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1.
Anesth Prog ; 70(3): 124-127, 2023 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-37850673

RESUMO

Dental treatment for patients with cerebral palsy (CP) is often performed under general anesthesia due to involuntary movements that can render dental treatment difficult. Since CP is often accompanied by spasticity, care must be taken when positioning patients during general anesthesia. We report the management of a 14-year-old girl with CP and epilepsy undergoing general anesthesia for dental treatment who experienced respiratory failure due to acute thoracoabdominal muscle hypertonia after extubation. She had a history of cardiac arrest due to respiratory failure caused by acute muscle hypertonia and successful resuscitation. General anesthesia was induced after careful positioning of the patient to prevent spastic muscle stretching, and the dental treatment was completed without complications. However, upon awakening after extubation, the patient developed respiratory failure due to acute muscle hypertonia. The patient was resedated and repositioned from a supine to a sitting position, and her symptoms improved. There was no recurrence of muscle hypertonia, and she recovered fully without complications. In this case, respiratory failure associated with acute muscle hypertonia was successfully managed by position change after initial treatment with positive-pressure ventilation and propofol. It is important to be prepared for the possibility of respiratory failure associated with acute muscle hypertonia and its countermeasures when providing general anesthesia for patients with CP.


Assuntos
Paralisia Cerebral , Propofol , Insuficiência Respiratória , Humanos , Criança , Feminino , Adolescente , Paralisia Cerebral/complicações , Hipertonia Muscular/complicações , Insuficiência Respiratória/etiologia , Insuficiência Respiratória/terapia , Espasticidade Muscular/complicações
2.
Sleep Med ; 100: 501-510, 2022 12.
Artigo em Inglês | MEDLINE | ID: mdl-36274383

RESUMO

INTRODUCTION: From an ongoing multicenter effort toward differentiation of Parkinsonian spectrum disorders (PSD) from other types of neurodegenerative disorders, the sleep biomarker non-rapid-eye-movement sleep with hypertonia (NRH) emerged. METHODS: This study included in the PSD group patients with dementia with Lewy bodies/Parkinson disease dementia (DLB/PDD = 16), Parkinson disease (PD = 16), and progressive supranuclear palsy (PSP = 13). The non-PSD group included patients with Alzheimer disease dementia (AD = 24), mild cognitive impairment (MCI = 35), and a control group with normal cognition (CG = 61). In-home, multi-night Sleep Profiler studies were conducted in all participants. Automated algorithms detected NRH, characterized by elevated frontopolar electromyographic power. Between-group differences in NRH were evaluated using Logistic regression, Mann-Whitney U and Chi-squared tests. RESULTS: NRH was greater in the PSD group compared to non-PSD (13.9 ± 11.0% vs. 3.1 ± 4.7%, P < 0.0001). The threshold NRH≥5% provided the optimal between-group differentiation (AUC = 0.78, P < 0.001). NRH was independently associated with the PSD group after controlling for age, sex, and SSRI/SNRI use (P < 0.0001). The frequencies of abnormal NRH by subgroup were PSP = 92%, DLB/PDD = 81%, PD = 56%, MCI = 26%, AD = 17%, and CG = 16%. The odds of abnormal NRH in each PSD subgroup ranged from 3.7 to 61.2 compared to each non-PSD subgroup. The night-to-night and test-retest intraclass correlations were excellent (0.78 and 0.84, both P < 0.0001). CONCLUSIONS: In this pilot study, NRH appeared to be a novel candidate sleep biomarker for PSD-related neurodegeneration. Future studies in larger cohorts are needed to confirm these findings, understand the etiology of NRH magnitude/duration, and determine whether it is an independent prodromal marker for specific neurodegenerative pathologies.


Assuntos
Doença de Alzheimer , Demência , Doença por Corpos de Lewy , Doença de Parkinson , Humanos , Doença de Parkinson/psicologia , Projetos Piloto , Demência/complicações , Doença de Alzheimer/complicações , Hipertonia Muscular/complicações , Biomarcadores , Sono
3.
Diabetes Metab Syndr ; 16(10): 102613, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-36116326

RESUMO

BACKGROUND AND AIMS: Non-alcoholic fatty liver (FL) is comorbid with obesity, metabolic syndrome and type 2 diabetes. Atherogenic dyslipidaemia (AD), frequent in FL, is associated with risk of micro- and macrovascular complications. Given the paradoxical ocular protection of FL in T2DM, we studied how FL modulates micro- and macrovascular complications as a function of AD. METHODS: Cross-sectional factorial analysis of 744 diabetic patients in whom FL, identified by ultrasonography, was present in 68%. AD, defined by low HDL-C plus elevated TG, was present in 45%. Four groups were analysed as regards cardiometabolic features, micro-/macroangiopathies, cataract and ocular hypertonia: FL[-]AD[-] (n = 171); FL[-]AD[+] (n = 66); FL[+]AD[-] (n = 235); and FL[+]AD[+] (n = 272). RESULTS: Age, gender and glycemic control were similar across groups. Prevalence of overall macroangiopathy and coronary artery disease were higher in patients with AD, irrespective of FL. Overall macroangiopathy was higher, by 64% in FL[-]AD[+] and by 38% in FL[+]AD[+]. Coronary artery disease was higher, by 128%, in FL[-]AD[+], and by 67%, in FL[+]AD[+]. (Micro)albuminuria was more frequent (+55%) in FL[-] AD[+] compared to FL[-] AD[-]. Retinopathy prevalence was 35% in FL[-], unaffected by AD. Retinopathy frequency was much lower in FL[+], irrespective of AD, decreased by -47% in FL[+]AD[-] and -32% in FL[+]AD[+] (vs. FL[-]AD[-]). Ocular hypertonia was present in 13%, and its prevalence was also markedly lower (-31%) in FL[+]. Cataract frequency was 29%, also lesser in FL[+] (24% vs. 39%), irrespective of AD. CONCLUSIONS: Multi-level eye protection in diabetes is linked to non-alcoholic fatty liver independently of atherogenic dyslipidemia.


