RESUMO
The anatomy of the human piriform cortex (PC) is poorly understood. We used a bimodal connectivity-based-parcellation approach to investigate subregions of the PC and its connectional differentiation from the amygdala. One hundred (55 % female) genetically unrelated subjects from the Human Connectome Project were included. A region of interest (ROI) was delineated bilaterally covering PC and amygdala, and functional and structural connectivity of this ROI with the whole gray matter was computed. Spectral clustering was performed to obtain bilateral parcellations at granularities of k = 2-10 clusters and combined bimodal parcellations were computed. Validity of parcellations was assessed via their mean individual-to-group similarity per adjusted rand index (ARI). Individual-to-group similarity was higher than chance in both modalities and in all clustering solutions. The amygdala was clearly distinguished from PC in structural parcellations, and olfactory amygdala was connectionally more similar to amygdala than to PC. At higher granularities, an anterior and ventrotemporal and a posterior frontal cluster emerged within PC, as well as an additional temporal cluster at their boundary. Functional parcellations also showed a frontal piriform cluster, and similar temporal clusters were observed with less consistency. Results from bimodal parcellations were similar to the structural parcellations. Consistent results were obtained in a validation cohort. Distinction of the human PC from the amygdala, including its olfactory subregions, is possible based on its structural connectivity alone. The canonical fronto-temporal boundary within PC was reproduced in both modalities and with consistency. All obtained parcellations are freely available.
Assuntos
Tonsila do Cerebelo , Conectoma , Córtex Piriforme , Humanos , Feminino , Masculino , Córtex Piriforme/anatomia & histologia , Córtex Piriforme/diagnóstico por imagem , Córtex Piriforme/fisiologia , Adulto , Conectoma/métodos , Tonsila do Cerebelo/anatomia & histologia , Tonsila do Cerebelo/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Vias Neurais/anatomia & histologia , Vias Neurais/diagnóstico por imagem , Adulto Jovem , Rede Nervosa/diagnóstico por imagem , Rede Nervosa/anatomia & histologiaRESUMO
BACKGROUND: Chronic pain is a common non-motor symptom in patients with Parkinson's disease (PD). AIM: To facilitate the diagnosis of pain in PD, we developed a new classification system the Parkinson's disease pain classification system (PD-PCS) and translated the corresponding validated questionnaire into German. METHODS: A causal relationship of the respective pain syndrome with PD can be determined by four questions before assigning it hierarchically into one of three pain categories (neuropathic, nociceptive and nociplastic). RESULTS: In the initial validation study 77% of the patients (122/159) had PD-associated pain comprising 87 (55%) with nociceptive, 36 (22%) with nociplastic and 24 (16%) with neuropathic pain. The study revealed a high validity of the questionnaire and a moderate intrarater and interrater reliability. The questionnaire has been adapted into German and employed in 30 patients. DISCUSSION: The PD-PCS questionnaire is a valid and reliable tool to determine the relationship of a pain syndrome with PD before classifying it according to the underlying category, facilitating further diagnostics and treatment.
Assuntos
Neuralgia , Doença de Parkinson , Humanos , Neuralgia/complicações , Neuralgia/diagnóstico , Neuralgia/terapia , Medição da Dor , Doença de Parkinson/complicações , Doença de Parkinson/diagnóstico , Reprodutibilidade dos Testes , Inquéritos e QuestionáriosRESUMO
OBJECTIVES: To evaluate whether there is an increased risk for noise-induced hearing loss at high altitude rsp. in hypobaric hypoxia. METHODS: Thirteen volunteers got standard audiometry at 125, 250, 500, 750, 1000, 1500, 2000, 3000, 4000, 6000, and 8000 Hz before and after 10 min of white noise at 90 dB. The system was calibrated for the respective altitude. Measurements were performed at Kathmandu (1400 m) and at Gorak Shep (5300 m) (Solo Khumbu/Nepal) after 10 days of acclimatization while on trek. Temporary threshold shift (TTS) was analyzed by descriptive statistics and by factor analysis. RESULTS: TTS is significantly more pronounced at high altitudes. Acclimatization does not provide any protection of the inner ear, although it increases arterial oxygen saturation. CONCLUSION: The thresholds beyond which noise protection is recommended (> 80 dB) or necessary (> 85 dB) are not sufficient at high altitudes. We suggest providing protective devices above an altitude of 1500 m ("ear threshold altitude") when noise level is higher than 75 dB and using them definitively above 80 dB. This takes the individual reaction on hypobaric hypoxia at high altitude into account.
