Physiological and biochemical role of nickel in nodulation and biological nitrogen fixation in Vigna unguiculata L. Walp.

Studies on the role of nickel (Ni) in photosynthetic and antioxidant metabolism, as well as in flavonoid synthesis and biological fixation nitrogen in cowpea crop are scarce. The aim of this study was to elucidate the role of Ni in metabolism, photosynthesis and nodulation of cowpea plants. A completely randomized experiment was performed in greenhouse, with cowpea plants cultivated under 0, 0.5, 1, 2, or 3 mg kg-1 Ni, as Ni sulfate. In the study the following parameters were evaluated: activity of urease, nitrate reductase, superoxide dismutase, catalase and ascorbate peroxidase; concentration of urea, n-compounds, photosynthetic pigments, flavonoids, H2O2 and MDA; estimative of gas exchange, and biomass as plants, yield and weight of 100 seeds. At whole-plant level, Ni affected root biomass, number of seeds per pot, and yield, increasing it at 0.5 mg kg-1 and leading to inhibition at 2-3 mg kg-1 (e.g. number of seeds per pot and nodulation). The whole-plant level enhancement by 0.5 mg Ni kg-1 occurred along with increased photosynthetic pigments, photosynthesis, ureides, and catalase, and decreased hydrogen peroxide concentration. This study presents fundamental new insights regarding Ni effect on N metabolism, and nodulation that can be helpful to increase cowpea yield. Considering the increasing population and its demand for staple food, these results contribute to the enhancement of agricultural techniques that increase crop productivity and help to maintain human food security.

Implementação do Programa Mais Médicos nos municípios do planalto norte catarinense: avanços, dificuldades e desafios / Implementation of the More Doctors Program in municipalities of the North Plateau of Santa Catarina: advances, difficulties, and challenges

Este estudo avalia os avanços, dificuldades e desafios inerentes à implementação do Programa Mais Médicos nos municípios do Planalto Norte Catarinense. Os coordenadores de Atenção Primária que aderiram ao Programa e receberam profissionais médicos vinculados ao Projeto Mais Médicos para o Brasil constituem a população de análise, respondente de questionário semiestruturado na modalidade de entrevista. A coleta de dados deu-se entre os meses de abril e junho do ano de 2015. Identificou-se, entre os avanços, a ampliação de acesso à saúde pública; entre as dificuldades, a comunicação entre usuários e os profissionais de língua estrangeira; e, entre os desafios, o engessamento burocrático e legislativo atinente à gestão dos recursos humanos. Os resultados apontam que o Programa Mais Médicos em fase inicial de implementação, embora apresentasse problemas burocráticos e gerenciais, permitiu a ampliação de acesso ao sistema de saúde.

This study evaluates the progress, difficulties, and challenges involved in implementing the More Doctors Program in the cities of the North Plateau of Santa Catarina. The Primary Care coordinators that have joined the program and received medical professionals involved in the More Doctors Project for Brazil constitute the population analysis, which answered a semi-structured questionnaire in interview mode. Data collection took place between April and June 2015. It was identified among the advances, expansion of access to public health; among the difficulties, communication between users and foreign language professionals; and among the challenges, bureaucratic and legislative inflexibility concerning human resources. The results show that the More Doctors program is in the early stage of implementation, and, although there are bureaucratic and managerial problems, it allowed the expansion of access to health care.

Is fatigue an important finding in patients with spinocerebellar ataxia type 10 (SCA10)?

Spinocerebellar ataxia type 10 (SCA10) is a rare dominantly inherited neurodegenerative disorder characterized by cerebellar ataxia, dysarthria, ocular dysmetria, and seizures in some populations. Fatigue has been described in SCA1, SCA3, but has not been objectively investigated in SCA10. Our aim is to investigate the presence and related causal factors of fatigue among SCA10 patients. Twenty-eight patients with SCA10 and matched healthy controls were included and assessed using the Scale for the Assessment and Rating of Ataxia (SARA), Modified Fatigue Impact Scale (MFIS), Beck Inventory Depression (BDI) and Epworth Sleepiness Scale (ESS). Fatigue was evidenced in 32% of SCA10 versus 3.6% for the control group (p = 0.005). The following independent variables were not significant predictors for MFIS-BR: duration of disease, SARA and BSS. Age at onset of disease (r = -0.307, p = 0.021) and EDS (r = -0.347, p = 0.014) were mild to moderate predictors of fatigue. Similar to other SCAs, fatigue is common in SCA10 patients, suggesting a possible role of a common topographic degenerative pattern in its pathophysiology.

