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1.
Artigo em Inglês | MEDLINE | ID: mdl-38899557

RESUMO

BACKGROUND: Parkinson's Disease (PD) is a progressive neurological disorder that results in potentially debilitating mobility deficits. Recently, spinal cord stimulation (SCS) has been proposed as a novel therapy for PD gait disorders. The highest levels of evidence remain limited for SCS. OBJECTIVES: In this systematic review and narrative synthesis, the literature was searched using combinations of key phrases indicating spinal cord stimulation and PD. METHODS: We included pre-clinical studies and all published clinical trials, case reports, conference abstracts as well as protocols for ongoing clinical trials. Additionally, we included trials of SCS applied to atypical parkinsonism. RESULTS: A total of 45 human studies and trials met the inclusion criteria. Based on the narrative synthesis, a number of knowledge gaps and future avenues of potential research were identified. This review demonstrated that evidence for SCS is currently not sufficient to recommend it as an evidence-based therapy for PD related gait disorders. There remain challenges and significant barriers to widespread implementation, including issues regarding patient selection, effective outcome selection, stimulation location and mode, and in programming parameter optimization. Results of early randomized controlled trials are currently pending. SCS is prone to placebo, lessebo and nocebo as well as blinding effects which may impact interpretation of outcomes, particularly when studies are underpowered. CONCLUSION: Therapies such as SCS may build on current evidence and be shown to improve specific gait features in PD. Early negative trials should be interpreted with caution, as more evidence will be required to develop effective methodologies in order to drive clinical outcomes.

2.
Sensors (Basel) ; 24(10)2024 May 13.
Artigo em Inglês | MEDLINE | ID: mdl-38793945

RESUMO

The progress in markerless technologies is providing clinicians with tools to shorten the time of assessment rapidly, but raises questions about the potential trade-off in accuracy compared to traditional marker-based systems. This study evaluated the OpenCap system against a traditional marker-based system-Vicon. Our focus was on its performance in capturing walking both toward and away from two iPhone cameras in the same setting, which allowed capturing the Timed Up and Go (TUG) test. The performance of the OpenCap system was compared to that of a standard marker-based system by comparing spatial-temporal and kinematic parameters in 10 participants. The study focused on identifying potential discrepancies in accuracy and comparing results using correlation analysis. Case examples further explored our results. The OpenCap system demonstrated good accuracy in spatial-temporal parameters but faced challenges in accurately capturing kinematic parameters, especially in the walking direction facing away from the cameras. Notably, the two walking directions observed significant differences in pelvic obliquity, hip abduction, and ankle flexion. Our findings suggest areas for improvement in markerless technologies, highlighting their potential in clinical settings.


Assuntos
Análise da Marcha , Marcha , Smartphone , Caminhada , Humanos , Projetos Piloto , Análise da Marcha/métodos , Análise da Marcha/instrumentação , Masculino , Fenômenos Biomecânicos/fisiologia , Feminino , Marcha/fisiologia , Caminhada/fisiologia , Adulto
3.
Medicina (Kaunas) ; 59(4)2023 Apr 06.
Artigo em Inglês | MEDLINE | ID: mdl-37109677

RESUMO

The differential diagnosis of idiopathic normal pressure hydrocephalus (iNPH) and progressive supranuclear palsy (PSP) is difficult. The importance of proper diagnosis is particularly important for iNPH, which can be effectively treated with a ventriculoperitoneal (VP) shunt. In our case report, we present a unique case of a patient with overlapping symptoms and radiological findings of iNPH and PSP. Our patient underwent the VP shunt after a differential diagnostic evaluation which resulted in significant improvement in their clinical condition and quality of life, albeit for a short time.