Assuntos
Catarata , Doença da Artéria Coronariana , Diabetes Mellitus Tipo 2 , Angiopatias Diabéticas , Dislipidemias , Hepatopatia Gordurosa não Alcoólica , Doenças Retinianas , Humanos , Angiopatias Diabéticas/etiologia , Angiopatias Diabéticas/complicações , Diabetes Mellitus Tipo 2/complicações , Diabetes Mellitus Tipo 2/epidemiologia , Estudos Transversais , Doença da Artéria Coronariana/complicações , Dislipidemias/complicações , Dislipidemias/epidemiologia , Hepatopatia Gordurosa não Alcoólica/complicações , Hepatopatia Gordurosa não Alcoólica/epidemiologia , Doenças Retinianas/complicações , Hipertonia Muscular/complicações
4.
Rev Med Liege ; 77(9): 484-490, 2022 Sep.
Artigo em Francês | MEDLINE | ID: mdl-36082592

RESUMO

Spasticity is a form of hypertonia frequently encountered in patients who suffered from stroke and is a cause of functional limitation, contractures, osteoarticular deformations, pain and wounds. The first-line treatment of focal or multifocal spasticity consists in intramuscular injections of botulinum toxin type A in the muscles concerned, in association with positioning measures and physical therapy. Despite the fact this pathology is common, there is often a delay in the diagnosis, and consequently the treatment, leading to harmful consequences for the patient. In some specific indications, surgery can also be a therapeutic option. Finally, spasticity should be distinguished from other types of hypertonia, e.g. dystonia.


: La spasticité est une forme d'hypertonie, fréquemment rencontrée dans les suites d'un accident vasculaire cérébral. Elle peut être à l'origine de limitations fonctionnelles, de rétractions tendineuses, de déformations ostéoarticulaires, de douleurs et de plaies. Le traitement de première ligne de la spasticité focale ou multifocale repose sur l'injection intramusculaire de toxine botulique de type A dans les muscles entrepris, en association à des mesures de positionnement et à une prise en charge kinésithérapeutique. Malgré la fréquence de cette affection, elle est encore trop souvent reconnue, et donc traitée, tardivement, ce qui aboutit à des conséquences délétères pour le patient. Dans certaines indications précises, la chirurgie a également une place dans l'arsenal thérapeutique de la spasticité. Enfin, la spasticité ne doit pas être confondue avec les autres formes d'hypertonie, comme la dystonie.


Assuntos
Toxinas Botulínicas Tipo A , Acidente Vascular Cerebral , Toxinas Botulínicas Tipo A/uso terapêutico , Humanos , Injeções Intramusculares/efeitos adversos , Hipertonia Muscular/complicações , Hipertonia Muscular/tratamento farmacológico , Espasticidade Muscular/diagnóstico , Espasticidade Muscular/etiologia , Espasticidade Muscular/terapia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/terapia , Resultado do Tratamento
5.
Exp Neurol ; 354: 114027, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-35245503

RESUMO

The majority of patients simultaneously develop motor dysfunction and spastic hypertonia after ischemic strokes, which can be associated with an increasing trend in motor impairments, seriously impeding the rehabilitation process. Evidence suggests that some deficits in the KCC2 expression in the spinal cord along with maladaptive endogenous plasticity via GABAA receptors are often involved in the pathology of spastic hypertonia after a stroke. In this respect, acupuncture has been commonly used in clinical settings for post-stroke patients' rehabilitation. Nevertheless, the mechanism of the modulating activity of this alternative medicine in the spinal pathways to relieve spasticity and improve functional recovery after a stroke has still remained unclear. Utilizing laser speckle imaging, functional assessments (viz. neurologic function scale, muscular tension scale, foot balance test, and gait analysis), H-reflex recording, TTC, Western blotting, RT-qPCR, ELISA, and immunofluorescence molecular assay, the study results illustrated that acupuncture could significantly alleviate the spinal hyperreflexia, decrease muscle tone, and enhance locomotor function by elevating the GABA, KCC2, and GABAAγ2 expressions in the lumbar spine of a rat model of post-ischemic stroke with spastic hypertonia. Furthermore, the KCC2 antagonist DIOA abolished the benefits induced by this practice. Overall, the data revealed that acupuncture is a promising therapeutic approach for spastic hypertonia after a stroke, and the positive outcomes in this sense could be achieved via activating the KCC2-mediated spinal GABAA signaling pathway.