Assuntos
Altitude , Limiar Auditivo , Exposição Ambiental/efeitos adversos , Ruído/efeitos adversos , Oxigênio , Aclimatação , Adulto , Audiometria , Expedições , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Adulto JovemRESUMO
BACKGROUND AND PURPOSE: Subthalamic nucleus (STN) deep brain stimulation (DBS) improves quality of life (QoL) and motor and non-motor symptoms in advanced Parkinson's disease (PD). However, its effect on alexithymia and its relationship to other neuropsychiatric symptoms and QoL in PD is unclear. METHODS: In this prospective, observational study of 39 patients with PD undergoing STN-DBS, we examined the Parkinson's Disease Questionnaire-8 (PDQ-8), 20-item Toronto Alexithymia Scale (TAS-20), Hospital Anxiety and Depression Scale (HADS), Self-Report Manic Inventory (SRMI), Apathy Evaluation Scale (AES), Unified Parkinson's Disease Rating Scale (UPDRS) activities of daily living, UPDRS motor examination and UPDRS complications (UPDRS-II/-III/-IV) and levodopa-equivalent daily dose (LEDD) pre-operatively and at 5-month follow-up. Outcome changes were tested with Wilcoxon signed-rank or paired t-test when parametric tests were applicable and corrected for multiple comparisons. The relationship between outcome changes was explored with bivariate correlations. Additionally, partial correlations between PDQ-8 and TAS-20 were computed controlling for HADS, SRMI and AES change scores. Predictor analyses for PDQ-8 improvement were calculated for all baseline parameters. RESULTS: The baseline prevalence of alexithymia was 17.9%. We observed significant beneficial effects of STN-DBS on PDQ-8, TAS-20, HADS, UPDRS-II, -III and -IV scores and significant LEDD reduction. The correlation between TAS-20 and PDQ-8 improvements remained significant after controlling for all other aforementioned outcomes. Predictor analyses for PDQ-8 improvement were significant for PDQ-8 and TAS-20. CONCLUSIONS: This is the first report of beneficial effects of STN-DBS on alexithymia. Alexithymia was significantly associated with QoL outcome independent of anxiety, depression, mania and apathy. Our study highlights the importance of alexithymia for holistic assessments of DBS outcomes.
Assuntos
Atividades Cotidianas/psicologia , Sintomas Afetivos/terapia , Estimulação Encefálica Profunda/métodos , Doença de Parkinson/psicologia , Qualidade de Vida/psicologia , Núcleo Subtalâmico/fisiopatologia , Sintomas Afetivos/complicações , Sintomas Afetivos/psicologia , Idoso , Feminino , Humanos , Masculino , Testes de Estado Mental e Demência , Pessoa de Meia-Idade , Doença de Parkinson/complicações , Doença de Parkinson/terapia , Estudos Prospectivos , Inquéritos e Questionários , Resultado do TratamentoRESUMO
OBJECTIVE: Parkinson's disease (PD) adversely affects oral health (OH). However, the informative value of xerostomia compared to objective parameters and its impact on quality of life (QoL) are still unclear. This study aimed to explore whether xerostomia correlates with hyposalivation and to define its impact on OH-related QoL. MATERIALS AND METHODS: Whole stimulated saliva (WSS) was collected from 30 patients with PD and 30 matched healthy controls. Objective parameters (community periodontal index of treatment needs, plaque/gingivitis index, mucosa situation and cheilitis angularis) and questionnaires (German Oral Health Impact Profile [OHIPG]-14, visual analogue scale [VAS], xerostomia [yes/no] and the Unified Parkinson's Disease Rating Scale-II) were assessed. RESULTS: Eighty-seven per cent of patients with PD showed hyposalivation vs 50% of controls (P = 0.001); 50% of patients with PD reported xerostomia, and none of controls (P < 0.001). The OHIPG-14 was impaired in patients with PD compared to controls (P < 0.001), PD patients with xerostomia reported mean VAS values of 4.1 (s.d.: 2.2). WSS did not correlate with VAS values. CONCLUSIONS: Half of the patients with PD reported xerostomia and underestimated their xerostomic status, with higher probability than healthy controls. WSS did not reflect the grade of xerostomia. Patients with PD suffered from impaired OH-related QoL. Dental teams should not overlook these oral health risks.