Nonmotor symptoms in spinocerebellar ataxias (SCAs).

Nonmotor symptoms (NMS) have been increasingly recognized in a number of neurodegenerative diseases with a burden of disability that parallels or even surpasses that induced by motor symptoms. As NMS have often been poorly recognized and inadequately treated, much of the most recent developments in the investigation of these disorders has focused on the recognition and quantification of NMS, which will form the basis of improved clinical care for these complex cases. NMS have been only sparsely investigated in a limited number of spinocerebellar ataxias (SCAs), particularly SCA3, and have not been systematically reviewed for other forms of SCAs. The aim of the present study was to review the available literature on the presence of NMS among different types of SCAs.

Clinical and Genetic Evaluation of Spinocerebellar Ataxia Type 10 in 16 Brazilian Families.

Spinocerebellar ataxia type 10 (SCA10) is an autosomal dominant disorder in which patients have a slowly progressive cerebellar ataxia, with dysarthria, dysphagia, and epilepsy. The aims of this study were to characterize the phenotypic expression of SCA10 and to examine its genotype-phenotype relationships. Ninety-one Brazilian patients with SCA10 from 16 families were selected. Clinical and epidemiological data were assessed by a standardized protocol, and severity of disease was measured by the Scale for the Assessment and Rating of Ataxia (SARA). The mean age of onset of symptoms was 34.8 ± 9.4 years. Sixty-two (68.2%) patients presented exclusively with pure cerebellar ataxia. Only 6 (6.6%) of the patients presented with epilepsy. Patients with epilepsy had a mean age of onset of symptoms lower than that of patients without epilepsy (23.5 ± 15.5 years vs 35.4 ± 8.7 years, p = 0.021, respectively). All cases of intention tremor were in women from one family. This family also had the lowest mean age of onset of symptoms, and a higher percentage of SCA10 cases in women. There was a positive correlation between duration of disease and severity of ataxia (rho = 0.272, p = 0.016), as quantified by SARA. We did not find a statistically significant correlation between age of onset of symptoms and expansion size (r = - 0.163, p = 0.185). The most common clinical presentation of SCA10 was pure cerebellar ataxia. Our data suggest that patients with epilepsy may have a lower age of onset of symptoms than those who do not have epilepsy. These findings and the description of a family with intention tremor in women with earlier onset of symptoms draw further attention to the phenotypic variability of SCA10.

Spinocerebellar ataxias in Southern Brazil: Genotypic and phenotypic evaluation of 213 families.

OBJECTIVES: To describe and correlate the genotype and phenotype of patients diagnosed with SCAs in southern of Brazil. PATIENTS AND METHODS: Data were collected from the records of our ataxia outpatient clinic. We included 460 patients from 213 families, who were divided into four groups: SCA3, SCA10, Other SCAs and Undetermined. RESULTS: The most frequent type was SCA3 (45.7%), followed by SCA10 (18.3%), SCA2 (6.5%), SCA1 (4.3%), SCA7 (1.8%), and SCA6 (0.65%). The Undetermined group represented 22.8% of all patients. We observed a high frequency of SCA10 when compared to data from other studies, which can be explained by a founder effect in our region. Statistically significant differences were found for several symptoms when comparing SCA groups, especially lid retraction (p < 0.001), ophthalmoplegia (p < 0.001), visual loss (p < 0.001) and slow saccades (p < 0.001) which may help clinically differentiate SCAs and allow neurologists to request the right confirmatory genetic test and define prognosis. Also, the prevalence of epilepsy in SCA10 patients was lower than usual (4.8%), suggesting a genetic variation of the disease. CONCLUSION: Although SCA3 remains the most common, we observed a high frequency of SCA10 in our region. In addition, some symptoms and signs might help differentiate the SCAs.