Assuntos
Terapia por Acupuntura , AVC Isquêmico , Acidente Vascular Cerebral , Simportadores , Animais , Humanos , Hipertonia Muscular/complicações , Hipertonia Muscular/terapia , Espasticidade Muscular/etiologia , Espasticidade Muscular/metabolismo , Espasticidade Muscular/terapia , Ratos , Receptores de GABA-A , Reflexo Anormal , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/terapia , Simportadores/metabolismo , Ácido gama-Aminobutírico
6.
J Clin Endocrinol Metab ; 107(5): e1938-e1945, 2022 04 19.
Artigo em Inglês | MEDLINE | ID: mdl-35022743

RESUMO

CONTEXT: It is well known that Graves disease (GD) causes sleep disorders (SDs). However, the characteristics and associated factors of SD and its clinical course post hyperthyroidism normalization remain unclear. OBJECTIVE: To clarify the characteristics and associated factors of subjective SD and its clinical course after GD treatment. METHODS: From November 2017 to October 2020, we enrolled 72 participants (22 newly diagnosed with GD with untreated hyperthyroidism, 20 previously diagnosed with GD with normal thyroid function, and 30 normal controls) with no other underlying SD-related diseases. We compared the groups at enrollment and conducted prospective observations after 12 months of treatment on participants with newly diagnosed GD. Main outcome measures were differences and changes in the Pittsburgh Sleep Quality Index (PSQI) global and component sleep quality scores. RESULTS: PSQI global sleep quality scores (P = .036) and sleep disturbance scores (P = .011) were significantly different among the 3 groups, and were highest in the untreated hyperthyroidism group. Multiple regression analysis demonstrated that free thyroxine level, which was positively correlated with sympathetic tone (ST) as evaluated by pulse rate, and urinary total metanephrines was associated with poorer PSQI global sleep quality scores independently of other factors (P = .006). Prospective observation showed that PSQI global sleep quality scores (P = .018) and sleep disturbance scores (P = .011) significantly improved with thyroid function normalization and ST attenuation. CONCLUSION: Hyperthyroidism caused by GD augmented ST and exacerbated subjective SD. Normalization of hyperthyroidism caused by GD improved subjective SD.


Assuntos
Doença de Graves , Hipertireoidismo , Transtornos do Sono-Vigília , Doença de Graves/complicações , Humanos , Hipertireoidismo/tratamento farmacológico , Hipertonia Muscular/complicações , Estudos Prospectivos , Sono , Transtornos do Sono-Vigília/diagnóstico , Transtornos do Sono-Vigília/epidemiologia , Transtornos do Sono-Vigília/etiologia
7.
Acupunct Med ; 40(4): 312-321, 2022 08.
Artigo em Inglês | MEDLINE | ID: mdl-34894776

RESUMO

BACKGROUND: Persons with stroke commonly have impairments associated with a reduction in functionality. Motor impairments are the most prevalent, causing an impact on activities of daily life. OBJECTIVE: The aim of this study was to evaluate the effect of a session of dry needling (DN) applied to the upper extremity muscles on the sensorimotor function, hypertonia, and quality of life of persons with chronic stroke. METHODS: A randomized, sham-controlled clinical trial was performed. Participants were randomly assigned into an intervention group that received a single session DN in the biceps brachii, brachialis, flexor digitorum superficialis and profundus, extensor digitorum, adductor pollicis and triceps brachii muscles, or into a control group that received the same treatment but with a sham DN intervention. Treatment outcomes included the Fugl-Meyer Assessment Scale for the upper extremity, the Modified Modified Ashworth Scale, and the EuroQol-5D questionnaire. Measurements were carried out before, immediately after, and 14 days after intervention. RESULTS: Twenty-three persons participated in the study. Significant differences between groups were observed after the intervention in the total wrist-hand motor score (p = 0.023) and sensorimotor score (p = 0.022), for hypertonia in the elbow extensors both after treatment (p = 0.002) and at follow-up (p = 0.018), and in quality of life at follow-up (p = 0.030). CONCLUSIONS: A single session of DN improved total wrist-hand motor function and total sensorimotor function in persons with chronic stroke immediately after treatment, as well as quality of life 2 weeks after treatment. TRIAL REGISTRATION NUMBER: NCT03546517 (ClinicalTrials.gov).


Assuntos
Agulhamento Seco , Reabilitação do Acidente Vascular Cerebral , Acidente Vascular Cerebral , Humanos , Hipertonia Muscular/complicações , Hipertonia Muscular/terapia , Qualidade de Vida , Acidente Vascular Cerebral/complicações , Resultado do Tratamento
8.
Chirurgia (Bucur) ; 115(5): 585-594, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33138895

RESUMO

PURPOSE: The role of augmented internal anal sphincter (IAS) tone in the genesis of posterior chronic anal fissure (CAPF) is still unknown. Lateral internal sphincterotomy is the most employed surgical procedure, nevertheless it is burdened by high risk post-operative anal incontinence. The aim of our study is to evaluate results of sphincter saving procedure with post-operative pharmacological sphincterotomy for patients affected by CAPF with IAS hypertonia. Methods: We enrolled 30 patients, undergone fissurectomy and anoplasty with V-Y cutaneous flap advancement; all patients received topical administration of nifedipine 0.3% and lidocaine 1.5% ointment-based therapy before and for 15 days after surgery. The primary goal was patient's complete healing and the evaluation of incontinence and recurrence rate; the secondary goal included the evaluation of manometry parameters, symptom relief and complications related to nifedipine and lidocaine administration. Results: All wounds healed within 40 days after surgery. We didn't observe any de novo postoperative anal incontinence case. We reported 2 cases of recurrences, healed after conservative therapy. We didn't report any local complications related to the administration of the ointment therapy; with whom all patients reported a good compliance. Conclusions: Fissurectomy and anoplasty with V-Y cutaneous advancement flap and topical administration of nifedipine and lidocaine, is an effective treatment for CAPF with IAS hypertonia.