Assuntos
Doença de Parkinson/complicações , Qualidade de Vida , Xerostomia/etiologia , Adulto , Idoso , Estudos de Casos e Controles , Feminino , Indicadores Básicos de Saúde , Humanos , Masculino , Pessoa de Meia-Idade , Xerostomia/diagnóstico , Xerostomia/epidemiologiaRESUMO
BACKGROUND: Subthalamic stimulation reduces motor disability and improves quality of life in patients with advanced Parkinson's disease who have severe levodopa-induced motor complications. We hypothesized that neurostimulation would be beneficial at an earlier stage of Parkinson's disease. METHODS: In this 2-year trial, we randomly assigned 251 patients with Parkinson's disease and early motor complications (mean age, 52 years; mean duration of disease, 7.5 years) to undergo neurostimulation plus medical therapy or medical therapy alone. The primary end point was quality of life, as assessed with the use of the Parkinson's Disease Questionnaire (PDQ-39) summary index (with scores ranging from 0 to 100 and higher scores indicating worse function). Major secondary outcomes included parkinsonian motor disability, activities of daily living, levodopa-induced motor complications (as assessed with the use of the Unified Parkinson's Disease Rating Scale, parts III, II, and IV, respectively), and time with good mobility and no dyskinesia. RESULTS: For the primary outcome of quality of life, the mean score for the neurostimulation group improved by 7.8 points, and that for the medical-therapy group worsened by 0.2 points (between-group difference in mean change from baseline to 2 years, 8.0 points; P=0.002). Neurostimulation was superior to medical therapy with respect to motor disability (P<0.001), activities of daily living (P<0.001), levodopa-induced motor complications (P<0.001), and time with good mobility and no dyskinesia (P=0.01). Serious adverse events occurred in 54.8% of the patients in the neurostimulation group and in 44.1% of those in the medical-therapy group. Serious adverse events related to surgical implantation or the neurostimulation device occurred in 17.7% of patients. An expert panel confirmed that medical therapy was consistent with practice guidelines for 96.8% of the patients in the neurostimulation group and for 94.5% of those in the medical-therapy group. CONCLUSIONS: Subthalamic stimulation was superior to medical therapy in patients with Parkinson's disease and early motor complications. (Funded by the German Ministry of Research and others; EARLYSTIM ClinicalTrials.gov number, NCT00354133.).
Assuntos
Terapia por Estimulação Elétrica , Doença de Parkinson/terapia , Qualidade de Vida , Atividades Cotidianas , Adulto , Antiparkinsonianos/efeitos adversos , Antiparkinsonianos/uso terapêutico , Terapia Combinada , Agonistas de Dopamina/efeitos adversos , Agonistas de Dopamina/uso terapêutico , Discinesias/etiologia , Terapia por Estimulação Elétrica/efeitos adversos , Feminino , Humanos , Neuroestimuladores Implantáveis/efeitos adversos , Análise de Intenção de Tratamento , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/tratamento farmacológico , Doença de Parkinson/fisiopatologia , Núcleo Subtalâmico , Inquéritos e Questionários , Resultado do TratamentoRESUMO
BACKGROUND: Clinical and ethical implications of personality and mood changes in Parkinson's disease (PD) patients treated with subthalamic deep brain stimulation (STN-DBS) are under debate. Although subjectively perceived personality changes are often mentioned by patients and caregivers, few empirical studies concerning these changes exist. Therefore, we analysed subjectively perceived personality and mood changes in STN-DBS PD patients. METHOD: In this prospective study of the ELSA-DBS group, 27 PD patients were assessed preoperatively and 1 year after STN-DBS surgery. Two categories, personality and mood changes, were analysed with semi-structured interviews. Patients were grouped into personality change yes/no, as well as positive/negative mood change groups. Caregivers were additionally interviewed about patients' personality changes. Characteristics of each group were assessed with standard neurological and psychiatric measurements. Predictors for changes were analysed. RESULTS: Personality changes were perceived by six of 27 (22%) patients and by 10 of 23 caregivers (44%). The preoperative hypomania trait was a significant predictor for personality change perceived by patients. Of 21 patients, 12 (57%) perceived mood as positively changed. Higher apathy and anxiety ratings were found in the negative change group. CONCLUSIONS: Our results show that a high proportion of PD patients and caregivers perceived personality changes under STN-DBS, emphasizing the relevance of this topic. Mood changed in positive and negative directions. Standard measurement scales failed to adequately reflect personality or mood changes subjectively perceived by patients. A more individualized preoperative screening and preparation for patients and caregivers, as well as postoperative support, could therefore be useful.