Assuntos
Hidrocefalia de Pressão Normal , Paralisia Supranuclear Progressiva , Humanos , Paralisia Supranuclear Progressiva/complicações , Paralisia Supranuclear Progressiva/diagnóstico , Hidrocefalia de Pressão Normal/diagnóstico , Hidrocefalia de Pressão Normal/diagnóstico por imagem , Qualidade de Vida , Síndrome , Derivação Ventriculoperitoneal
4.
Front Endocrinol (Lausanne) ; 14: 1137048, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37033257

RESUMO

Objective: Strong evidence supports the benefits of exercise for healthy ageing, including reduced risk of neurodegenerative diseases. Recent studies suggested interorgan crosstalk as a key element of systemic adaptive response, however, the role of specific molecules in mediating exercise effects on the human brain are not fully understood. In the present study, we explored the exercise-related regulation of Growth Differentiation Factor 11 (GDF11) in cerebrospinal fluid (CSF) and blood. Methods: The samples of serum, plasma and CSF were obtained before and 60min after acute exercise (90min run) from twenty healthy young individuals. Additional serum and plasma samples were collected immediately after run. GDF11 protein content (immunoblotting), body composition (bioelectrical impedance), physical fitness (VO2max, cycle spiroergometry) and cognitive functions (standardized computerized tests, Cogstate) were evaluated. Results: Running decreased GDF11 protein content in CSF (-20.6%. p=0.046), while GDF11 in plasma and serum were not regulated. Two GDF11-specific antibodies of different origin were used to corroborate this result. Individuals with higher physical fitness displayed greater exercise-induced decrease of GDF11 in CSF than those with lower physical fitness (p=0.025). VO2max correlated positively with GDF11 in serum (r=0.63, p=0.020) as well as with the exercise-induced change in GDF11 levels in CSF (r=0.59, p=0.042). Indirect measure of blood-brain barrier permeability (i.e. CSF/serum albumin ratio) tended to positively correlate with CSF/serum GDF11 ratio (p=0.060). CSF levels of GDF11 correlated positively with cognitive functions, including working memory, both before and after run (p<0.05). Conclusion: Running-induced down-regulation of the GDF11 protein in the cerebrospinal fluid of healthy young individuals indicates the potential role of GDF11 in the exercise-induced cross-talk between periphery and the brain.


Assuntos
Exercício Físico , Fatores de Diferenciação de Crescimento , Corrida , Humanos , Adulto Jovem , Proteínas Morfogenéticas Ósseas , Exercício Físico/fisiologia , Fatores de Diferenciação de Crescimento/líquido cefalorraquidiano , Aptidão Física , Corrida/fisiologia
5.
Neurotherapeutics ; 20(4): 1109-1119, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37097344

RESUMO

Essential tremor (ET) is a disabling condition resulting from a dysfunction of cerebello-thalamo-cortical circuitry. Deep brain stimulation (DBS) or lesion of the ventral-intermediate thalamic nucleus (VIM) is an effective treatment for severe ET. Transcranial cerebellar brain stimulation has recently emerged as a non-invasive potential therapeutic option. Here, we aim to investigate the effects of high-frequency non-invasive cerebellar transcranial alternating current stimulation (tACS) in severe ET patients already operated for VIM-DBS. Eleven ET patients with VIM-DBS, and 10 ET patients without VIM-DBS and matched for tremor severity, were included in this double-blind proof-of-concept controlled study. All patients received unilateral cerebellar sham-tACS and active-tACS for 10 min. Tremor severity was blindly assessed at baseline, without VIM-DBS, during sham-tACS, during and at 0, 20, 40 min after active-tACS, using kinetic recordings during holding posture and action ('nose-to-target') task and videorecorded Fahn-Tolosa-Marin (FTM) clinical scales. In the VIM-DBS group, active-tACS significantly improved both postural and action tremor amplitude and clinical (FTM scales) severity, relative to baseline, whereas sham-tACS did not, with a predominant effect for the ipsilateral arm. Tremor amplitude and clinical severity were also not significantly different between ON VIM-DBS and active-tACS conditions. In the non-VIM-DBS group, we also observed significant improvements in ipsilateral action tremor amplitude, and clinical severity after cerebellar active-tACS, with a trend for improved postural tremor amplitude. In non-VIM-DBS group, sham- active-tACS also decreased clinical scores. These data support the safety and potential efficacy of high-frequency cerebellar-tACS to reduce ET amplitude and severity.