Decarboxylation mechanisms of C4 photosynthesis in Saccharum spp.: increased PEPCK activity under water-limiting conditions.

BACKGROUND: C4 plants have been classified into three subtypes based on the enzymes used to decarboxylate C4 acids in the bundle sheath cells (NADP-ME, NAD-ME and PEPCK pathways). Evidences indicate that, depending on environmental factors, C4 plants may exhibit a certain degree of flexibility in the use of the decarboxylation mechanisms. In this context, the objective was to extend the knowledge on the degree of flexibility between the pathways of decarboxylation in sugarcane, a NADP-ME species, at different levels of water deficit. RESULTS: An experiment was carried out with two cultivars - RB92579 (tolerant to water deficit) and SP80-3280 (susceptible to water deficit) subjected to moderate level (- 1.5 to - 1.8 MPa), severe level (below - 2.0 MPa) and recovery (48 h after rehydration) and changes in the activities of the enzymes involved in the three C4 mechanisms and in gene expression were investigated. Our results showed that sugarcane uses the PEPCK pathway as a decarboxylation mechanism in addition to the NADP-ME, which was more evident under water deficit conditions for both cultivars. CONCLUSIONS: The results obtained here, show that sugarcane increases the use of the PEPCK pathway as a decarboxylation mechanism, in addition to the NADP-ME pathway, under conditions of water deficit, particularly in the tolerant cultivar.

Estimation of skeletal muscle mass in patients with spinocerebellar ataxia type 3 and 10.

BACKGROUND: Neurodegenerative diseases may progress to a level in which patients present spontaneous weight loss, resulting in increased falls and functional disabilities when the disease is associated with muscle mass depletion. OBJECTIVE: Evaluate the muscle compartment in patients presenting spinocerebellar ataxia (SCA) type 3 and 10. METHODS: Forty-six patients presenting SCA type 3 and 10 were assessed and 76 volunteers were selected to the control group. In order to evaluate the muscle compartment, muscle mass anthropometric measurements were assessed and total skeletal muscle mass calculated through a predictive equation. RESULTS: Women with SCA3 presented greater weight loss and muscle mass reduction compared to those with SCA10 and the control group. Among the predictive measurements, calf muscle circumference showed a more significant correlation with total skeletal muscle mass (p = 0.718). CONCLUSION: Patients presenting both types of ataxia did not show severe depletion in their nutritional status; however, those with SCA3 displayed greater weight loss and muscle mass reduction compared to the SCA10 group.

Olfactory Function in SCA10.

Although the main clinical manifestations of spinocerebellar ataxias (SCAs) result from damage of the cerebellum, other systems may also be involved. Olfactory deficits have been reported in other types of ataxias, especially in SCA3; however, there are no studies on olfactory deficits in SCA type 10 (SCA10). To analyze olfactory function of SCA10 patients compared with that of SCA3, Parkinson's, and healthy controls. Olfactory identification was tested in three groups of 30 patients (SCA10, SCA3, and Parkinson's disease (PD)) and 44 healthy controls using the Sniffin' Sticks (SS16) test. Mean SS16 score was 11.9 ± 2.9 for the SCA10 group, 12.3 ± 1.9 for the SCA3 group, 6.6 ± 2.8 for the PD group, and 12.1 ± 2.0 for the control group. Mean SS16 score for the SCA10 group was not significantly different from the scores for the SCA3 and control groups but was significantly higher than the score for the PD group (p < 0.001) when adjusted for age, gender, and history of smoking. There was no association between SS16 scores and disease duration in the SCA10 or SCA3 groups or number of repeat expansions. SS16 and Mini Mental State Examination scores were correlated in the three groups: SCA10 group (r = 0.59, p = 0.001), SCA3 group (r = 0.50, p = 0.005), and control group (r = 0.40, p = 0.007). We found no significant olfactory deficits in SCA10 in this large series.