Assuntos
Canal Anal/efeitos dos fármacos , Bloqueadores dos Canais de Cálcio/administração & dosagem , Fissura Anal , Hipertonia Muscular/tratamento farmacológico , Nifedipino , Administração Tópica , Canal Anal/cirurgia , Anestésicos Locais/administração & dosagem , Doença Crônica , Terapia Combinada , Fissura Anal/complicações , Fissura Anal/tratamento farmacológico , Fissura Anal/cirurgia , Humanos , Lidocaína , Hipertonia Muscular/complicações , Hipertonia Muscular/cirurgia , Nifedipino/administração & dosagem , Pomadas/administração & dosagem , Estudos Prospectivos , Retalhos Cirúrgicos , Resultado do Tratamento
9.
Eur J Orthop Surg Traumatol ; 30(4): 621-627, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31863270

RESUMO

PURPOSE: The purpose of this study is to evaluate whether patients with high-tone neuromuscular early-onset scoliosis have different surgical outcome and complication rate, when compared to patients with low-tone neuromuscular early-onset scoliosis treated with a rib-to-pelvis rib-based dual growing system. METHODS: This is a retrospective cohort study of 67 neuromuscular early-onset scoliosis patients, collected from a multicenter database, treated with a rib-to-pelvis rib-based dual growing system. All patients were divided into two groups: high tone and low tone. Pre-, intra- and postoperative data were compared between both groups. Complications were reported by a standardized system. RESULTS: Twenty-six high-tone and 41 low-tone patients were found homogeneous regarding gender, age at surgery, weight, height, estimated blood loss and surgery time. High-tone group (19/26 = 73.1%) experiences more postoperative complications than low-tone group (22/41 = 53.7%). Most common complications were infection, device migration, death and hardware failure. Permanent abandonment of rib-based growing technique and device removal was required in 21% of high-tone patients (P < 0.001). None of the low-tone patients required abandonment. CONCLUSION: High-tone patients had more complications than those with low tone in management of neuromuscular early-onset scoliosis treated with a rib-to-pelvis rib-based dual growing system. A different surgical approach may be required to treat the high-tone neuromuscular early-onset scoliosis.


Assuntos
Procedimentos Ortopédicos , Complicações Pós-Operatórias , Próteses e Implantes , Costelas , Escoliose , Coluna Vertebral , Idade de Início , Desenvolvimento Ósseo , Criança , Feminino , Seguimentos , Humanos , Incidência , Masculino , Hipertonia Muscular/complicações , Hipertonia Muscular/diagnóstico , Hipotonia Muscular/complicações , Hipotonia Muscular/diagnóstico , Procedimentos Ortopédicos/efeitos adversos , Procedimentos Ortopédicos/instrumentação , Procedimentos Ortopédicos/métodos , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Porto Rico/epidemiologia , Estudos Retrospectivos , Costelas/diagnóstico por imagem , Costelas/cirurgia , Fatores de Risco , Escoliose/epidemiologia , Escoliose/fisiopatologia , Escoliose/cirurgia , Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/cirurgia , Resultado do Tratamento
10.
Dysphagia ; 34(5): 713-715, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31230142

RESUMO

The etiopathogenesis of Zenker's diverticulum (ZD) remains uncertain. Increased hypopharyngeal pressure due to a hypertonic upper esophageal sphincter results in herniation proximal to the sphincter producing a pulsion diverticulum. Gastroesophageal reflux, which is known to induce shortening of the injured esophagus, likely plays a prominent role in ZD formation by pulling the cricopharyngeus muscle (CPM) away from the anchored inferior constrictor muscle. This creates a "weak zone" encouraging herniation. A bilobed diverticulum may originate from continuation of the fibrous midline raphe inferiorly to developmentally include part of the CPM. We report using laser endoscopy to divide the inter-diverticular septum followed by transmucosal cricopharyngeus myotomy. Presentation of a rare, bilobed diverticulum emphasizes the importance of the midline prevertebral raphe in anchoring the pharyngeal constrictor muscles with respect to the CPM. This lends support to the hypothesis that the etiopathogenesis of ZD is multifactorial while guiding us to a unified understanding of ZD.


Assuntos
Divertículo/patologia , Doenças Faríngeas/patologia , Divertículo de Zenker/patologia , Divertículo/etiologia , Esfíncter Esofágico Superior/patologia , Refluxo Gastroesofágico/complicações , Humanos , Hipofaringe/patologia , Hipertonia Muscular/complicações , Hipertonia Muscular/patologia , Doenças Faríngeas/etiologia , Músculos Faríngeos/patologia , Pressão , Divertículo de Zenker/etiologia
12.
Exp Neurol ; 293: 13-26, 2017 07.
Artigo em Inglês | MEDLINE | ID: mdl-28347765

RESUMO

While antenatal hypoxia-ischemia (H-I) is a well-established cause of brain injury, the effects of H-I on the spinal cord remain undefined. This study examined whether hypertonia in rabbits was accompanied by changes in spinal architecture. Rabbit dams underwent global fetal H-I at embryonic day 25 for 40min. High resolution diffusion tensor imaging was performed on fixed neonatal CNS. Fractional anisotropy (FA) and regional volumetric measurements were compared between kits with and without hypertonia after H-I and sham controls using Tract Based Spatial Statistics. Hypertonic kits showed evidence of damage from hypoxia not only in the brain, but in spinal cord as well. Hypertonic kits showed reduced FA and thickness in corticospinal tracts, external capsule, fimbria, and in white and gray matter of both cervical and lumbar spinal cord. Dorsal white matter of the spinal cord was the exception, where there was thickening and increased FA in hypertonic kits. Direct damage to the spinal cord was demonstrated in a subset of dams imaged during H-I with a 3T magnetic resonance scanner, where apparent diffusion coefficient in fetal spinal cords acutely decreased during hypoxia. Hypertonic kits showed subsequent decreases in lumbar motoneuron counts and extensive TUNEL- and Fluoro-Jade C-positive labeling was present in the spinal cord 48h after H-I, demonstrating spinal neurodegeneration. We speculate that global H-I causes significant loss of both spinal white and gray matter in hypertonic newborns due to direct H-I injury to the spinal cord as well as due to upstream brain injury and consequent loss of descending projections.