Assuntos
Estimulação Encefálica Profunda , Tremor Essencial , Estimulação Transcraniana por Corrente Contínua , Humanos , Estimulação Encefálica Profunda/métodos , Tremor Essencial/terapia , Tálamo , Resultado do Tratamento , Tremor/terapia , Método Duplo-Cego
6.
Front Aging Neurosci ; 15: 1117473, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36967818

RESUMO

Parkinson's disease (PD) affects the language processes, with a significant impact on the patients' daily communication. We aimed to describe specific alterations in the comprehension of syntactically complex sentences in patients with PD (PwPD) as compared to healthy controls (HC) and to identify the neural underpinnings of these deficits using a functional connectivity analysis of the striatum. A total of 20 patients PwPD and 15 HC participated in the fMRI study. We analyzed their performance of a Test of sentence comprehension (ToSC) adjusted for fMRI. A task-dependent functional connectivity analysis of the striatum was conducted using the psychophysiological interaction method (PPI). On the behavioral level, the PwPD scored significantly lower (mean ± sd: 77.3 ± 12.6) in the total ToSC score than the HC did (mean ± sd: 86.6 ± 8.0), p = 0.02, and the difference was also significant specifically for sentences with a non-canonical word order (PD-mean ± sd: 69.9 ± 14.1, HC-mean ± sd: 80.2 ± 11.5, p = 0.04). Using PPI, we found a statistically significant difference between the PwPD and the HC in connectivity from the right striatum to the supplementary motor area [SMA, (4 8 53)] for non-canonical sentences. This PPI connectivity was negatively correlated with the ToSC accuracy of non-canonical sentences in the PwPD. Our results showed disturbed sentence reading comprehension in the PwPD with altered task-dependent functional connectivity from the right striatum to the SMA, which supports the synchronization of the temporal and sequential aspects of language processing. The study revealed that subcortical-cortical networks (striatal-frontal loop) in PwPD are compromised, leading to impaired comprehension of syntactically complex sentences.

8.
Medicine (Baltimore) ; 101(32): e29526, 2022 Aug 12.
Artigo em Inglês | MEDLINE | ID: mdl-35960120

RESUMO

BACKGROUND: Patients with Parkinson disease (PD) treated with levodopa/carbidopa intestinal gel (LCIG) have higher prevalence of hyperhomocysteinemia and peripheral nerves damage. OBJECTIVE: The aim of our study was to test the effect of catechol-O-methyl transferase inhibitor tolcapone-as an add-on therapy to LCIG in patients with PD-on homocysteine (HCY) metabolism and nerve conduction study (NCS) parameters. METHODS: We evaluated NCS and serum B12, folic acid, and homocysteine in 16 patients with advanced PD on LCIG. Quality of life (QoL) was also assessed. Six subjects were treated with tolcapone add-on therapy (and LCIG dose reduction), 5 with B vitamin supplementation, and 5 without additional treatment. RESULTS: The level of HCY increased among patients without treatment (4.95 ± 12.54), and decreased in the vitamin (-17.73 ± 11.82) and tolcapone groups (-8.81 ± 8.36). Patients with tolcapone demonstrated improvement in polyneuropathic symptoms and signs compared with patients treated with vitamins or those without additional treatment (-0.83, d = 0.961). Although the most robust improvement in NCS parameters were observed with tolcapone, the findings were inconsistent to prove the effect of any intervention. Only tolcapone treatment was associated with improvement in QoL (d = 1.089). CONCLUSION: Our study indicates potential of tolcapone add-on therapy in LCIG treated patients in control of homocysteine levels, and improvement of polyneuropathic symptoms, as well as QoL.