Apomorphine in the treatment of Parkinson's disease: a review / O uso da apomorfina no tratamento da doença de Parkinson: revisão da literatura

ABSTRACT Optimizing idiopathic Parkinson's disease treatment is a challenging, multifaceted and continuous process with direct impact on patients' quality of life. The basic tenet of this task entails tailored therapy, allowing for optimal motor function with the fewest adverse effects. Apomorphine, a dopamine agonist used as rescue therapy for patients with motor fluctuations, with potential positive effects on nonmotor symptoms, is the only antiparkinsonian agent whose capacity to control motor symptoms is comparable to that of levodopa. Subcutaneous administration, either as an intermittent injection or as continuous infusion, appears to be the most effective and tolerable route. This review summarizes the historical background, structure, mechanism of action, indications, contraindications and side effects, compares apomorphine infusion therapy with other treatments, such as oral therapy, deep brain stimulation and continuous enteral infusion of levodopa/carbidopa gel, and gives practical instructions on how to initiate treatment.

RESUMO A optimização do tratamento da doença de Parkinson idiopática se faz um desafio, pois tem impacto direto na qualidade de vida do paciente. O melhor esquema terapêutico é o que permite o melhor controle motor com os menores efeitos adversos, através de terapêutica individualizada. A apomorfina é o único medicamento antiparkinsoniano que pode ser comparável à potência da levodopa no controle dos sintomas motores. Trata-se de um agonista dopaminérgico empregado na terapia de resgate em pacientes com flutuações motoras e também contribui para a melhora de muitos sintomas não motores. A via subcutânea, com injeções intermitentes, ou com infusão contínua, parece ser a melhor opção pela eficácia e tolerabilidade. Essa revisão resume aspectos históricos, estrutura da molécula, mecanismo de ação, indicação, contra-indicação e efeitos colaterais, compara a terapia de infusão com apomorfina com outros tratamentos, como a terapia oral, estimulação cerebral profunda e infusão enteral contínua de levodopa/carbidopa gel, e fornece instruções práticas de como iniciar o tratamento.

Dystonia in Patients with Spinocerebellar Ataxia 3 - Machado-Joseph disease: An Underestimated Diagnosis?

BACKGROUND: Spinocerebellar Ataxia type 3 (SCA3) or Machado-Joseph Disease (MJD) is characterized by cerebellar, central and peripheral symptoms, including movement disorders. Dystonia can be classified as hereditary and neurodegenerative when present in SCA3. OBJECTIVE: The objective of this study was to evaluate the dystonia characteristics in patients with MJD. METHOD: We identified all SCA3 patients with dystonia from the SCA3 HC-UFPR database, between December 2015 and December 2016.Their medical records were reviewed to verify the diagnosis of dystonia and obtain demographic and clinical data. Standardized evaluation was carried out through the classification of Movement Disorders Society of 2013 and Burke Fahn-Marsden scale (BFM). RESULTS: Amongst the presenting some common characteristics, 381 patients with SCA3, 14 (3.7%) subjects presented dystonia: 5 blepharospasm, 1 cervical dystonia, 3 oromandibular, 3 multifocal and 2 generalized dystonia. Regarding dystonia's subtypes, 71.4% had SCA3 subtype I and 28.6% SCA3 subtype II. The average age of the disease onset was 40±10.7 years; the SCA3 disease duration was 11.86± 6.13 years; the CAG repeat lengths ranged from 75 to 78, and the BFM scores ranged from 1.0 to 40. There was no correlation between the dystonia severity and CAG repeat lengths or the SCA3 clinical evolution. CONCLUSION: Dystonia in SCA3 is frequent and displays highly variable clinical profiles and severity grades. Dystonia is therefore a present symptom in SCA3, which may precede the SCA3 classic symptoms. Dystonia diagnosis is yet to be properly recognized within SCA3 patient.