Assuntos
Estimulação Encefálica Profunda/efeitos adversos , Transtornos do Humor/epidemiologia , Transtornos da Personalidade/epidemiologia , Adulto , Idoso , Análise de Variância , Cuidadores , Feminino , Alemanha/epidemiologia , Humanos , Entrevistas como Assunto , Masculino , Pessoa de Meia-Idade , Transtornos do Humor/etiologia , Doença de Parkinson/cirurgia , Transtornos da Personalidade/etiologia , Estudos Prospectivos , Escalas de Graduação Psiquiátrica , Qualidade de Vida , SubtálamoRESUMO
BACKGROUND AND PURPOSE: Patients with Parkinson's disease (PD) are at high risk for cognitive dysfunction. Non-pharmacological interventions have attracted increasing interest for enhancing PD patients' cognitive functions. METHODS: One-year follow-up data (T2 ) of a randomized controlled trial evaluating two 6-week cognitive trainings - a structured (NEUROvitalis, NV) and an unstructured (mentally fit, MF) program - compared with a waiting list control group (CG) in non-demented PD patients (Hoehn and Yahr I-III) are presented. Forty-seven PD patients were examined at T2 . Effects on overall cognitive functions (Mini-Mental State Examination and DemTect) were compared between all groups with repeated measurement analyses of variance. A combined score of the percentage change value from baseline (T0 ) to T2 was calculated to identify patients who retained or improved their cognitive state (responders). The risk of developing mild cognitive impairment (MCI) was analyzed. RESULTS: Significant time × treatment effects on overall cognitive functions were found for both training groups, each compared separately to the CG (DemTect, P < 0.05). Nine patients (56.3%) of the NV group, seven (41.2%) of the MF group and three (21.4%) of the CG were responders. Comparing NV to CG the odds ratio was 4.7 [95% confidence interval (0.8; 33.3)], and comparing MF to CG it was 2.6 [95% confidence interval (0.4; 17.4)]. MCI risk for patients without prior MCI was 40.0% in CG, 18.2% in MF and 18.2% in NV. The odds ratio was 3 comparing NV to CG, MF to CG. DISCUSSION: This study gives evidence that cognitive training may be effective to prevent cognitive decline and onset of MCI in PD patients.