Assuntos
Carbidopa , Doença de Parkinson , Antiparkinsonianos/uso terapêutico , Carbidopa/uso terapêutico , Catecol O-Metiltransferase , Combinação de Medicamentos , Homocisteína , Humanos , Levodopa/uso terapêutico , Doença de Parkinson/tratamento farmacológico , Doença de Parkinson/metabolismo , Projetos Piloto , Qualidade de Vida , Tolcapona/uso terapêutico
9.
J Pers Med ; 12(5)2022 Apr 29.
Artigo em Inglês | MEDLINE | ID: mdl-35629141

RESUMO

Parkinson's disease (PD) is currently considered progressive neurodegeneration of both the central and peripheral nervous systems. Widespread neuropathological changes lead to a complex clinical presentation with typical motor (hypokinesia, tremor, and rigidity) and various nonmotor symptoms. Orthostatic hypotension is one of the most disabling nonmotor features contributing to increased morbidity and mortality and decreased quality of life (QoL). Our study aimed to disclose the effect of a continuous infusion of levodopa-carbidopa intestinal gel (LCIG) on symptoms of orthostatic hypotension. Nine patients indicated for LCIG and eight matched patients on optimized medical treatment (OMT) were examined with scales for orthostatic symptoms (SCOPA-AUT), nonmotor symptoms and motor fluctuations (MDS-UPDRS), and QoL (PDQ39) at both baseline and after six months. The scores of "light-headedness after standing" and "fainting" decreased in the LCIG group compared to the OMT group. Treatment with LCIG was associated with a significantly higher decrease in the score of "light-headedness after standing". Change in the PDQ39 correlated positively with fluctuation improvement and with change in the scores of both "light-headedness" and "fainting". LCIG treatment improved symptoms of orthostatic hypotension in patients with PD mainly by a reduction in motor complications. Decreased severity in both motor and nonmotor fluctuations was connected also with improved QoL. Continuous treatment with LCIG should be considered not only in the case of severe motor fluctuation but also in patients with nonmotor fluctuations responsive to dopaminergic treatment.

10.
Toxins (Basel) ; 13(5)2021 05 20.
Artigo em Inglês | MEDLINE | ID: mdl-34065541

RESUMO

The visualization of the human body has frequently been groundbreaking in medicine. In the last few years, the use of ultrasound (US) imaging has become a well-established procedure for botulinum toxin therapy in people with cervical dystonia (CD). It is now undisputed among experts that some of the most relevant muscles in this indication can be safely injected under visual US guidance. This review will explore the method from basic technical considerations, current evidence to conceptual developments of the phenomenology of cervical dystonia. We will review the implications of introducing US to our understanding of muscle function and anatomy of common cervical dystonic patterns. We suggest a flow chart for the use of US to achieve a personalized treatment of people with CD. Thus, we hope to contribute a resource that is useful in clinical practice and that stimulates the ongoing development of this valuable technique.


Assuntos
Toxinas Botulínicas/administração & dosagem , Torcicolo/tratamento farmacológico , Ultrassonografia de Intervenção/métodos , Humanos , Fármacos Neuromusculares/administração & dosagem , Medicina de Precisão , Torcicolo/diagnóstico por imagem
11.
Mov Disord ; 36(8): 1835-1842, 2021 08.
Artigo em Inglês | MEDLINE | ID: mdl-33772851