Assuntos
Paralisia Cerebral/etiologia , Hipóxia Fetal/complicações , Hipertonia Muscular/complicações , Hipertonia Muscular/etiologia , Traumatismos da Medula Espinal/etiologia , Animais , Animais Recém-Nascidos , Sistema Nervoso Central/diagnóstico por imagem , Paralisia Cerebral/diagnóstico por imagem , Imagem de Tensor de Difusão , Modelos Animais de Doenças , Feminino , Fluoresceínas/metabolismo , Proteína Glial Fibrilar Ácida/metabolismo , Gliose/etiologia , Processamento de Imagem Assistida por Computador , Marcação In Situ das Extremidades Cortadas , Vias Neurais/diagnóstico por imagem , Vias Neurais/patologia , Gravidez , Coelhos , Traumatismos da Medula Espinal/diagnóstico por imagem , Fatores de Tempo , Substância Branca/diagnóstico por imagem , Substância Branca/patologia
13.
J Child Neurol ; 32(1): 132-138, 2017 01.
Artigo em Inglês | MEDLINE | ID: mdl-27742862

RESUMO

The Hypertonia Assessment Tool is a 7-item instrument that discriminates spasticity, dystonia, and rigidity on 3 levels: item scores, subtype, and hypertonia diagnosis for each extremity. We quantified the inter- and intrarater reliability using Kappa statistics, Gwet's first-order agreement coefficient (both with 95% confidence interval), and percentage agreement for all levels. For validity, we compared the Hypertonia Assessment Tool subtype with the clinical diagnosis provided by the physicians. Two physiotherapists tested 45 children with neuromotor disorders. The interrater reliability (n = 45) of the Hypertonia Assessment Tool subtype was moderate to substantial whereas the intrarater reliability (n = 42) was almost perfect. The Hypertonia Assessment Tool showed good agreement in detecting spasticity. On the contrary, there was a higher presence of dystonia of 24% to 25% tested with the Hypertonia Assessment Tool compared to the clinical diagnosis. Even some individual items showed lower agreement between raters; the Hypertonia Assessment Tool subtypes and diagnosis were reliable. Validity of the Hypertonia Assessment Tool to test spasticity is confirmed, whereas, for dystonia and rigidity, further studies are needed.


Assuntos
Transtornos dos Movimentos/complicações , Transtornos dos Movimentos/diagnóstico , Hipertonia Muscular/complicações , Hipertonia Muscular/diagnóstico , Adolescente , Criança , Pré-Escolar , Estudos Transversais , Distonia/complicações , Distonia/diagnóstico , Distonia/fisiopatologia , Feminino , Humanos , Extremidade Inferior/fisiopatologia , Masculino , Transtornos dos Movimentos/fisiopatologia , Hipertonia Muscular/fisiopatologia , Psicometria , Reprodutibilidade dos Testes , Tradução , Extremidade Superior/fisiopatologia , Adulto Jovem
14.
Neuropediatrics ; 47(4): 273-7, 2016 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27214504

RESUMO

Distal arthrogryposis (DA) is a feature in genetically and clinically heterogeneous groups of disorders. Mostly myopathic and neurogenic defects have been described, but many patients remain without genetic diagnosis. We are elaborating on the clinical presentation of neonatal cases with DA who carry novel mutations in the nonselective sodium leak channel (NALCN). Two patients reported herein were remarkable for central hypertonicity in addition to DA. By trio-whole exome sequencing, two undescribed de novo mutations in NALCN were revealed. Both mutations (p.F317C and p.V595F) are located on pore-forming segments of NALCN. Dominant NALCN mutations in the pore-forming segments have been identified in similar patients, whereas recessive mutations outside the pore-forming segments result in different phenotypes. Our findings with central hypertonia broaden the phenotypic spectrum of de novo mutations in the pore-forming segments of NALCN. Recent findings of successful acetazolamide treatment in patients with channelopathies might point to potential therapies based on the ion channel similarities and the location of the mutation.


Assuntos
Disostose Craniofacial/genética , Hipertonia Muscular/genética , Canais de Sódio/genética , Encéfalo/diagnóstico por imagem , Disostose Craniofacial/complicações , Disostose Craniofacial/diagnóstico , Disostose Craniofacial/diagnóstico por imagem , Feminino , Humanos , Recém-Nascido , Recém-Nascido Prematuro , Canais Iônicos , Imageamento por Ressonância Magnética , Proteínas de Membrana , Hipertonia Muscular/complicações , Hipertonia Muscular/diagnóstico , Radiografia Torácica
15.
J Clin Sleep Med ; 12(6): 839-47, 2016 06 15.
Artigo em Inglês | MEDLINE | ID: mdl-26857056

RESUMO

STUDY OBJECTIVES: Rigidity is a muscle hypertonia typical of Parkinson disease (PD), whereas rapid eye movement (REM) sleep behavior disorder (RBD) is characterized by abnormally increased muscle tone during REM sleep (REM sleep without atonia) and enacting dream behaviors. Because movements are not bradykinetic during RBD in patients with PD, we investigated whether the background, wake postural rigidity is attenuated during REM sleep without atonia, in absence of movement. METHODS: The amplitude of levator menti (postural muscle) electromyographic activity during relaxed evening wakefulness (considered as reference) and sleep (N2, N3, atonic REM sleep, and quiet REM sleep without atonia) was measured in 20 patients with PD (with and without RBD), 10 patients with idiopathic RBD patients and 10 healthy subjects. RESULTS: The chin tone amplitude progressively decreased from wake to N2, N3, and atonic REM sleep in the four groups, but the highest amplitude was observed in PD patients with RBD during atonic REM sleep. Furthermore, chin muscle tone amplitude did not attenuate from wake to REM sleep without atonia in patients with both PD and RBD but dramatically attenuated (by 40% on average) in patients with idiopathic RBD. CONCLUSIONS: The high amplitude of chin muscle tone in PD with RBD (but not in idiopathic RBD) during REM sleep with and without atonia suggests that both PD-related hypertonia and RBD-related enhanced muscle tone coexist during REM sleep, together affecting chin muscle tone. Consequently, some rapid RBD movements likely start against a rigid postural tone.


