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1.
Nat Neurosci ; 23(3): 327-336, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-32066981

RESUMO

Parkinson's disease is a synucleinopathy that is characterized by motor dysfunction, death of midbrain dopaminergic neurons and accumulation of α-synuclein (α-Syn) aggregates. Evidence suggests that α-Syn aggregation can originate in peripheral tissues and progress to the brain via autonomic fibers. We tested this by inoculating the duodenal wall of mice with α-Syn preformed fibrils. Following inoculation, we observed gastrointestinal deficits and physiological changes to the enteric nervous system. Using the AAV-PHP.S capsid to target the lysosomal enzyme glucocerebrosidase for peripheral gene transfer, we found that α-Syn pathology is reduced due to the increased expression of this protein. Lastly, inoculation of α-Syn fibrils in aged mice, but not younger mice, resulted in progression of α-Syn histopathology to the midbrain and subsequent motor defects. Our results characterize peripheral synucleinopathy in prodromal Parkinson's disease and explore cellular mechanisms for the gut-to-brain progression of α-Syn pathology.


Assuntos
Encéfalo/patologia , Doenças do Sistema Digestório/patologia , Sinucleinopatias/metabolismo , Sinucleinopatias/patologia , Animais , Duodeno/patologia , Sistema Nervoso Entérico/patologia , Glucosilceramidase/biossíntese , Glucosilceramidase/genética , Mesencéfalo/patologia , Camundongos , Camundongos Endogâmicos C57BL , Transtornos dos Movimentos/etiologia , Transtornos dos Movimentos/patologia , Fibras Nervosas/patologia , Nociceptividade , Gânglio Nodoso/patologia
2.
PLoS One ; 15(1): e0226494, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-31978134

RESUMO

BACKGROUND: GBA mutation carriers with PD (PD-GBA) are at higher risk of cognitive decline, but there is limited data regarding whether there are differences in gait dysfunction between GBA mutation and non-mutation carriers with PD. OBJECTIVES/METHODS: The primary aim of this study was to use quantitative inertial sensor-based gait analysis to compare gait asymmetry in 17 PD-GBA subjects, 17 non-mutation carriers with PD, and 15 healthy control subjects using parameters that had gait laterality and were markers of bradykinesia, in particular arm swing velocity and arm swing range of motion and stride length. RESULTS: Arm swing velocity was more symmetric in PD-GBA subjects vs. non-mutation carriers in the OFF state (12.5 +/- 8.3 vs. 22.9 +/- 11.8%, respectively, p = 0.018). In the ON-medication state, non-mutation carriers with PD, but not PD-GBA subjects, exhibited arm swing velocity (16.8 +/- 8.6 vs. 22.9 +/- 11.8%, p = 0.006) and arm range of motion (26.7 +/- 16.3 vs. 33.4 +/- 18.6%, p = 0.02) that was more asymmetric compared with the OFF-medication state. CONCLUSIONS: In the OFF medication state, arm swing velocity asymmetry may be a useful parameter in helping to distinguish GBA mutation carriers with PD from non-mutation carriers.


Assuntos
Braço/fisiopatologia , Marcha/fisiologia , Glucosilceramidase/genética , Transtornos dos Movimentos/etiologia , Mutação , Doença de Parkinson/complicações , Estudos de Casos e Controles , Disfunção Cognitiva/diagnóstico , Disfunção Cognitiva/etiologia , Feminino , Heterozigoto , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos dos Movimentos/diagnóstico , Doença de Parkinson/genética , Amplitude de Movimento Articular
3.
BMC Neurol ; 19(1): 301, 2019 Nov 27.
Artigo em Inglês | MEDLINE | ID: mdl-31775676

RESUMO

BACKGROUND: McLeod syndrome is a rare X-linked recessive acanthocytosis associated with neurological manifestations including progressive chorea, cognitive impairment, psychiatric disturbances, seizures, and sensorimotor axonal polyneuropathy. However, no studies have investigated the functioning of central sensorimotor tracts in patients with McLeod syndrome. CASE PRESENTATION: A 66-year-old man had experienced slowly progressive chorea and gait disturbance due to lower limb muscle weakness since his early fifties. Blood examinations showed erythrocyte acanthocytosis and the reduction of Kell antigens in red blood cells. Brain magnetic resonance imaging showed atrophy of the bilateral caudate nuclei and putamen. The diagnosis of McLeod syndrome was confirmed by the presence of a mutation of the XK gene on the X chromosome. Somatosensory-evoked potential and transcranial magnetic stimulation studies demonstrated that the central sensory and motor conduction times were abnormally prolonged for the lower extremity but normal for the upper extremity. CONCLUSIONS: This is the first report of the involvement of the central sensorimotor tracts for the legs in a patient with McLeod syndrome. The clinical neurophysiological technique revealed the central sensorimotor tracts involvements clinically masked by neuropathy.