RESUMO

BACKGROUND: Primary orthostatic tremor (POT) is a rare disorder, characterized by 13 to 18 Hz tremor in the legs when standing and is often refractory to medical treatment. Epidural spinal cord stimulation has been proposed as an alternative treatment. However, this approach is invasive, which limits its application. OBJECTIVE: Trans-spinal direct current stimulation (tsDCS) is a non-invasive method to modulate spinal cord circuits. The aim of this proof-of-concept study was to investigate the potential beneficial effect of tsDCS in POT. METHODS: We conducted a double-blind, sham-controlled study in 16 patients with POT. In two separate visits, patients received sham tsDCS first followed by active (either cathodal or anodal) tsDCS. The primary outcome was the change in time in standing position. Secondary outcomes comprised quantitative assessment of tremor, measurement of corticospinal excitability including short-latency afferent inhibition, and clinical global impression-improvement (CGI-I). Measurements were made at baseline, after sham tsDCS, 0-30 min, and 30-60 min after active conditions. RESULTS: Cathodal-tsDCS reduced tremor amplitude and frequency and lowered corticospinal excitability whereas anodal-tsDCS reduced tremor frequency only. CGI-I scores positively correlated with the time in standing position after both active tsDCS conditions. CONCLUSION: A single session of tsDCS can improve instability in POT. This opens a new vista for experimental treatment options using multiple sessions of spinal DC stimulation. © 2021 International Parkinson and Movement Disorder Society.


Assuntos
Estimulação da Medula Espinal , Tremor , Tontura , Potencial Evocado Motor , Humanos , Medula Espinal , Tremor/terapia
12.
Int J Gynaecol Obstet ; 152(3): 365-373, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-32975312

RESUMO

OBJECTIVE: To assess the concordance of in-utero magnetic resonance imaging (MRI) findings at 3.0T in fetuses with suspect abnormalities of the central nervous system (CNS) on ultrasonography. METHODS: A retrospective study was done on 222 pregnant women indicated for fetal MRI, with the examination performed within 2 weeks from indication. The inclusion criteria for patients were age 18 years or older with the fetus at 18 weeks of gestation or more. Fetal CNS pathologies were divided into six categories: ventriculomegaly; supratentorial midline abnormalities (ACC); supratentorial space-occupying lesions; abnormalities of the posterior fossa; destructive cerebral lesions; and cortical formation abnormalities (CFA). Chance-adjusted agreement was assessed using unweighted Cohen's kappa (κ). RESULTS: The best agreement between ultrasound and MRI was observed in ventriculomegaly (κ=0.817; 95% confidence interval [CI] 0.76-0.88). There was only a moderate agreement in ACC (κ=0.483; 95% CI 0.35-0.61). CFA pathologies had a poor agreement between the modalities (κ=0.140; 95% CI -0.03 to 0.31). CONCLUSION: Ultrasonography has good overall agreement with MRI in diagnosing fetal CNS anomalies. CFA had the most disagreement between ultrasound and MRI. The prognostic implication of these findings can be used for parental neuro-counseling but should be investigated further.


Assuntos
Imageamento por Ressonância Magnética , Malformações do Sistema Nervoso/diagnóstico por imagem , Complicações na Gravidez/diagnóstico por imagem , Ultrassonografia Pré-Natal , Adolescente , Adulto , Feminino , Idade Gestacional , Humanos , Pessoa de Meia-Idade , Malformações do Sistema Nervoso/embriologia , Gravidez , Estudos Retrospectivos , Eslováquia , Adulto Jovem
13.
PeerJ ; 8: e10363, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33240666

RESUMO

BACKGROUND: The ability to maintain balance in an upright stance gradually worsens with age and is even more difficult for patients with cognitive disorders. Cognitive impairment plays a probable role in the worsening of stability. The purpose of this study was to expose subjects with mild cognitive impairment (MCI) and healthy, age-matched controls to moving visual scenes in order to examine their postural adaptation abilities. METHODS: We observed postural responses to moving visual stimulation while subjects stood on a force platform. The visual disturbance was created by interposing a moving picture in four directions (forward, backward, right, and left). The pre-stimulus (a static scene for 10 s), stimulus (a dynamic visual scene for 20 seconds) and post-stimulus (a static scene for 20 seconds) periods were evaluated. We separately analyzed the total path (TP) of the center of pressure (COP) and the root mean square (RMS) of the COP displacement in all four directions. RESULTS: We found differences in the TP of the COP during the post-stimulus period for all stimulus directions except in motion towards the subject (left p = 0.006, right p = 0.004, and away from the subject p = 0.009). Significant RMS differences between groups were also observed during the post-stimulus period in all directions except when directed towards the subject (left p = 0.002, right p = 0.007, and away from the subject p = 0.014). CONCLUSION: Exposing subjects to a moving visual scene induced greater destabilization in MCI subjects compared to healthy elderly controls. Surprisingly, the moving visual scene also induced significant aftereffects in the MCI group. Our findings indicate that the MCI group had diminished adaptation to the dynamic visual scene and recovery. These results suggest that even mild cognitive deficits can impair sensory information integration and alter the sensory re-weighing process.