Assuntos
Transtornos Cognitivos/prevenção & controle , Terapia Cognitivo-Comportamental/métodos , Doença de Parkinson/terapia , Idoso , Transtornos Cognitivos/etiologia , Disfunção Cognitiva/etiologia , Disfunção Cognitiva/prevenção & controle , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/complicações , Resultado do TratamentoRESUMO
OBJECTIVES: Severe hepatic encephalopathy gives rise to asterixis, a striking motor symptom also called flapping tremor, which is characterized by a sudden ceasing of muscle tone in all muscles of a limb. In this study, we aimed at scrutinizing the cortical activation associated with asterixis and unraveling the underlying pathophysiological mechanisms. MATERIAL AND METHODS: We recorded simultaneously neural activity with magnetoencephalography (MEG) and muscle activity with surface EMG in nine patients with manifest hepatic encephalopathy showing asterixis. Asterixis events were detected semiautomatically and served as triggers for averaging MEG signals. Evoked responses averaged time-locked to asterixis events were subjected to equivalent current dipole (ECD) modeling. Additionally, we localized the strongest cortico-muscular coherence in the frequency of the co-occurring tremulousness. RESULTS: Evoked fields averaged time-locked to asterixis events were best explained by a single dipolar source in the contralateral primary motor cortex (M1, Talairach coordinates of mean localization: -40, -20, and 64; Brodmann area 4). This dipole showed a twofold field reversal, that is biphasic wave, with frontal dipole orientation at 49 ms before flap onset and 99 ms after flap onset. Conversely, two maxima with occipital dipole orientation were observed 2 ms and 160 ms after flap onset. Cortico-muscular coherence for the tremulousness was likewise localized in the contralateral M1 confirming earlier findings in the present patient cohort. CONCLUSIONS: Our results reveal an involvement of M1 in the generation of asterixis. As also tremulousness, also called mini-asterixis, was shown to originate in M1, asterixis and mini-asterixis may share common pathophysiological mechanisms.
Assuntos
Córtex Cerebral/fisiopatologia , Discinesias/etiologia , Discinesias/fisiopatologia , Encefalopatia Hepática/complicações , Encefalopatia Hepática/fisiopatologia , Idoso , Eletromiografia , Feminino , Humanos , Magnetoencefalografia , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/fisiopatologiaRESUMO
The interaction of basal ganglia and other brain regions is more complex regarding anatomic and functional perspectives than previously assumed. Hence, the classical basal ganglia model has to be extended to at least four satellite systems modulating motor-executive, associative and limbic-motivational brain regions: (i) an indirect projection system, (ii) a striato-nigro-striatal loop, (iii) a "hyperdirect" projection system as well as additional projections to the subthalamic nucleus and (iv) multisynaptic connections from the cerebellum exerting influence on the indirect projection system. The investigation of these satellite systems would be invaluable to foster our understanding of basal ganglia circuitries and may yield a better appreciation of largely opaque symptoms like resting tremor in Parkinson's disease; analysis of these anatomic pathways and functional implications may facilitate explanatory model approaches to side effects due to dopaminergic therapy and deep brain stimulation in humans and thereby offer the possibility for new therapeutic approaches in movement disorders.
Assuntos
Gânglios da Base/anatomia & histologia , Gânglios da Base/patologia , Gânglios da Base/fisiopatologia , Doenças dos Gânglios da Base/patologia , Doenças dos Gânglios da Base/fisiopatologia , Doenças dos Gânglios da Base/terapia , Humanos , Modelos NeurológicosRESUMO
Neurodegenerative movement disorders, such as Huntington's disease (HD), have become a promising field for Deep Brain Stimulation (DBS). This study aims to contribute to the establishment of a well-grounded database including both expected and unexpected effects of pallidal DBS in HD, and to discuss the ethical and legal restrictions of DBS in cognitively limited patients. Evaluation of the outcome data indicates that pallidal DBS exerted an independent effect on motor symptoms but probably also on the patient's cognitive and affective state. The cognitive decline, however, that characterizes the late stage of neurodegenerative disorders implicates ethical and legal problems given the patients' inability to give informed consent to DBS.
Assuntos
Estimulação Encefálica Profunda/métodos , Globo Pálido/fisiologia , Doença de Huntington/terapia , Adulto , Administração de Caso , Cognição/fisiologia , Estimulação Encefálica Profunda/efeitos adversos , Depressão/etiologia , Depressão/terapia , Eletrodos Implantados , Feminino , Humanos , Doença de Huntington/psicologia , Transtornos Mentais/etiologia , Transtornos Mentais/terapia , Transtornos dos Movimentos/etiologia , Transtornos dos Movimentos/terapia , Testes Neuropsicológicos , Medição de Risco , Técnicas Estereotáxicas , Resultado do TratamentoRESUMO
Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an effective treatment for Parkinson's disease, but can lead to adverse effects including psychiatric disturbance. Little is known about the risk factors and treatment options for such effects. Here, we describe a patient who reproducibly developed stimulation-induced hypomania when using ventrally located electrodes and responded well to pharmacological intervention while leaving the stimulation parameters unchanged to preserve motor benefits. In spite of clinical remission, [¹5O]-positron-emission-tomography (PET) demonstrated activation patterns similar to those reported during mania. This case, therefore, highlights an important treatment option of adverse effects of DBS, but also points toward the need for investigations of its risk factors and their underlying neurobiological mechanisms.