Assuntos
Neuroacantocitose/diagnóstico , Idoso , Atrofia , Humanos , Imagem por Ressonância Magnética , Masculino , Transtornos dos Movimentos/etiologia , Debilidade Muscular/etiologia , Mutação
4.
Medicine (Baltimore) ; 98(38): e17249, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31567992

RESUMO

RATIONALE: Diabetic striatopathy (DS) is an uncommon movement disorder among diabetic patients characterized by clinical hemichorea-hemiballism with neuroimage change of the striatum. Here, we report a case of DS with relapsed hemichorea-hemiballism attacks even during euglycemic period, and the MRI changes by volumetric analysis. PATIENT CONCERNS: A 69-year-old diabetic female suffered from a relapsed episode of hemichorea-hemiballism during her euglycemic period after the treatment of hyperglycemia. DIAGNOSES: To investigate the serial MRI changes in a case with diabetic striatopathy who had clinical hemichorea-hemiballism syndrome. INTERVENTIONS: Semi-quantitative volumetric analyses from T1 images of these brain MRIs were obtained during the disease course. OUTCOMES: Besides, the negative finding of the first brain MRI during her first hospital admission, three afterward MRI examinations disclosed a waxing-and-waning mode of volume change from high-signal T1 images in left striatum. The clinical symptoms paralleled with the neuroimage changes in striatum. The MR signal volume changes were valuable for the clinical course of the hemichorea-hemiballism caused by diabetic striatopathy LESSONS:: Serial MR images for the diabetic striatopathy presented as a key pathognomonic relationship with the clinical hemichorea-hemiballism syndrome, assessed by our simplied volumetric analysis. Clinical involuntary movements may relapse and persist even with euglycemic condition as our case.


Assuntos
Corpo Estriado/diagnóstico por imagem , Complicações do Diabetes/diagnóstico por imagem , Transtornos dos Movimentos/etiologia , Idoso , Corpo Estriado/fisiopatologia , Complicações do Diabetes/diagnóstico , Complicações do Diabetes/fisiopatologia , Feminino , Humanos , Imagem por Ressonância Magnética , Transtornos dos Movimentos/diagnóstico por imagem , Transtornos dos Movimentos/fisiopatologia , Neuroimagem , Síndrome
5.
BMC Neurol ; 19(1): 168, 2019 Jul 18.
Artigo em Inglês | MEDLINE | ID: mdl-31319800

RESUMO

BACKGROUND: The coexistence of distinct neurodegenerative diseases in single cases has recently attracted greater attention. The phenotypic co-occurrence of progressive supranuclear palsy (PSP) and amyotrophic lateral sclerosis (ALS) has been documented in several cases. That said, the clinicopathological comorbidity of these two diseases has not been demonstrated. CASE PRESENTATION: A 77-year-old man presented with gait disturbance for 2 years, consistent with PSP with progressive gait freezing. At 79 years old, he developed muscle weakness compatible with ALS. The disease duration was 5 years after the onset of PSP and 5 months after the onset of ALS. Neuropathological findings demonstrated the coexistence of PSP and ALS. Immunohistochemical examination confirmed 4-repeat tauopathy, including globose-type neurofibrillary tangles, tufted astrocytes, and oligodendroglial coiled bodies as well as TAR DNA-binding protein 43 kDa pathology in association with upper and lower motor neuron degeneration. Immunoblotting showed hyperphosphorylated full-length 4-repeat tau bands (64 and 68 kDa) and C-terminal fragments (33 kDa), supporting the diagnosis of PSP and excluding other parkinsonian disorders, such as corticobasal degeneration. Genetic studies showed no abnormalities in genes currently known to be related to ALS or PSP. CONCLUSIONS: Our case demonstrates the clinicopathological comorbidity of PSP and ALS in a sporadic patient. The possibility of multiple proteinopathies should be considered when distinct symptoms develop during the disease course.