Assuntos
Hipertonia Muscular/complicações , Hipertonia Muscular/fisiopatologia , Doença de Parkinson/complicações , Doença de Parkinson/fisiopatologia , Sono REM/fisiologia , Idoso , Queixo , Eletromiografia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/fisiopatologia , Polissonografia , Postura
16.
Dev Med Child Neurol ; 58(3): 292-7, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26426208

RESUMO

AIM: To explore whether health-related quality of life (HRQOL) can be predicted by pain, age, Gross Motor Function Classification System (GMFCS) level, and sex in children with cerebral palsy (CP) and whether different pain etiologies have varying effects on HRQOL. METHODS: Children with CP aged 3 to 19 years and their caregivers were consecutively recruited. Caregivers reported their child's pain (Health Utilities Index 3 [HUI3] pain subset) and HRQOL (DISABKIDS questionnaires). Physicians identified pain etiologies. A multiple linear regression model determined whether pain, GMFCS level, sex, and age predicted HRQOL. An ANOVA evaluated the effects of pain etiologies on HRQOL. RESULTS: Three hundred and forty-four participants were approached and 87% (n=300) participated. Sufficient data were available on 248 (72% of total sample). Sixty-six participants (27%) formed the pain group with HUI3 pain scores of at least 3. The presence of pain and increasing age significantly negatively predicted HRQOL (p<0.001, R(2) =0.141), while GMFCS and sex did not. Musculoskeletal deformity (24%) and hypertonia (18%) were the most frequent pain causes. HRQOL statistically differed depending on the pain etiology (p=0.028) with musculoskeletal deformity showing the lowest mean HRQOL. INTERPRETATION: The presence of pain and increasing age negatively predict HRQOL in CP. musculoskeletal deformity has the greatest negative impact on HRQOL.


Assuntos
Paralisia Cerebral , Destreza Motora/classificação , Dor , Qualidade de Vida , Adolescente , Adulto , Fatores Etários , Paralisia Cerebral/complicações , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Hipertonia Muscular/complicações , Anormalidades Musculoesqueléticas/complicações , Dor/etiologia , Medição da Dor , Índice de Gravidade de Doença , Fatores Sexuais , Adulto Jovem
17.
Biomed Res Int ; 2015: 317047, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25649546

RESUMO

Spasticity is considered an important neural contributor to muscle hypertonia in children with cerebral palsy (CP). It is most often treated with antispasticity medication, such as Botulinum Toxin-A. However, treatment response is highly variable. Part of this variability may be due to the inability of clinical tests to differentiate between the neural (e.g., spasticity) and nonneural (e.g., soft tissue properties) contributions to hypertonia, leading to the terms "spasticity" and "hypertonia" often being used interchangeably. Recent advancements in instrumented spasticity assessments offer objective measurement methods for distinction and quantification of hypertonia components. These methods can be applied in clinical settings and their results used to fine-tune and improve treatment. We reviewed current advancements and new insights with respect to quantifying spasticity and its contribution to muscle hypertonia in children with CP. First, we revisit what is known about spasticity in children with CP, including the various definitions and its pathophysiology. Second, we summarize the state of the art on instrumented spasticity assessment in CP and review the parameters developed to quantify the neural and nonneural components of hypertonia. Lastly, the impact these quantitative parameters have on clinical decision-making is considered and recommendations for future clinical and research investigations are discussed.


Assuntos
Paralisia Cerebral/complicações , Hipertonia Muscular/complicações , Espasticidade Muscular/complicações , Animais , Paralisia Cerebral/fisiopatologia , Humanos , Hipertonia Muscular/fisiopatologia , Espasticidade Muscular/fisiopatologia , Neurônios/patologia
18.
Phys Med Rehabil Clin N Am ; 26(1): 69-78, 2015 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-25479780

RESUMO

Tone management is one of the primary roles of a pediatric physiatrist. Hypertonicity frequently inhibits normal movement patterns in children with central nervous system lesions but at times can reinforce muscle group firing and be useful for a child's function. Treatment approaches should be individualized based on functional goals, degree of impairment, interference with care, and type and location of hypertonicity. Treatment plans should be created in collaboration with all individuals caring for the child. There are many causes of hypertonicity as well as many nonsurgical and surgical treatments. Historical and current evidence-based treatments are reviewed.