Assuntos
Antimaníacos/uso terapêutico , Transtorno Bipolar/tratamento farmacológico , Transtorno Bipolar/etiologia , Clozapina/uso terapêutico , Estimulação Encefálica Profunda/efeitos adversos , Ácido Valproico/uso terapêutico , Humanos , Masculino , Pessoa de Meia-Idade , Doença de Parkinson/terapia , Resultado do TratamentoRESUMO
Deep brain stimulation (DBS), which is already established as an effective treatment for movement disorders, such as Parkinson's disease, is increasingly being considered as a therapy option for mental diseases. Due to the increasing number of successful applications of DBS for otherwise therapy-resistant psychiatric diseases, DBS is becoming more and more of interest in fields of fundamental research as well as clinical care. However, the stimulation system is a medical product which has to be neurosurgically implanted and this fact is often used to draw certain analogies to earlier psychosurgical approaches in the era of Freeman. But, looking at the historical development of DBS, as is the aim of the present systematic and literature-based overview, it becomes obvious that DBS did not arise exclusively from the inglorious period of psychosurgery. In fact, two partly in parallel evolving lines of medical progress have contributed to the development of DBS as it is applied today. One of these lines is the use of lesional neurosurgical procedures, such as incision of capsules and cingulotomy, which in contrast to psychosurgical interventions in the era of Freeman, is aimed at subcortical structures and provides important basic knowledge for the choice of target points. In addition DBS is rooted in the application of an electrical charge with the goal to stimulate neuronal networks.
Assuntos
Estimulação Encefálica Profunda/história , Transtornos Mentais/história , Transtornos Mentais/terapia , Alemanha , História do Século XX , História do Século XXIRESUMO
Idiopathic Parkinson's disease is still a clinical diagnosis. However, modern imaging and nuclear techniques allow very early diagnosis and lead to higher security in the differential diagnosis between idiopathic Parkinson's disease and atypical Parkinson syndromes. At early stages of the disease, modification of disease progression and symptom control are key factors of the therapy. Continuous dopaminergic stimulation is even more important at later stages with first fluctuations. In stages where conservative medical options have been exhausted continuous pump therapies with Duodopa and apomorphine are attractive options. Deep brain stimulation in the subthalamic nucleus has turned out in the last years, especially in younger patients, to be a highly successful treatment option. Deep drain stimulation requires, however, a close preoperative work-up and individual consideration of potential effects and side effects.
Assuntos
Doença de Parkinson/diagnóstico , Doença de Parkinson/terapia , Antidepressivos/uso terapêutico , Antiparkinsonianos/efeitos adversos , Antiparkinsonianos/uso terapêutico , Estimulação Encefálica Profunda , Transtornos Disruptivos, de Controle do Impulso e da Conduta/tratamento farmacológico , Transtornos Disruptivos, de Controle do Impulso e da Conduta/etiologia , Implantes de Medicamento/uso terapêutico , Transtornos Neurológicos da Marcha/etiologia , Transtornos Neurológicos da Marcha/terapia , Humanos , Levodopa/uso terapêutico , Doença de Parkinson/tratamento farmacológico , Doença de Parkinson/psicologia , Psicoses Induzidas por Substâncias/tratamento farmacológico , Tremor/etiologia , Tremor/terapiaRESUMO
Neurodegeneration with brain iron accumulation encompasses a heterogeneous group of rare neurodegenerative disorders that are characterized by iron accumulation in the brain. Severe generalized dystonia is frequently a prominent symptom and can be very disabling, causing gait impairment, difficulty with speech and swallowing, pain and respiratory distress. Several case reports and one case series have been published concerning therapeutic outcome of pallidal deep brain stimulation in dystonia caused by neurodegeneration with brain iron degeneration, reporting mostly favourable outcomes. However, with case studies, there may be a reporting bias towards favourable outcome. Thus, we undertook this multi-centre retrospective study to gather worldwide experiences with bilateral pallidal deep brain stimulation in patients with neurodegeneration with brain iron accumulation. A total of 16 centres contributed 23 patients with confirmed neurodegeneration with brain iron accumulation and bilateral pallidal deep brain stimulation. Patient details including gender, age at onset, age at operation, genetic status, magnetic