Assuntos
Esclerose Amiotrófica Lateral/complicações , Encéfalo/patologia , Paralisia Supranuclear Progressiva/complicações , Idoso , Esclerose Amiotrófica Lateral/diagnóstico por imagem , Esclerose Amiotrófica Lateral/patologia , Astrócitos/patologia , Encéfalo/diagnóstico por imagem , Comorbidade , Proteínas de Ligação a DNA , Evolução Fatal , Humanos , Masculino , Transtornos dos Movimentos/etiologia , Emaranhados Neurofibrilares/patologia , Paralisia Supranuclear Progressiva/diagnóstico por imagem , Paralisia Supranuclear Progressiva/patologia , Proteínas tau/análise
6.
Emerg Med J ; 36(7): 415-422, 2019 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-31320334

RESUMO

CLINICAL INTRODUCTION: An 88-year-old woman presented with acute onset of involuntary limb movements for one day. Two days prior she had fallen a suffered a left hip contusion but no head trauma. There was no fever or difficulty breathing. Her heart rate was 72 bpm with blood pressure of 109/68 mm Hg. Physical examination revealed restricted left hip motion due to pain and a sustained twisted posture of the upper extremity without paresis. Glasgow Coma Scale was 15, and there was no evidence of Kernig's or Brudzinski's sign. She underwent a hip X-ray and non-contrast CT scan (figures 1 and 2).emermed;36/7/415/F1F1F1Figure 1Anteroposterior X-radiograph of the hip.emermed;36/7/415/F2F2F2Figure 2A non-contrast brain CT. QUESTION: What is the most likely cause of the clinical presentation?Acute meningitisCerebral fat embolismHaemorrhagic strokeHypertensive encephalopathy For answer see page 2 For question see page 1.


Assuntos
Embolia Gordurosa/complicações , Embolia Intracraniana/complicações , Perna (Membro)/anormalidades , Transtornos dos Movimentos/etiologia , Idoso de 80 Anos ou mais , Embolia Gordurosa/epidemiologia , Feminino , Humanos , Embolia Intracraniana/epidemiologia , Perna (Membro)/diagnóstico por imagem , Perna (Membro)/fisiopatologia , Imagem por Ressonância Magnética/métodos , Transtornos dos Movimentos/epidemiologia , Radiografia/métodos , Tomografia Computadorizada por Raios X/métodos
7.
Neurodiagn J ; 59(3): 133-141, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31233378

RESUMO

Awake craniotomy is advocated for the resection of supratentorial low-grade gliomas (LGG). The combination of neurophysiological electrical mapping techniques and performing the craniotomy awake has demonstrated increased total and supratotal resection of LGG, as well as increased overall survival rates. We present an illustrative case where the patient's gross motor function deteriorated during the resection of a LGG and mapping techniques using the phase reversal technique and Taniguchi direct cortical stimulation technique while the patient was awake proved to be valuable in determining continuity of the corticospinal tracts.


Assuntos
Mapeamento Encefálico/métodos , Craniotomia/métodos , Glioma/cirurgia , Monitorização Neurofisiológica Intraoperatória/métodos , Procedimentos Neurocirúrgicos/métodos , Neoplasias Supratentoriais/cirurgia , Estimulação Elétrica , Humanos , Masculino , Pessoa de Meia-Idade , Córtex Motor/cirurgia , Transtornos dos Movimentos/diagnóstico , Transtornos dos Movimentos/etiologia , Tratos Piramidais/fisiologia , Vigília
9.
J Integr Neurosci ; 18(1): 57-64, 2019 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-31091849

