The cerebellum in Parkinson's disease and parkinsonism in cerebellar disorders

Universidade Federal de São Paulo, Ataxia Unit, Dept Neurol, São Paulo, BrazilUniversidade Federal de São Paulo, Ataxia Unit, Dept Neurol, São Paulo, BrazilWeb of Scienc

Conceitos Atuais No Tratamento Das Ataxias Hereditárias

Hereditary ataxias (HA) represents an extensive group of clinically and genetically heterogeneous neurodegenerative diseases, characterized by progressive ataxia combined with extra-cerebellar and multi-systemic involvements, including peripheral neuropathy, pyramidal signs, movement disorders, seizures, and cognitive dysfunction. There is no effective treatment for HA, and management remains supportive and symptomatic. In this review, we will focus on the symptomatic treatment of the main autosomal recessive ataxias, autosomal dominant ataxias, X-linked cerebellar ataxias and mitochondrial ataxias. We describe management for different clinical symptoms, mechanismbased approaches, rehabilitation therapy, disease modifying therapy, future clinical trials and perspectives, genetic counseling and preimplantation genetic diagnosis. © 2016, Associacao Arquivos de Neuro-Psiquiatria. All rights reserved.74324425

Astrometry of the main satellites of Uranus: 18 years of observations

We determine accurate positions of the main satellites of Uranus: Miranda, Ariel, Umbriel, Titania, and Oberon. Positions of Uranus, as derived from those of these satellites, are also determined. The observational period spans from 1992 to 2011. All runs were made at the Pico dos Dias Observatory, Brazil. We used the software called Platform for Reduction of Astronomical Images Automatically (PRAIA) to minimise (digital coronography) the influence of the scattered light of Uranus on the astrometric measurements and to determine accurate positions of the main satellites. The positions of Uranus were then indirectly determined by computing the mean differences between the observed and ephemeris positions of these satellites. A series of numerical filters was applied to filter out spurious data. These filters are mostly based on the comparison between the positions of Oberon with those of the other satellites and on the offsets as given by the differences between the observed and ephemeris positions of all satellites. We have, for the overall offsets of the five satellites, -29 (+/-63) mas in right ascension and -27 (+/-46) mas in declination. For the overall difference between the offsets of Oberon and those of the other satellites, we have +3 (+/-30) mas in right ascension and -2 (+/-28) mas in declination. Ephemeris positions for the satellites were determined from DE432+ura111. Comparisons using other modern ephemerides for the solar system -INPOP13c- and for the motion of the satellites -NOE-7-2013- were also made. They confirm that the largest contribution to the offsets we find comes from the motion of the barycenter of the Uranus system around the barycenter of the solar system, as given by the planetary ephemerides. Catalogues with the observed positions are provided.Comment: 13 pages, 21 figure

Ataxia espástica autossômica recessiva de Charlevoix-Saguenay (ARSACS): aspectos clínicos e de neuroimagem típicos em uma família brasileira

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a neurodegenerative disorder characterized by late-infantile onset spastic ataxia and other neurological features. ARSACS has a high prevalence in northeastern Quebec, Canada. Several ARSACS cases have been reported outside Canada in recent decades. This is the first report of typical clinical and neuroimaging features in a Brazilian family with probable diagnosis of ARSACS.A ataxia espástica autossômica recessiva de Charlevoix-Saguenay (ARSACS) é doença degenerativa do sistema nervoso, caracterizada por ataxia associada a espasticidade, entre outras manifestações neurológicas, de início na infância. A doença tem alta prevalência na região de Quebec, no Canadá. Muitos relatos de ARSACS têm sido descritos fora do Canadá nas últimas décadas. Nesse artigo, relatamos a primeira descrição dos aspectos clínicos e de neuroimagem típicos em uma família brasileira com provável diagnóstico de ARSACS.Universidade Federal de São Paulo (UNIFESP) Division of General Neurology and Ataxias Department of Neurology and NeurosurgeryUniversidade Federal de São Paulo (UNIFESP) Departament of Diagnostic ImagingUNIFESP, Division of General Neurology and Ataxias Department of Neurology and NeurosurgeryUNIFESP, Departament of Diagnostic ImagingSciEL

Reliable Classifier to Differentiate Primary and Secondary Acute Dengue Infection Based on IgG ELISA

Dengue virus infection causes a wide spectrum of illness, ranging from sub-clinical to severe disease. Severe dengue is associated with sequential viral infections. A strict definition of primary versus secondary dengue infections requires a combination of several tests performed at different stages of the disease, which is not practical.We developed a simple method to classify dengue infections as primary or secondary based on the levels of dengue-specific IgG. A group of 109 dengue infection patients were classified as having primary or secondary dengue infection on the basis of a strict combination of results from assays of antigen-specific IgM and IgG, isolation of virus and detection of the viral genome by PCR tests performed on multiple samples, collected from each patient over a period of 30 days. The dengue-specific IgG levels of all samples from 59 of the patients were analyzed by linear discriminant analysis (LDA), and one- and two-dimensional classifiers were designed. The one-dimensional classifier was estimated by bolstered resubstitution error estimation to have 75.1% sensitivity and 92.5% specificity. The two-dimensional classifier was designed by taking also into consideration the number of days after the onset of symptoms, with an estimated sensitivity and specificity of 91.64% and 92.46%. The performance of the two-dimensional classifier was validated using an independent test set of standard samples from the remaining 50 patients. The classifications of the independent set of samples determined by the two-dimensional classifiers were further validated by comparing with two other dengue classification methods: hemagglutination inhibition (HI) assay and an in-house anti-dengue IgG-capture ELISA method. The decisions made with the two-dimensional classifier were in 100% accordance with the HI assay and 96% with the in-house ELISA.Once acute dengue infection has been determined, a 2-D classifier based on common dengue virus IgG kits can reliably distinguish primary and secondary dengue infections. Software for calculation and validation of the 2-D classifier is made available for download

Apresentação clínica de SCA2 como uma síndrome ataxia-parkinsonismo-doença do neurônio motor

Universidade Federal de São Paulo (UNIFESP) Department of Neurology and Neurosurgery Division of General Neurology and AtaxiasUNIFESP, Department of Neurology and Neurosurgery Division of General Neurology and AtaxiasSciEL