15.
Front Neurol ; 10: 1024, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31611840

RESUMO

Background: Gait disorders (GD) are frequent and disabling symptoms in patients with Parkinson's disease, mostly because they significantly limit mobility and often lead to fear of falls or actual falls. Nowadays, rehabilitation is considered to be the most effective nonpharmacological approach to reduce risk of falls. Using paradigms in virtual reality (VR) is a promising tool in neurorehabilitation because of the potential improvement in motor learning and improvement in daily functioning by replicating everyday real-life scenarios. Objective: To identify the most prevalent everyday situations which impair gait in PD that could be simulated in virtual reality (VR) environment. Methods: A newly developed self-report questionnaire consisting of 15 binary response items (YES/NO) encompassing everyday walking situations was administered to 62 patients diagnosed with idiopathic PD according to MDS Clinical Diagnostic Criteria. We included patients able to walk unassisted for at least 10 min and without significant cognitive impairment. Mokken Scale Analysis was used to evaluate psychometric properties of the scale. Results: Questionnaires from 58 patients were analyzed (31 men, age = 63 ± 9.9 y, disease duration = 7.02 ± 4.03 y, LEDD = 1115 ± 549.4 mg, H&Y = 2.4 ± 0.6). Only 10 items (out of 15) were identified as scalable and these were included in Gait Disorders Questionnaire (GDQ). The most prevalent trigger of gait disorders was walking under time pressure, followed by gait in crowded places and walking while dual-tasking. The total score of GDQ significantly correlated with the disease duration (r s = 0.347, p = 0.008) and modified H&Y staging (r s = 0.288, p = 0.028). Conclusion: With the use of GDQ we identified the most prevalent everyday transition activities that provoke gait disorders in patients with PD. The results may be useful for further development and systematic application of VR paradigms for physiotherapy of PD patients.

16.
J Geriatr Psychiatry Neurol ; 32(4): 178-185, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30961413

RESUMO

OBJECTIVES: Theory of Mind (ToM), the ability to understand other people's mental states, is essential in everyday social interactions. The relationship between cognitive domains and ToM impairment in Parkinson disease (PD) has been receiving growing attention with ambiguous findings. The objective of the current study was to ascertain which cognitive domain predicts understanding of intentions and the impact of PD-specific clinical measures on ToM performance. A secondary aim was to evaluate whether cognitive impairment mediates the relationship between severity of illness and ToM impairment. METHODS: Fifty-one nondemented patients with idiopathic PD, ranging from early to advanced stages, were enrolled. A comprehensive neurocognitive battery and 2 ToM tasks (Hinting Task and Comic Strip Task) were administered during the patients' best "on" medication state. RESULTS: Only the task of measuring working memory capacity was significantly associated with both ToM tasks (Hinting Task Spearman rank correlation [ rs] = 0.309, P ≤ .05; Comic Strip Task rs = 0.595, P ≤ .01). Patients with more progressed disease and higher doses of dopaminergic medication performed significantly worse in the Comic Strip Task. Based on the mediation analysis, relationship between the severity of the illness and understanding of intentions was mediated by cognitive flexibility. CONCLUSION: In PD, understanding of intentions is related to neurocognition, with working memory and cognitive flexibility playing a crucial role. The severity of PD predicts ToM performance.