RESUMO

Spinal cord injury is a disabling disorder, leading to neurological impairments. Although some microRNAs have been reported to be associated with spinal cord injury, the function of microRNA-362-3p, as one of downregulated miRNAs after spinal cord injury, is still unclear. In current study, spinal cord injury models were established. Then, we performed microRNA-362-3p overexpression in spinal cord injury rats, which expressed the low microRNA-362-3p. Results from behavioral testing, hematoxylin and eosin staining and Nissl staining revealed that microRNA-362-3p over expresssion improved the functional resoration in spinal cord injury rats. Furthermore, it caused the decrease of neuronal apoptosis and inhibition of the neuronal inflammation in these rats. Besides, Paired box gene 2 was verified as a target gene of microRNA-362-3p using luciferase assay, which predicted via bioinformatics technology. Moreover, microRNA-362-3p alleviated the neuralgia and reduced the activation of ERK and p38 through inhibition of Paired box gene 2. In conclusion, the findings demonstrated that microRNA-362-3p attenuated neuropathic pain following spinal cord injury through targeting Paired box gene 2. It provides us the new biomarker to diagnose and monitor spinal cord injury.


Assuntos
MicroRNAs/fisiologia , Transtornos dos Movimentos/metabolismo , Neuralgia/metabolismo , Fator de Transcrição PAX2/metabolismo , Traumatismos da Medula Espinal/metabolismo , Animais , Apoptose/fisiologia , Modelos Animais de Doenças , MAP Quinases Reguladas por Sinal Extracelular/metabolismo , Inflamação/metabolismo , Inflamação/terapia , MicroRNAs/genética , Atividade Motora/fisiologia , Transtornos dos Movimentos/etiologia , Transtornos dos Movimentos/terapia , Neuralgia/etiologia , Neuralgia/terapia , Ratos Sprague-Dawley , Medula Espinal/metabolismo , Traumatismos da Medula Espinal/complicações , Traumatismos da Medula Espinal/terapia , Proteínas Quinases p38 Ativadas por Mitógeno/metabolismo
10.
Mol Genet Metab ; 127(1): 28-30, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30928149

RESUMO

About a third of patients with inherited metabolic diseases with neurologic involvement suffer from a movement disorder, in the form of ataxia, hyperkinetic movements, or hypokinetic-rigid syndrome. We reviewed and updated the list of known metabolic etiologies associated with various types of movement disorders, and found approximately 200 relevant inborn errors of metabolism. This represents the first of a series of articles attempting to create and maintain a comprehensive list of clinical and metabolic differential diagnoses according to system involvement.


Assuntos
Erros Inatos do Metabolismo/complicações , Transtornos dos Movimentos/diagnóstico , Transtornos dos Movimentos/etiologia , Ataxia/etiologia , Diagnóstico Diferencial , Distonia/etiologia , Humanos , Transtornos Parkinsonianos/etiologia
11.
Orv Hetil ; 160(17): 662-669, 2019 Apr.
Artigo em Húngaro | MEDLINE | ID: mdl-31010306

RESUMO

Introduction: The motor and non-motor complications of Parkinson's disease impair the patients' quality of life and limit therapeutical options. There are no clear criteria for 'advanced' Parkinson's disease or for the optimal moment for invasive therapies. There is little evidence regarding the upper limits of levodopa doses, and how these may be influenced by the availability of device-aided therapies. Aim: To analyze substitution therapy in patients with advanced Parkinson's disease. Method: In our retrospective study, we analyzed the data from all patients with advanced Parkinson's disease hospitalized between 1st June 2011 and 31st May 2017, receiving combined levodopa treatment at least 4×/day, reporting a minimum of 2 hours off periods, with or without dyskinesia. We analyzed levodopa therapy for patients who were recommended either device-aided or conservative therapy. Results: Out of 311 patients with advanced Parkinson's disease, for 125 we proposed device-aided therapies whereas in 42 patients we increased the levodopa dose. The average levodopa doses and the administration rate were higher for the 107 patients tested for levodopa-carbidopa intestinal gel. Disease duration, mean levodopa doses and frequency of dosing were all higher in patients proposed for device-aided therapies versus patients with continued conservative treatment. Conclusion: Our patients were on lower levodopa doses (compared to literature), but the combinations were used more often. Device-aided therapies should be considered in patients with severe motor complications who receive at least 750-1000 mg levodopa daily, divided minimum 5×/day. These patients need to be tested in specialized centers by multidisciplinary teams in order to make the best decision for further action. Orv Hetil. 2019; 160(17): 662-669.