Botulinum toxin for hereditary spastic paraplegia: effects on motor and non-motor manifestations.

Motor and non-motor manifestations are common and disabling features of hereditary spastic paraplegia (HSP). Botulinum toxin type A (Btx-A) is considered effective for spasticity and may improve gait in these patients. Little is known about the effects of Btx-A on non-motor symptoms in HSP patients. Objective To assess the efficacy of Btx-A on motor and non-motor manifestations in HSP patients. Methods Thirty-three adult patients with a clinical and molecular diagnosis of HSP were evaluated before and after Btx-A injections. Results Mean age was 41.7 ± 13.6 years and there were 18 women. Most patients had a pure phenotype and SPG4 was the most frequent genotype. The Btx-A injections resulted in a decrease in spasticity at the adductor muscles, and no other motor measure was significantly modified. In contrast, fatigue scores were significantly reduced after Btx-A injections. Conclusion Btx-A injections resulted in no significant functional motor improvement for HSP, but fatigue improved after treatment.

Botulinum toxin for hereditary spastic paraplegia: effects on motor and non-motor manifestations / Toxina botulínica nas paraplegias espásticas hereditárias: efeitos nas manifestações motoras e não-motoras

ABSTRACT Motor and non-motor manifestations are common and disabling features of hereditary spastic paraplegia (HSP). Botulinum toxin type A (Btx-A) is considered effective for spasticity and may improve gait in these patients. Little is known about the effects of Btx-A on non-motor symptoms in HSP patients. Objective To assess the efficacy of Btx-A on motor and non-motor manifestations in HSP patients. Methods Thirty-three adult patients with a clinical and molecular diagnosis of HSP were evaluated before and after Btx-A injections. Results Mean age was 41.7 ± 13.6 years and there were 18 women. Most patients had a pure phenotype and SPG4 was the most frequent genotype. The Btx-A injections resulted in a decrease in spasticity at the adductor muscles, and no other motor measure was significantly modified. In contrast, fatigue scores were significantly reduced after Btx-A injections. Conclusion Btx-A injections resulted in no significant functional motor improvement for HSP, but fatigue improved after treatment.

RESUMO Manifestações motoras e não motoras são comuns e incapacitantes nas paraparesias espásticas hereditárias (PEH). Toxina botulínica do tipo A (TB-A) é considerada eficaz no tratamento da espasticidade e pode melhorar a marcha nesses pacientes. Pouco se sabe sobre os efeitos da TB-A sobre sintomas não-motores. Objetivo avaliar a eficácia da TB-A sobre manifestações motoras e não-motoras nas PEH. Método trinta e três pacientes adultos com PEH foram avaliados antes e depois das aplicações de TB-A. Resultados A média de idade foi 41,7 ± 13,6 anos e havia 18 mulheres. A maioria dos pacientes portava a forma pura e o genótipo mais comum foi SPG4. Houve diminuição da espasticidade dos músculos adutores da coxa sem melhora da marcha. A pontuação da fadiga reduziu após as injeções. Conclusão As aplicações de TB-A não melhoraram a marcha nos pacientes mas a redução da fadiga foi significativa após o tratamento.

Apomorphine in the treatment of Parkinson's disease: a review.

Optimizing idiopathic Parkinson's disease treatment is a challenging, multifaceted and continuous process with direct impact on patients' quality of life. The basic tenet of this task entails tailored therapy, allowing for optimal motor function with the fewest adverse effects. Apomorphine, a dopamine agonist used as rescue therapy for patients with motor fluctuations, with potential positive effects on nonmotor symptoms, is the only antiparkinsonian agent whose capacity to control motor symptoms is comparable to that of levodopa. Subcutaneous administration, either as an intermittent injection or as continuous infusion, appears to be the most effective and tolerable route. This review summarizes the historical background, structure, mechanism of action, indications, contraindications and side effects, compares apomorphine infusion therapy with other treatments, such as oral therapy, deep brain stimulation and continuous enteral infusion of levodopa/carbidopa gel, and gives practical instructions on how to initiate treatment.