Assuntos
Memória de Curto Prazo/fisiologia , Testes Neuropsicológicos/normas , Doença de Parkinson/psicologia , Teoria da Mente/fisiologia , Feminino , Humanos , Intenção , Masculino , Pessoa de Meia-Idade
17.
Mov Disord ; 34(1): 87-94, 2019 01.
Artigo em Inglês | MEDLINE | ID: mdl-30302819

RESUMO

BACKGROUND: Good short-term results of pallidal deep brain stimulation have been reported in myoclonus-dystonia. Efficacy and safety in the long term remain to be established. In addition, the actual impact of DBS treatment on social inclusion is unknown. The objective of this study was to assess the long-term clinical outcome, quality of life, and social adjustment of GPi-DBS in patients with ε-sarcoglycan (DYT11)-positive myoclonus-dystonia. METHODS: Consecutive myoclonus-dystonia patients with ε-sarcoglycan mutations who underwent GPi-DBS were evaluated at least 5 years postoperatively. Motor symptoms were assessed using the Burke-Fahn-Marsden Dystonia Rating Scale including the Disability Scale, a composite score combining the rest and action parts of the Unified Myoclonus Rating Scale and modified Abnormal Involuntary Movement Scale. Standardized video-protocols were assessed by a blinded and external movement disorder specialist. Social adjustment, cognition, and mood were evaluated. RESULTS: Nine patients (5 women) with long-term GPi-DBS (8.7 ± 3.1 years) were included. There was significant improvement in the composite myoclonus score (94.1% ± 4% improvement; P = 0.008). Dystonia severity was also markedly improved (71.4% ± 28.33% improvement; P = 0.008) as well as motor disability (88.3% ± 20% improvement; P = 0.008) and abnormal involuntary movement score (71.1% ± 15.0% improvement; P = 0.008). No patients experienced postoperative speech or gait problems or any permanent adverse effects. Eight of the 9 patients had fully enhanced social adjustment and personal achievement, with little or no mood or behavioral disorders. CONCLUSIONS: GPi-DBS seems to be a safe and efficacious treatment for medically refractory ɛ-sarcoglycan myoclonus-dystonia, with sustained motor benefit, good quality of life, and social adjustment in long-term follow-up. © 2018 International Parkinson and Movement Disorder Society.


Assuntos
Estimulação Encefálica Profunda , Distúrbios Distônicos/terapia , Transtornos Motores/terapia , Ajustamento Social , Adolescente , Adulto , Idoso , Estimulação Encefálica Profunda/métodos , Pessoas com Deficiência/psicologia , Distúrbios Distônicos/psicologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos Motores/psicologia , Tempo , Resultado do Tratamento , Adulto Jovem
18.
BMC Neurol ; 18(1): 44, 2018 Apr 20.
Artigo em Inglês | MEDLINE | ID: mdl-29678162

RESUMO

BACKGROUND: Hepatic encephalopathy may manifest by a wide spectrum of neuropsychiatric symptoms, including cognitive impairment, seizures or extrapyramidal symptoms. The liver transplant can lead to improvement of the signs of encephalopathy but subsequent immunosuppressive treatment might possess pronounced neurotoxicity. CASE PRESENTATION: We present a case report of a patient with chronic liver disease who developed signs of Parkinsonism after an orthotopic liver transplant, with consecutive immunosuppressant treatment with tacrolimus. Despite the improvement of liver functions due to the cytostatic treatment, a progressive worsening of neuropsychiatric symptoms associated with the presence of tremor was observed. Metabolic as well as endocrine dysfunctions were excluded as the primary causes of this condition. A brain CT did not reveal structural pathology. Signs of severe, symmetric Parkinsonism - with resting tremor, bradykinesia, rigidity and severe postural instability were observed. A brain MRI was performed with the presence of T2- hyperintensities in basal ganglia bilaterally. Tacrolimus blood concentration was elevated; hence the dose was reduced and later switched to less toxic sirolimus. Subsequently, clinical signs markedly improved after treatment modification. Improvement of clinical symptomatology after tacrolimus discontinuation supports the drug-induced etiology of this neurological condition. CONCLUSIONS: Cytostatic treatment after solid organ transplantation often leads to signs of encephalopathy. If necessary, the dose of cytostatics needs to be reduced, or a less toxic agent must be chosen for the therapy. This modification is usually efficient with no further need for neurological intervention.