resonance imaging status, history and clinical findings were requested. Data on severity of dystonia (Burke Fahn Marsden Dystonia Rating Scale-Motor Scale, Barry Albright Dystonia Scale), disability (Burke Fahn Marsden Dystonia Rating Scale-Disability Scale), quality of life (subjective global rating from 1 to 10 obtained retrospectively from patient and caregiver) as well as data on supportive therapy, concurrent pharmacotherapy, stimulation settings, adverse events and side effects were collected. Data were collected once preoperatively and at 2-6 and 9-15 months postoperatively. The primary outcome measure was change in severity of dystonia. The mean improvement in severity of dystonia was 28.5% at 2-6 months and 25.7% at 9-15 months. At 9-15 months postoperatively, 66.7% of patients showed an improvement of 20% or more in severity of dystonia, and 31.3% showed an improvement of 20% or more in disability. Global quality of life ratings showed a median improvement of 83.3% at 9-15 months. Severity of dystonia preoperatively and disease duration predicted improvement in severity of dystonia at 2-6 months; this failed to reach significance at 9-15 months. The study confirms that dystonia in neurodegeneration with brain iron accumulation improves with bilateral pallidal deep brain stimulation, although this improvement is not as great as the benefit reported in patients with primary generalized dystonias or some other secondary dystonias. The patients with more severe dystonia seem to benefit more. A well-controlled, multi-centre prospective study is necessary to enable evidence-based therapeutic decisions and better predict therapeutic outcomes.
Assuntos
Encefalopatias/terapia , Encéfalo/fisiopatologia , Estimulação Encefálica Profunda/métodos , Distonia/terapia , Ferro/metabolismo , Doenças Neurodegenerativas/terapia , Adolescente , Adulto , Encefalopatias/fisiopatologia , Criança , Pré-Escolar , Estimulação Encefálica Profunda/efeitos adversos , Distonia/fisiopatologia , Feminino , Lateralidade Funcional , Globo Pálido/fisiopatologia , Humanos , Lactente , Masculino , Doenças Neurodegenerativas/fisiopatologia , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
For more than 15 years deep brain stimulation of the subthalamic nucleus and globus pallidus internus have become therapeutic options in advanced Parkinson's disease. The number of patients with long-term treatment is increasing steadily. This review focuses on issues of the long-term care of these Parkinson's patients, including differences of the available deep brain stimulation systems, recommendations for follow-up examinations, implications for medical diagnostics and therapies and an algorithm for symptom deterioration. Today, there is no profound evidence that deep brain stimulation prevents disease progression. However, symptomatic relief from motor symptoms is maintained during long-term follow-up and interruption of the therapy remains an exception.
Assuntos
Estimulação Encefálica Profunda , Doença de Parkinson/terapia , Algoritmos , Progressão da Doença , Eletrodos Implantados , Eletrônica , Falha de Equipamento , Humanos , Infecções/etiologia , Assistência de Longa Duração , Procedimentos Neurocirúrgicos , Doença de Parkinson/fisiopatologia , Doença de Parkinson/psicologia , Resultado do TratamentoRESUMO
AIM: Manifest hepatic encephalopathy (HE) goes along with motor symptoms such as ataxia, mini-asterixis, and asterixis. The relevance of motor impairments in cirrhotics without and with minimal HE (mHE) is still a matter of debate. PATIENTS AND METHODS: We tested three different groups of patients with liver cirrhosis: no signs of HE (HE 0), mHE, and manifest HE grade 1 according to the West Haven criteria (HE 1). All patients (n = 24) and 11 healthy control subjects were neuropsychometrically tested including critical flicker frequency (CFF), a reliable measure for HE. Motor abilities were assessed using Fahn Tremor Scale and International Ataxia Rating Scale. Fastest alternating index finger movements were analyzed for frequency and amplitude. RESULTS: Statistical analyses showed an effect of HE grade on tremor and ataxia (P < 0.01). Additionally, both ratings yielded strong negative correlation with CFF (P < 0.01, R = -0.5). Analysis of finger movements revealed an effect of HE grade on movement frequency (P < 0.03). Moreover, decreasing movement frequency and increasing movement amplitude parallel decreasing CFF (P < 0.01, R = 0.6). CONCLUSION: Our results indicate that ataxia, tremor, and slowing of finger movements are early markers for cerebral dysfunction in HE patients even prior to neuropsychometric alterations becoming detectable.