Assuntos
Antiparkinsonianos/uso terapêutico , Carbidopa/administração & dosagem , Levodopa/uso terapêutico , Doença de Parkinson/tratamento farmacológico , Antiparkinsonianos/administração & dosagem , Carbidopa/uso terapêutico , Relação Dose-Resposta a Droga , Combinação de Medicamentos , Humanos , Transtornos dos Movimentos/tratamento farmacológico , Transtornos dos Movimentos/etiologia , Neurologia , Doença de Parkinson/complicações , Qualidade de Vida , Estudos Retrospectivos , Romênia , Resultado do Tratamento
12.
Br J Neurosurg ; 33(5): 514-521, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-30882248

RESUMO

Purpose: To assess whether the instrument handling and image quality of endoscopic spinal decompression procedures is adequate and effects the treatment of degenerative spinal disorders. Material and Methods: Forty-six patients underwent endoscopic procedures for radicular pain or sensorimotor deficit due to a degenerative disorder using a 15mm tubular retractor. Endoscopic video recordings were reviewed with focus on instrument handling and intraoperative complication. At final follow-up the clinical outcome was assessed via a standardized questionnaire including the Oswestry Disability Index (ODI) Neck Disability Index (NDI), Odoms criteria and a personal examination focusing on pain, and sensorimotor deficits. Results: Forty out of 46 patients attended a final follow-up (86.9%). The mean follow-up time was 51.8 month (range 15-84 month). At final follow-up, of patients who were operate at the lumbar spine 93.9% and at the cervical spine 85.7% were free of radicular pain, no weakness was documented in 84.9% of cases after lumbar and 85.7% after cervical spine procedure, and according to Odoms criteria clinical success was noted in 84.5% and 100%, respectively. The mean ODI was 9.0% and mean NDI was 11.7%. The dural tear rate was 4.3%, all dural tear were closed endoscopically. The recurrent disc herniation rate was 6.1%. Conclusions: Endoscopic decompression using a 15m tubular retractor offers a good view onto the surgical field and a high clinical success rate. The decompression of degenerative pathologies in bimanual technique is not limited by a 15mm tubular retractor.


Assuntos
Endoscopia/instrumentação , Degeneração do Disco Intervertebral/diagnóstico por imagem , Degeneração do Disco Intervertebral/cirurgia , Deslocamento do Disco Intervertebral/diagnóstico por imagem , Deslocamento do Disco Intervertebral/cirurgia , Coluna Vertebral/diagnóstico por imagem , Coluna Vertebral/cirurgia , Avaliação da Deficiência , Feminino , Seguimentos , Humanos , Degeneração do Disco Intervertebral/complicações , Deslocamento do Disco Intervertebral/complicações , Imagem por Ressonância Magnética , Masculino , Transtornos dos Movimentos/etiologia , Transtornos dos Movimentos/cirurgia , Dor/cirurgia , Estudos Retrospectivos , Transtornos das Sensações/etiologia , Transtornos das Sensações/cirurgia , Falha de Tratamento , Resultado do Tratamento
13.
Neurorehabil Neural Repair ; 33(4): 319-328, 2019 04.
Artigo em Inglês | MEDLINE | ID: mdl-30880592

RESUMO

BACKGROUND: Persistent deficits in arm function are common after stroke. An improved understanding of the factors that contribute to the performance of skilled arm movements is needed. One such factor may be self-efficacy (SE). OBJECTIVE: To determine the level of SE for skilled, goal-directed reach actions in individuals with mild motor impairment after stroke and whether SE for reach performance correlated with actual reach performance. METHODS: A total of 20 individuals with chronic stroke (months poststroke: mean 58.1 ± 38.8) and mild motor impairment (upper-extremity Fugl-Meyer [FM] motor score: mean 53.2, range 39 to 66) and 6 age-matched controls reached to targets presented in 2 directions (ipsilateral, contralateral). Prior to each block (24 reach trials), individuals rated their confidence on reaching to targets accurately and quickly on a scale that ranged from 0 ( not very confident) to 10 ( very confident). RESULTS: Overall reach performance was slower and less accurate in the more-affected arm compared with both the less-affected arm and controls. SE for both reach speed and reach accuracy was lower for the more-affected arm compared with the less-affected arm. For reaches with the more-affected arm, SE for reach speed and age significantly predicted movement time to ipsilateral targets ( R2 = 0.352), whereas SE for reach accuracy and FM motor score significantly predicted end point error to contralateral targets ( R2 = 0.291). CONCLUSIONS: SE relates to measures of reach control and may serve as a target for interventions to improve proximal arm control after stroke.