Assuntos
Hipertonia Muscular/terapia , Procedimentos Neurocirúrgicos , Criança , Humanos , Hipertonia Muscular/complicações , Hipertonia Muscular/tratamento farmacológico , Espasticidade Muscular/tratamento farmacológico , Espasticidade Muscular/etiologia , Bloqueio Nervoso , Procedimentos Ortopédicos , Modalidades de Fisioterapia
19.
Rev. esp. investig. quir ; 18(1): 12-20, 2015. tab, graf
Artigo em Espanhol | IBECS | ID: ibc-137250

RESUMO

Introducción: Existe suficiente evidencia sobre el tratamiento de la fisura anal crónica con el gel de Diltiazem 2% (esfinterotomía química). Sin embargo, hay pocos estudios en los que se compare este tratamiento con la esfinterotomía quirúrgica (esfinterotomía lateral interna), a corto y largo plazo. Objetivo: El propósito de este estudio es comparar los resultados a corto y largo plazo, en cuanto a eficacia, recidiva, efectos adversos, complicaciones y coste, de la esfinterotomía lateral interna y el gel de diltiazem 2% en el tratamiento de la fisura anal crónica, en dos poblaciones homogéneas. Pacientes y Método: Se realizó un estudio observacional de todos los pacientes diagnosticados de fisura anal crónica, que cumplían los criterios de inclusión y exclusión, en nuestro hospital, durante el periodo comprendido entre el 1 de enero de 2008 y el 31 de diciembre de 2013 (6 años). A todos los pacientes se les propuso de inicio tratamiento médico, estableciéndose dos grupos. Un primer grupo tratado farmacológicamente y un segundo grupo quirúrgicamente. A los pacientes del primer grupo, se les indicó el gel de Diltiazem al 2%, tres aplicaciones al día durante ocho semanas como máximo. Ante la persistencia de la fisura a pesar de las ocho semanas de tratamiento, recidiva o abandono por efecto adverso, se indicó la esfinterotomía. A los pacientes resistentes al tratamiento médico de inicio, se les realizó tratamiento quirúrgico. Se establecieron controles presenciales a las 4 y 8 semanas del tratamiento. Posteriormente se llevó a cabo una encuesta telefónica anual o presencial siempre que fuese necesario. Se compararon los grupos mediante el test de la X2 para variables cualitativas y la t de Student para variables cuantitativas. Resultados: Se analizan 265 pacientes (137 mujeres) del grupo del Diltiazem con una media de edad de 46,7 (18-65) años, varianza de 184,2 y desviación típica 13,6. Localización de la fisura: posterior 96%, anterior 3% y lateral 1%. Todos los pacientes tenían dolor (100%), 231(80,65) sangrado y 198 (74,7%) escozor y prurito. Antecedentes de multiparidad en el51,3% y de estreñimiento en el 79,3% de los pacientes. Duración media de los síntomas 17,5 (5-60) meses. De los 265 pacientes tratados, 174 (65,7%) pacientes curaron a las 8 semanas de tratamiento. Treinta y un (11,7%) pacientes experimenta-ron algún efecto adverso relacionado con el tratamiento, de los que 7 (2,6%) abandonaron la terapia por esta causa. Se excluyeron del estudio a 46 pacientes por pérdida en el seguimiento. Tras una media de seguimiento de 31 (8-72) meses, se detectaron 43 (16,2%) recurrencias. No se detectó ningún caso de incontinencia. El tiempo medio de recurrencia tras finalizar el tratamiento fue de 5,7 (2,5-32) meses. La mayoría de las recurrencias (81,4%) ocurrieron antes de los 12 meses de iniciado el tratamiento. Los 174 pacientes curados precisaron una media de 1,4 (1-5) dispensaciones del gel de Diltiazem 2 %, con un coste medio por paciente de 40,2 (28,7-143,5) euros. En el grupo quirúrgico, se analizan 176 pacientes (81mujeres), edad media 46 (19-65) años, varianza 156,96, y desviación típica 12,5. Localización de la fisura: posterior, 95%, anterior 4,5% y lateral 0,5%. Todos los pacientes tenían dolor (100%), sangrado (80,6%) y escozor y prurito (74,7%). Antecedentes de multiparidad en 48,8% y de estreñimiento en el 38%, de los pacientes. Duración media de los síntomas 19,7 (1-72) meses. Estancia media un día y rango 0-6 días. De los 176 pacientes tratados, 152 (96,2%) pacientes curaron, la mayoría lo hicieron a las 3 (2-6) semanas de tratamiento. Quince (9,9%) pacientes experimentaron alguna complicación relacionada con la esfinterotomía, entre ellas 6(3,9%) casos de incontinencia reversible. Se excluyeron del estudio a 18 pacientes por pérdida en el seguimiento. Tras una media de seguimiento de34,3 (10-72) meses, se detectaron 12 (8,3%) recurrencias. El tiempo medio de recurrencia tras finalizar el tratamiento fue de 18,2 (2-60) meses. La mayoría (75%) de las recidivas se presentaron en los dos primeros años. El coste medio por proceso fue de 1.838,5 (1.711-1.966) euros. No hubo diferencias significativas (p>0,05), entre los grupos en relación a la edad, sexo, localización de la fisura, síntomas, duración media de los síntomas y enfermedad asociada. Se encontró diferencia estadísticamente significativa (p < 0,05) entre los grupos en cuanto al tiempo de respuesta, efectividad del tratamiento, duración de la respuesta, recidiva, tiempo de recidiva, presencia de incontinencia y coste. Conclusión: El tratamiento de la fisura anal crónica con Diltiazem gel 2%, es un tratamiento eficaz, seguro, fácil de administrar, reversible, económico y con efectos adversos leves, sin embargo la efectividad es menor y mayor la recaída, con res-pecto a la esfinterotomía quirúrgica, pero esta se ve gravada por la posibilidad de alteraciones en la continencia y un mayor coste


