación en VideoRESUMEN
We report a case of accommodative spasm in an 8-year-old girl discovered by handheld photoscreener. The patient was found to have a Chiari I malformation, and managed with atropine drops and reading glasses, ultimately with improvement in her symptoms. We believe this to be the first case of accommodative spasm diagnosis aided by the use of a handheld photoscreener.
Asunto(s)
Acomodación Ocular/fisiología , Malformación de Arnold-Chiari/diagnóstico , Diplopía/diagnóstico , Espasmo/diagnóstico , Selección Visual/instrumentación , Acomodación Ocular/efectos de los fármacos , Atropina/uso terapéutico , Niño , Diplopía/tratamiento farmacológico , Diplopía/fisiopatología , Femenino , Humanos , Midriáticos/uso terapéutico , Espasmo/tratamiento farmacológico , Espasmo/fisiopatología , Agudeza VisualRESUMEN
BACKGROUND: The loss of functional ability of patients after stroke is mostly caused by dysfunction of the upper limbs, especially the hands. Hand functional exercise is the premise of alleviating hand dysfunction, and the relief of hand spasm is the basis of timely and effective hand functional exercise. Previous clinical observation have shown that fascial-point needling can effectively alleviate hand spasm immediately after stroke, but further evidence from large-sample studies is needed. The overall objective of this trial is to further evaluate the clinical efficacy of fascial-point acupuncture on hand spasm after stroke. METHODS/DESIGN: This multicenter randomized controlled trial will compare the efficacy of fascial-point acupuncture versus sham acupuncture and routine rehabilitation therapy in stroke patients with hand spasm. Patients will be randomized to undergo either the fascial-point acupuncture, the sham acupuncture or the control (routine rehabilitation therapy). We will recruit 210 stroke inpatients who meet the trial criteria and observe the remission of hand spasm and improvement of limb function after 4 weeks of intervention. The first evaluation indices are the remission of hand spasm and the duration of spasm remission. The second evaluation indices are the hand function of the affected limbs and the activities of daily living. When the accumulative total number of cases included reaches 120, a mid-term analysis will be conducted to determine any evidence that experimental intervention does have an advantage. DISCUSSION: Our aim is to evaluate the efficacy of fascial-point acupuncture in relieving hand spasm after stroke. The results should provide more evidence for the clinical application of this therapy in the future. TRIAL REGISTRATION: Chinese Clinical Trial Registry (ChiCTR), ID: ChiCTR1900022379. Registered on 9 April 2019.