Assuntos
Transtornos dos Movimentos/fisiopatologia , Transtornos dos Movimentos/psicologia , Autoeficácia , Acidente Vascular Cerebral/complicações , Extremidade Superior , Fenômenos Biomecânicos , Avaliação da Deficiência , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos dos Movimentos/etiologia , Índice de Gravidade de Doença , Acidente Vascular Cerebral/fisiopatologia , Acidente Vascular Cerebral/psicologia , Reabilitação do Acidente Vascular Cerebral , Extremidade Superior/fisiopatologia
14.
Neurorehabil Neural Repair ; 33(2): 130-140, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30744527

RESUMO

BACKGROUND: Stroke is a leading cause of adult disability owing largely to motor impairment and loss of function. After stroke, there may be abnormalities in γ-aminobutyric acid (GABA)-mediated inhibitory function within primary motor cortex (M1), which may have implications for residual motor impairment and the potential for functional improvements at the chronic stage. OBJECTIVE: To quantify GABA neurotransmission and concentration within ipsilesional and contralesional M1 and determine if they relate to upper limb impairment and function at the chronic stage of stroke. METHODS: Twelve chronic stroke patients and 16 age-similar controls were recruited for the study. Upper limb impairment and function were assessed with the Fugl-Meyer Upper Extremity Scale and Action Research Arm Test. Threshold tracking paired-pulse transcranial magnetic stimulation protocols were used to examine short- and long-interval intracortical inhibition and late cortical disinhibition. Magnetic resonance spectroscopy was used to evaluate GABA concentration. RESULTS: Short-interval intracortical inhibition was similar between patients and controls ( P = .10). Long-interval intracortical inhibition was greater in ipsilesional M1 compared with controls ( P < .001). Patients who did not exhibit late cortical disinhibition in ipsilesional M1 were those with greater upper limb impairment and worse function ( P = .002 and P = .017). GABA concentration was lower within ipsilesional ( P = .009) and contralesional ( P = .021) M1 compared with controls, resulting in an elevated excitation-inhibition ratio for patients. CONCLUSION: These findings indicate that ipsilesional and contralesional M1 GABAergic inhibition are altered in this small cohort of chronic stroke patients. Further study is warranted to determine how M1 inhibitory networks might be targeted to improve motor function.


Assuntos
Córtex Motor/fisiopatologia , Transtornos dos Movimentos/fisiopatologia , Inibição Neural , Acidente Vascular Cerebral/fisiopatologia , Extremidade Superior/fisiopatologia , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Eletromiografia , Potencial Evocado Motor , Feminino , Humanos , Imagem por Ressonância Magnética , Espectroscopia de Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Córtex Motor/diagnóstico por imagem , Transtornos dos Movimentos/diagnóstico por imagem , Transtornos dos Movimentos/etiologia , Imagem Multimodal , Receptores de GABA-B/metabolismo , Acidente Vascular Cerebral/diagnóstico por imagem , Estimulação Magnética Transcraniana , Ácido gama-Aminobutírico/metabolismo
15.
Neurorehabil Neural Repair ; 33(2): 141-152, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-30744528

RESUMO

BACKGROUND: Spasticity is common in patients with stroke, yet current quantification methods are insufficient for determining the relationship between spasticity and voluntary movement deficits. This is partly a result of the effects of spasticity on spatiotemporal characteristics of movement and the variability of voluntary movement. These can be captured by Gaussian mixture models (GMMs). OBJECTIVES: To determine the influence of spasticity on upper-limb voluntary motion, as assessed by the bidirectional Kullback-Liebler divergence (BKLD) between motion GMMs. METHODS: A total of 16 individuals with subacute stroke and 13 healthy aged-equivalent controls reached to grasp 4 targets (near-center, contralateral, far-center, and ipsilateral). Two-dimensional GMMs (angle and time) were estimated for elbow extension motion. BKLD was computed for each individual and target, within the control group and between the control and stroke groups. Movement time, final elbow angle, average elbow velocity, and velocity smoothness were computed. RESULTS: Between-group BKLDs were much larger than within control-group BKLDs. Between-group BKLDs for the near-center target were lower than those for the far-center and contralateral targets, but similar to that for the ipsilateral target. For those with stroke, the final angle was lower for the near-center target, and the average velocity was higher. Velocity smoothness was lower for the near-center than for the ipsilateral target. Elbow flexor and extensor passive muscle resistance (Modified Ashworth Scale) strongly explained BKLD values. CONCLUSIONS: Results support the view that individuals with poststroke spasticity have a velocity-dependent reduction in active elbow joint range and that BKLD can be used as an objective measure of the effects of spasticity on reaching kinematics.