Introduction: There is sufficient evidence on the treatment of chronic anal fissure with diltiazem 2% gel (sphincterotomy chemistry), however there are few studies that compare this treatment with surgical sphincterotomy (lateral internal sphincterotomy), short and long-term outcomes. Objective: The purpose of this study is to compare short and long-term results in terms of efficacy, recurrence, adverse effects, complications and costs, between sphincterotomy and diltiazem 2% gel in the treatment of chronic anal fissure, in two homogeneous populations. Patients and Method: We studied patients diagnosed of chronic anal fissure in our hospital which met the criteria for inclusion and exclusion, during the period January 2008 to December 2013 (6 years). Two groups, the first with farmacological treatment and the second group with surgical treatment were established. Patients in the first group we used a formulation of Diltiazem gel 2%, in three daily applications for eight weeks. In case of inefficiency, recurrence or abandonment of treatment due to adverse effect a sphincterotomy was indicated. The second group consisted of patients treated with surgical sphincterotomy. Controls at 4 and 8 weeks of treatment were established. Subsequently an annually telephone survey was conducted or face control if considered necessary. Groups were compared using the X2 test for qualitative variables and the Student test for quantitative variables. Results: We analyzed 265 patients (137 women) in the Diltiazem group with a mean age of 46.7 (18-65) years, with 184.2 variance and standard deviation 13.6. Location of the fissure: posterior 96%, anterior 3% and lateral 1%. All patients had pain, 231 bleeding and 198 stinging and itching. Average duration of symptoms 17.5 (5-60) months. Of the 265 patients,174 (65.7%) patients were cured after 8 weeks of treatment. Thirty-one (11.7%) patients experienced adverse effects relatedto treatment, of which 7 (2.6%) discontinued therapy because of this. 46 patients were excluded, because they couldn ́t becontacted for follow-up (lost patients). After a mean follow up of 31 (8-72) months, 43 recurrences were detected (16.2%). No cases of incontinence were detected. The median time to recurrence after treatment was 5.7 (2.5 to 32) months. Most recurrences (81.4%) occurred in the first 12 months following treatment. The 174 cured patients required an average of 1.4 (1-5) dispensations of Diltiazem 2 % gel, with an average cost per patient of 40.2 (28.7 to 143.5) euros.In the surgical group, we analized 176 patients (81mujeres), mean age 46 (19-65) years. Location of the fissure: posterior95%, anterior 4.5% and lateral 0.5%. All patients had pain, 80,6% bleeding and 74,7% stinging and itching. Average dura-tion of symptoms 19.7 (1-72) months. Average hospital stay one day and range 0-6 days. Of the 176 patients, 152 (96.2%) patients were cured, most of them after 3 (2-6) weeks of treatment. Fifteen (9.9%) patients experienced complications rela-ted to sphincterotomy, among which, 6 (3.9%) cases of reversible incontinence we observed . 18 patients were excluded,because they couldn ́t be contacted for follow-up (lost patients). After a mean follow up of 34.3 (10-72) months, 12 (8.3%) recurrences were detected. The median time of recurrence after treatment was 18.2 (2-60) months. Most (75%) recurrences occurred in the first twoyears.The average cost per process was 1838.5 (1711-1966) euros. There were no significant differences (p > 0.05) between groupsrelated to age, sex, location of the fissure, symptoms, mean duration of symptoms and associated disease. Statistically significant difference (p < 0.05) was found between the groups in fairy effectiveness of treatment, response time, recurrence, timeto recurrence, incontinence and cost.Conclusion.The treatment of chronic anal fissure with diltiazem 2% gel is an effective, safe, easy to administer, reversible,economic and with slight adverse effects, however effectiveness is lower and recurrence rate is higher, with respect to the sur-gical sphincterotomy, but this is burdened by the possibility of alterations in continence and a higher cost


Assuntos
Feminino , Humanos , Masculino , Esfinterotomia Endoscópica/métodos , Esfinterotomia Endoscópica/psicologia , Preparações Farmacêuticas/administração & dosagem , Fissura Anal/patologia , Cirurgia Geral/instrumentação , Cirurgia Geral/métodos , Incontinência Fecal/patologia , Hipertonia Muscular/complicações , Hipertonia Muscular/metabolismo , Esfinterotomia Endoscópica/classificação , Esfinterotomia Endoscópica , Preparações Farmacêuticas , Fissura Anal/complicações , Fissura Anal/enfermagem , Cirurgia Geral/classificação , Cirurgia Geral , Incontinência Fecal/diagnóstico , Hipertonia Muscular/enfermagem , Estudo Observacional
20.
Biomed Res Int ; 2014: 637450, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25309915

RESUMO

Since 1997, focused shock waves therapy (FSWT) has been reported to be useful in the treatment of muscle hypertonia and dystonia. More recently, also radial shock wave therapy (RSWT) has been successfully used to treat muscle hypertonia. The studies where FSWT and RSWT have been used to treat muscle hypertonia and dystonia are reviewed in this paper. The more consistent and long lasting results were obtained in the lower limb muscles of patients affected by cerebral palsy with both FSWT and RSWT and in the distal upper limb muscles of adult stroke patients using FSWT. The most probable mechanism of action is a direct effect of shock waves on muscle fibrosis and other nonreflex components of muscle hypertonia. However, we believe that up to now the biological effects of shock waves on muscle hypertonia and dystonia cannot be clearly separated from a placebo effect.


Assuntos
Distonia/complicações , Ondas de Choque de Alta Energia/uso terapêutico , Hipertonia Muscular/terapia , Paralisia Cerebral/complicações , Paralisia Cerebral/fisiopatologia , Paralisia Cerebral/terapia , Distonia/fisiopatologia , Distonia/terapia , Humanos , Hipertonia Muscular/complicações , Hipertonia Muscular/fisiopatologia , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/fisiopatologia , Acidente Vascular Cerebral/terapia
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