The thalidomide tragedy: the struggle for victims' rights and improved pharmaceutical regulation. / A tragédia da talidomida: a luta pelos direitos das vítimas e por melhor regulação de medicamentos.

The thalidomide tragedy in the late 1950s was a watershed moment for pharmaceutical regulation. New uses for this medication are being researched and implemented today. This article revisits the history of thalidomide and its consequences for regulation and for victims' rights in Brazil, based on a literature review, documentary analysis, and newspaper reports. The events highlighted herein show the lack of strong standards for safe medication use, as well as how the tragedy was transformed into a public problem through its divulgation in the press; the article also shows that the increasing mobilization of victims was essential in pushing for more effective drug regulations.

Resumo A tragédia da talidomida, no final dos anos 1950, constituiu um divisor de águas na regulação de medicamentos. Novos usos da droga estão sendo pesquisados e implementados atualmente. O artigo revisita a história da talidomida e seus desdobramentos no plano regulatório e dos direitos das vítimas no Brasil, com base em revisão de literatura, análise documental e reportes de jornais. Destaca que os eventos evidenciaram a carência de normas fortes para o uso seguro de medicamentos e mostra que a transformação dos acontecimentos em um problema público, mediante sua veiculação na mídia, e a crescente mobilização das vítimas foram forças essenciais que pressionaram por uma regulação de medicamentos mais efetiva.

Caffeine as symptomatic treatment for Parkinson disease (Café-PD): A randomized trial.

OBJECTIVE: To assess effects of caffeine on Parkinson disease (PD). METHODS: In this multicenter parallel-group controlled trial, patients with PD with 1-8 years disease duration, Hoehn & Yahr stages I-III, on stable symptomatic therapy were randomized to caffeine 200 mg BID vs matching placebo capsules for 6-18 months. The primary research question was whether objective motor scores would differ at 6 months (Movement Disorder Society-sponsored Unified Parkinson's Disease Rating Scale [MDS-UPDRS]-III, Class I evidence). Secondary outcomes included safety and tolerability, motor symptoms (MDS-UPDRS-II), motor fluctuations, sleep, nonmotor symptoms (MDS-UPDRS-I), cognition (Montreal Cognitive Assessment), and quality of life. RESULTS: Sixty patients received caffeine and 61 placebo. Caffeine was well-tolerated with similar prevalence of side effects as placebo. There was no improvement in motor parkinsonism (the primary outcome) with caffeine treatment compared to placebo (difference between groups -0.48 [95% confidence interval -3.21 to 2.25] points on MDS-UPDRS-III). Similarly, on secondary outcomes, there was no change in motor signs or motor symptoms (MDS-UPDRS-II) at any time point, and no difference on quality of life. There was a slight improvement in somnolence over the first 6 months, which attenuated over time. There was a slight increase in dyskinesia with caffeine (MDS-UPDRS-4.1+4.2 = 0.25 points higher), and caffeine was associated with worse cognitive testing scores (average Montreal Cognitive Assessment = 0.66 [0.01, 1.32] worse than placebo). CONCLUSION: Caffeine did not provide clinically important improvement of motor manifestations of PD (Class I evidence). Epidemiologic links between caffeine and lower PD risk do not appear to be explained by symptomatic effects. CLINICALTRIALSGOV IDENTIFIER: NCT01738178. CLASSIFICATION OF EVIDENCE: This study provides Class I evidence that for patients with PD, caffeine does not significantly improve motor manifestations.

A tragédia da talidomida: a luta pelos direitos das vítimas e por melhor regulação de medicamentos / The thalidomide tragedy: the struggle for victims' rights and improved pharmaceutical regulation

Resumo A tragédia da talidomida, no final dos anos 1950, constituiu um divisor de águas na regulação de medicamentos. Novos usos da droga estão sendo pesquisados e implementados atualmente. O artigo revisita a história da talidomida e seus desdobramentos no plano regulatório e dos direitos das vítimas no Brasil, com base em revisão de literatura, análise documental e reportes de jornais. Destaca que os eventos evidenciaram a carência de normas fortes para o uso seguro de medicamentos e mostra que a transformação dos acontecimentos em um problema público, mediante sua veiculação na mídia, e a crescente mobilização das vítimas foram forças essenciais que pressionaram por uma regulação de medicamentos mais efetiva.