Assuntos
Modelos Teóricos , Transtornos dos Movimentos , Espasticidade Muscular , Acidente Vascular Cerebral , Extremidade Superior , Adulto , Idoso , Fenômenos Biomecânicos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos dos Movimentos/etiologia , Transtornos dos Movimentos/fisiopatologia , Espasticidade Muscular/etiologia , Espasticidade Muscular/fisiopatologia , Análise Espaço-Temporal , Processos Estocásticos , Acidente Vascular Cerebral/complicações , Acidente Vascular Cerebral/fisiopatologia , Extremidade Superior/fisiopatologia
16.
Curr Neurol Neurosci Rep ; 19(2): 7, 2019 02 09.
Artigo em Inglês | MEDLINE | ID: mdl-30739241

RESUMO

PURPOSE OF REVIEW: We provide a review of the movement disorders that complicate selected metabolic disorders, including the abnormal movements that may appear during or after their treatment. RECENT FINDINGS: Movement disorders may be underrecognized when arising in the context of a broad range of metabolic disorders. Abnormal movements may occur as the initial manifestation of a systemic disease, at any time during its course, or as a result of the medical interventions required for its management. Ascertaining movement phenomenology in acute and subacute presentations may assist in the determination of the specific underlying metabolic disorder. The management of movement disorders associated with metabolic disorders depends on the underlying pathophysiology.


Assuntos
Doenças Metabólicas/complicações , Transtornos dos Movimentos/etiologia , Discinesias , Humanos
17.
Gait Posture ; 69: 156-161, 2019 03.
Artigo em Inglês | MEDLINE | ID: mdl-30731319

RESUMO

BACKGROUND: Slipped capital femoral epiphysis (SCFE) is a common disorder in adolescent for which no consensus exists regarding management. The aim of the present study was to analyze gait modifications following SCFE treated with in situ fixation (ISF) and to relate it to radiologic stage. RESEARCH QUESTION: To verify if gait biomechanics are impaired in patients with SCFE and to try to determine a degree of slippage from which gait modifications would appear. METHODS: We evaluated 16 patients treated by ISF for SCFE with slippage ranging from 11° to 61°. Gait variables were compared to normal population according to age and walking speed and were normalized in Z-scores. RESULTS: Spatiotemporal parameters, mechanical and energetic variables were inferior to |1.5| Z-scores and considered as normal. Kinematics showed increase of pelvic tilt and hip adduction. Kinetic variables showed modifications with increased hip extension moment. There was also a strong increase in power of hip extensor. Hip extension moment and power of hip extensors were significantly correlated to radiologic stage. Analysis of ROC curves showed a cut-off value of slippage about 25°-30° affecting kinematics of pelvis and hip and kinetic variables. CONCLUSION: The gait variables were close to normal values. Main modifications were observed in kinematic and kinetic data with a significant increase in extension moment and power generated at the operated hip. This could participate to long-term joint degradation observed in SCFE, even in mild slips. The clinical message is to control regularly SCFE with initial slippage greater than >25-30° to allow for early diagnosis of premature hip osteoarthritis.