Assuntos
Discinesias/diagnóstico , Discinesias/etiologia , Encefalopatia Hepática/complicações , Cirrose Hepática/complicações , Idoso , Alcoolismo/complicações , Ataxia/diagnóstico , Ataxia/etiologia , Feminino , Dedos/fisiologia , Lateralidade Funcional , Humanos , Masculino , Pessoa de Meia-Idade , Testes Neuropsicológicos , Tremor/diagnóstico , Tremor/etiologiaRESUMO
Deep brain stimulation (DBS) is a safe and successful therapeutic option for patients with dystonia and tremor syndrome who do not respond sufficiently to conservative therapies. The most common target of DBS in patients with dystonia is the internal region of the globus pallidus (GPI). DBS of the GPI leads to long-lasting and remarkable improvement of dystonic movements in about 80% of patients. Recently it could be shown that not only patients with idiopathic dystonia but also patients with secondary dystonia can benefit from DBS although to a somewhat lesser extent. In patients with tremor syndromes, such as essential tremor, tremor-dominant Parkinson's disease or tremor in multiple sclerosis (MS) the intermediate ventral nucleus of the thalamus (VIM) as well as the subthalamic region proved to be promising targets for DBS electrodes. Especially in patients with essential tremor VIM-DBS leads to an often acute reduction of the tremor syndrome. In long-term observations, however, patients with essential tremor showed some tolerability to VIM-DBS leading to a slow increase of stimulation parameters to maintain a stable effect. VIM-DBS in patients with Parkinson's disease is rare and is reserved for elderly patients with pronounced tremor syndrome and little disease progression. Controlled studies and data on DBS in MS tremor are lacking and data are sparse and heterogeneous. Therefore, VIM-DBS in MS tremor patients has to be evaluated individually with caution. In summary patients with tremor syndromes as well as dystonia who cannot be adequately controlled with conservative therapy are good candidates for deep brain stimulation, a therapeutic option with moderate complications and risks and very good outcome for most patients.
Assuntos
Estimulação Encefálica Profunda , Distonia/terapia , Tremor/terapia , Mapeamento Encefálico , Dominância Cerebral/fisiologia , Distonia/etiologia , Distonia/fisiopatologia , Globo Pálido/fisiopatologia , Humanos , Esclerose Múltipla/fisiopatologia , Esclerose Múltipla/terapia , Doença de Parkinson/fisiopatologia , Doença de Parkinson/terapia , Seleção de Pacientes , Núcleo Subtalâmico/fisiopatologia , Tremor/etiologia , Tremor/fisiopatologia , Núcleos Ventrais do Tálamo/fisiopatologiaRESUMO
Cognitive decline is a common disorder in idiopathic Parkinson's syndrome, the risk for the development of a dementia is four- to six-fold higher for Parkinsonian patients. The cognitive profile in Parkinson's disease dementia (PDD) differs from that of Alzheimer-type dementias. The affected cognitive functions include attention, executive functions, visual-spatial functions and recall. The main differential diagnosis for PDD is the Lewy body dementia (LBD), which can be differentiated through the temporal development of motor and cognitive symptoms. Cognitive symptoms in Parkinsonian syndromes have a relevant negative impact on quality of life, on the burden for the care-givers, on the prognosis of the disease and on the possible referral to a nursing home. Dementias in Parkinsonian syndromes (PDD and LBD) need a confirmatory diagnosis at an early stage in order to initiate further therapeutic steps with, e. g., acetylcholine esterase inhibitors or, perspectively, neuropsychological training methods.