Abstract The thalidomide tragedy in the late 1950s was a watershed moment for pharmaceutical regulation. New uses for this medication are being researched and implemented today. This article revisits the history of thalidomide and its consequences for regulation and for victims' rights in Brazil, based on a literature review, documentary analysis, and newspaper reports. The events highlighted herein show the lack of strong standards for safe medication use, as well as how the tragedy was transformed into a public problem through its divulgation in the press; the article also shows that the increasing mobilization of victims was essential in pushing for more effective drug regulations.

Death certificate data and causes of death in patients with parkinsonism.

INTRODUCTION: Assessment of variables related to mortality in Parkinson disease (PD) and other parkinsonian syndromes relies, among other sources, on accurate death certificate (DC) documentation. We assessed the documentation of the degenerative disorder on DCs and evaluated comorbidities and causes of death among parkinsonian patients. METHODS: Demographic and clinical data were systematically and prospectively collected on deceased patients followed at a tertiary movement disorder clinic. DCs data included the documentation of parkinsonism, causes, and place of death. RESULTS: Among 138 cases, 84 (60.9%) male, mean age 77.9 years, mean age of onset 66.7, and mean disease duration 10.9 years. Clinical diagnoses included PD (73.9%), progressive supranuclear palsy (10.9%), multiple system atrophy (7.2%), Lewy body dementia (7.2%) and corticobasal degeneration (0.7%). Psychosis occurred in 60.1% cases, dementia in 48.5%. Most PD patients died due to heterogeneous causes before reaching advanced stages. Non-PD parkinsonian patients died earlier due to causes linked to the advanced neurodegenerative process. PD was documented in 38.4% of DCs with different forms of inconsistencies. That improved, but remained significant when it was signed by a specialist. CONCLUSIONS: More than half of PD cases died while still ambulatory and independent, after a longer disease course and due to causes commonly seen in that age group. Deaths among advanced PD patients occurred due to causes similar to what we found in non-PD cases. These findings can be useful for clinical, prognostic and counseling purposes. Underlying parkinsonian disorders are poorly documented in DCs, undermining its' use as sources of data collection.

Nonmotor Symptoms in Patients with Spinocerebellar Ataxia Type 10.

Nonmotor symptoms (NMS) have been described in several neurodegenerative diseases but have not been systematically evaluated in spinocerebellar ataxia type 10 (SCA10). The objective of the study is to compare the frequency of NMS in patients with SCA10, Machado-Joseph disease (MJD), and healthy controls. Twenty-eight SCA10, 28 MJD, and 28 healthy subjects were prospectively assessed using validated screening tools for chronic pain, autonomic symptoms, fatigue, sleep disturbances, psychiatric disorders, and cognitive function. Chronic pain was present with similar prevalence among SCA10 patients and healthy controls but was more frequent in MJD. Similarly, autonomic symptoms were found in SCA10 in the same proportion of healthy individuals, while the MJD group had higher frequencies. Restless legs syndrome and REM sleep behavior disorder were uncommon in SCA10. The mean scores of excessive daytime sleepiness were worse in the SCA10 group. Scores of fatigue were higher in the SCA10 sample compared to healthy individuals, but better than in the MJD. Psychiatric disorders were generally more prevalent in both spinocerebellar ataxias than among healthy controls. The cognitive performance of healthy controls was better compared with SCA10 patients and MJD, which showed the worst scores. Although NMS were present among SCA10 patients in a higher proportion compared to healthy controls, they were more frequent and severe in MJD. In spite of these comparisons, we were able to identify NMS with significant functional impact in patients with SCA10, indicating the need for their systematic screening aiming at optimal treatment and improvement in quality of life.