Assuntos
Imunossupressores/efeitos adversos , Transplante de Fígado , Transtornos Parkinsonianos , Tacrolimo/efeitos adversos , Feminino , Humanos , Imunossupressores/uso terapêutico , Pessoa de Meia-Idade , Transtornos Parkinsonianos/induzido quimicamente , Transtornos Parkinsonianos/fisiopatologia , Tacrolimo/uso terapêutico
19.
Neurosci Lett ; 636: 151-157, 2017 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-27836806

RESUMO

BACKGROUND: Dystonia is a movement disorder with patterned, directional, and often sustained muscle contractions that produce abnormal postures or repetitive movements. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is an effective and safe treatment for medically refractory dystonia. However, recent studies reported gait problems, gait freezing and falls in patients treated with DBS. Because these symptoms may point to deficient gait initiation processes, we systematically assessed the anticipatory postural adjustments (APAs) prior to stepping in dystonia patients with GPi-DBS. METHODS: Thirteen patients with focal/segmental dystonia under GPi-DBS and twelve healthy control subjects were included in the study. Data were collected using pressure sensitive sensors and APAs were studied by centre of pressure measures. We compared APAs of both groups and analysed the influence of GPi-DBS on APAs in patients. RESULTS: Medio-lateral and antero-posterior COP displacements, total COP path, maximal APA velocity and 1st step length were all smaller in patients for both ON (p=0.006, p=0.018, p=0.002, p=0.016, p=0.04) and OFF (p=0.001, p=0.01, p=0.001, p=0.03, p=0.024) condition compared to healthy subjects. GPi-DBS did not change APA parameters in patients. CONCLUSIONS: Observations that APAs are impaired in dystonia and are at the same time not affected by the stimulation current are compatible with the assumption that APAs and dystonic symptoms may rely on distinct networks, possibly within the same cortical and basal ganglia structures. With no effect of stimulation on APAs it is unlikely that this would be a mechanism of impaired balance in the patients after the surgery.


Assuntos
Distonia/terapia , Transtornos Neurológicos da Marcha/fisiopatologia , Marcha/fisiologia , Globo Pálido/fisiopatologia , Contração Muscular/fisiologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estimulação Encefálica Profunda/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade
20.
Gait Posture ; 42(3): 246-50, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-26164353

RESUMO

BACKGROUND: It has been suggested that dynamical measures such as sample entropy may be more appropriate than conventional measures when analyzing time series data such as postural sway. We evaluated conventional and dynamical measures of postural sway in Parkinson disease (PD) patients with and without freezing episodes. METHODS: COP (center of pressure) data were recorded during quiet standing with eyes open, eyes closed and while performing a dual task. Data for 16 patients with freezing of gait, 17 patients with no history of freezing and 24 healthy subjects were analyzed. The amount of postural sway was quantified using conventional measures, whereas for the characterization of the temporal structure of the COP data the normalized sway path and sample entropy was calculated. RESULTS: Mean radius was higher and sample entropy was lower in patients with freezing symptoms as compared to healthy subjects in all three conditions. Dual-tasking significantly increased sway path length in patients with freezing, while normalized sway path did not change over conditions in this group. CONCLUSIONS: Our findings show that postural sway is characterized by a combination of large radius, short normalized sway path and high regularity of the COP only in patients with freezing. This pattern becomes most prominent in a dual-task paradigm. This may explain higher occurrence of gait freezing in dual task situations with subsequent higher risk of falls. Results suggested that dynamic measures may add valuable information for characterizing postural stability in PD patients.


Assuntos
Marcha/fisiologia , Doença de Parkinson/fisiopatologia , Equilíbrio Postural/fisiologia , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/diagnóstico
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