Assuntos
Marcha , Transtornos dos Movimentos/etiologia , Procedimentos Ortopédicos , Complicações Pós-Operatórias , Escorregamento das Epífises Proximais do Fêmur/fisiopatologia , Escorregamento das Epífises Proximais do Fêmur/cirurgia , Adolescente , Fenômenos Biomecânicos , Criança , Feminino , Seguimentos , Articulação do Quadril/fisiopatologia , Articulação do Quadril/cirurgia , Humanos , Cinética , Masculino , Transtornos dos Movimentos/diagnóstico , Transtornos dos Movimentos/fisiopatologia , Osteoartrite do Quadril/diagnóstico , Osteoartrite do Quadril/etiologia , Osteoartrite do Quadril/prevenção & controle , Complicações Pós-Operatórias/diagnóstico , Complicações Pós-Operatórias/fisiopatologia , Amplitude de Movimento Articular , Estudos Retrospectivos , Resultado do Tratamento
18.
Neurobiol Dis ; 127: 32-44, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30798005

RESUMO

Functional movement disorders (FMDs), known over time as "hysteria", "dissociative", "conversion", "somatoform", "non-organic" and "psychogenic" disorders, are characterized by having a voluntary quality, being modifiable by attention and distraction but perceived by the patient as involuntary. Although a high prevalence of depression and anxiety is observed in these patients, a definitive role of psychiatric disorders in FMDs has not been proven, and many patients do not endorse such manifestations. Stressful events, social influences and minor trauma may precede the onset of FMDs, but their pathogenic mechanisms are unclear. Patients with FMDs have several abnormalities in their neurobiology including strengthened connectivity between the limbic and motor networks. Additionally, there is altered top-down regulation of motor activities and increased activation of areas implicated in self-awareness, self-monitoring, and active motor inhibition such as the cingulate and insular cortex. Decreased activation of the supplementary motor area (SMA) and pre-SMA, implicated in motor control and preparation, is another finding. The sense of agency defined as the feeling of controlling external events through one's own action also seems to be impaired in individuals with FMDs. Correlating with this is a loss of intentional binding, a subjective time compression between intentional action and its sensory consequences. Organic and functional dystonia may be difficult to differentiate since they share diverse neurophysiological features including decreased cortical inhibition, and similar local field potentials in the globus pallidus and thalamus; although increased cortical plasticity is observed only in patients with organic dystonia. Advances in the pathogenesis and pathophysiology of FMDs may be helpful to understand the nature of these disorders and plan further treatment strategies.


Assuntos
Distúrbios Distônicos/etiologia , Córtex Motor/fisiopatologia , Transtornos dos Movimentos/etiologia , Distúrbios Distônicos/fisiopatologia , Humanos , Transtornos dos Movimentos/fisiopatologia
20.
Neurol Sci ; 40(5): 1067-1070, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30645749

RESUMO

OBJECTIVE: Diencephalon is frequently affected in multiple sclerosis (MS), and lesions of this region are associated with increased disability. Orexin-A and melatonin, two foremost mediators of diencephalon, modulate cognitive and motor functions through several pathways including the brain-derived neurotrophic factor (BDNF)-cAMP response element-binding protein (CREB) signaling pathway. In this pilot study, our aim was to investigate the prognostic value of these factors in progression of cognitive and physical disability. METHODS: Levels of BDNF, melatonin, CREB, and orexin-A were determined by ELISA in sera of 25 relapsing remitting MS (RRMS) patients, 15 secondary progressive MS (SPMS) patients, and 20 healthy controls. Cognitive and motor functions were assessed by a neuropsychological test battery, timed 25-ft walk (T25-FW) and 9-hole peg (9-HP) tests. RESULTS: MS patients had significantly lower serum levels of orexin-A and BDNF than healthy controls, and SPMS patients had significantly lower levels of melatonin and orexin-A than RRMS patients. Serum orexin-A levels were negatively correlated with 9-HP, T25-FW test scores, and progression index in RRMS patients. BDNF, CREB, and melatonin levels did not show any significant correlation with clinical features including EDSS and cognitive/motor performance of the patients. CONCLUSION: Our results suggest that orexin-A levels are decreased in parallel to disease progression and motor system deterioration in the earlier stages of the disease. Thus, orexin-A might be used as a potential biomarker of physical disability.


Assuntos
Transtornos dos Movimentos/sangue , Esclerose Múltipla Crônica Progressiva/sangue , Esclerose Múltipla Recidivante-Remitente/sangue , Orexinas/sangue , Adulto , Biomarcadores/sangue , Estudos de Coortes , Avaliação da Deficiência , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos dos Movimentos/etiologia , Esclerose Múltipla Crônica Progressiva/complicações , Esclerose Múltipla Recidivante-Remitente/complicações , Testes Neuropsicológicos , Projetos Piloto
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