Huntington's disease (HD) is a neurodegenerative disorder characterized by chorea, behavioral disturbances and dementia, caused by a pathological expansion of the CAG trinucleotide in the HTT gene. Several patients have been recognized with the typical HD phenotype without the expected mutation. The objective of this study was to assess the occurrence of diseases such as Huntington's disease-like 2 (HDL2), spinocerebellar ataxia (SCA) 1, SCA2, SCA3, SCA7, dentatorubral-pallidoluysian atrophy (DRPLA) and chorea-acanthocytosis (ChAc) among 29 Brazilian patients with a HD-like phenotype. In the group analyzed, we found 3 patients with HDL2 and 2 patients with ChAc. The diagnosis was not reached in 79.3% of the patients. HDL2 was the main cause of the HD-like phenotype in the group analyzed, and is attributable to the African ancestry of this population. However, the etiology of the disease remains undetermined in the majority of the HD negative patients with HD-like phenotype.

Objective To validate the Clinical Gait and Balance Scale (GABS) for a Brazilian population of patients with Parkinson's disease (PD) and to compare it to the Berg Balance Scale (BBS). Methods One hundred and seven PD patients were evaluated by shortened UPDRS motor scale (sUPDRSm), Hoehn and Yahr (HY), Schwab and England scale (SE), Falls Efficacy Scale International (FES-I), Freezing of Gait Questionnaire (FOG-Q), BBS and GABS. Results The internal consistency of the GABS was 0.94, the intra-rater and inter-rater reliability were 0.94 and 0.98 respectively. The area under the receiver operating characteristic (ROC) curve was 0.72, with a sensitivity of 0.75 and specificity of 0.6, to discriminate patients with a history of falls in the last twelve months, for a cut-off score of 13 points. Conclusions Our study shows that the Brazilian version of the GABS is a reliable and valid instrument to assess gait and balance in PD.

Background: Theory of Mind (ToM) is the ability to attribute mental states to oneself and others. Individuals with a brain lesion following a stroke exhibit a compromised ability to perform ToM tasks. Objective: To analyze studies that evaluated ToM in stroke survivors considering the lesion localization and performance on ToM tests. Methods: The searches were carried out until November 28, 2018, using the following search terms: “social cognition” or “Theory of Mind” and “stroke”. Searches were conducted in the PubMed, PsycInfo, Web of Science and Scopus data bases. The initial search led to the retrieval of 425 articles. After the exclusion of duplicates and the analysis of the titles, abstracts and full texts, 20 articles were selected for the present review. Results: The studies showed that patients with lesion in the right hemisphere present lower performance on ToM tasks compared to those with lesion in the left hemisphere. In addition, patients with lesion in the right hemisphere presented significant impairment in the performance on ToM tasks compared to healthy individuals. Furthermore, the studies that evaluated lesions in specific regions such as temporal lobe, prefrontal cortex, posterior parietal cortex, and temporo-parietal junction, indicated a significant deficit in ToM performance of these patients compared to healthy individuals. Discussion: This review showed that stroke survivors have a poor performance on ToM tasks. The right hemisphere and prefrontal cortex seem to be associated with the deficit of this ability.

ABSTRACT Introduction: A diagnosis of rapid eye movement sleep behavior disorder (RBD) currently requires confirmation with polysomnography (PSG). However, PSG may not be sufficiently available. In these situations, a clinical diagnostic measure might be useful. Objective: To validate the Brazilian Portuguese version of RBD screening questionnaire (RBDSQ) for patients with Parkinson’s disease (PD). Methods: Using detailed clinical interviews and PSG analysis (diagnostic gold standard), a convenience sample of 69 subjects was divided into the following subgroups: patients with PD and RBD (PD+RBD; n=50) and patients with PD alone (PD-RBD; n=19). Results: RBDSQ-BR showed adequate internal consistency (Cronbach’s α=0.809) and, except for item 8, adequate item-test correlation. The retest performed in a second sample (n=13, consecutive) showed high agreement for total score (intraclass correlation coefficient, ICC=0.863) and acceptable agreement for items 2, 3, 6.2, 6.3, 7, and 8 (K>0.60). The receiver operating characteristic (ROC) curve analysis had an area under the curve (AUC) of 0.728. A cut-off score of 4 enabled the correct diagnosis of 76.8% subjects and provided the best balance between sensitivity (84%) and specificity (57.9%), with a 2.0 likelihood ratio of a positive result (LR+) and a 0.3 likelihood ratio of a negative result (LR-). Items 2 and 6.2 had 84.2% specificity and 3.2 LR+. Combined items 1+2+6.2, 2+6.1, and 6.1+6.2 increased the specificity to 94.7%, with LR+ ranging from 6.1 to 7.6. Conclusions: RBDSQ-BR is a reliable instrument, which may be useful for RBD diagnosis of Brazilian patients with PD. The instrument is also valid and may help in a better selection of cases for a more detailed clinical evaluation or even PSG analysis.

ABSTRACT The Guidelines for Stroke Rehabilitation are the result of a joint effort by the Scientific Department of Neurological Rehabilitation of the Brazilian Academy of Neurology aiming to guide professionals involved in the rehabilitation process to reduce functional disability and increase individual autonomy. Members of the group participated in web discussion forums with predefined themes, followed by videoconference meetings in which issues were discussed, leading to a consensus. These guidelines, divided into two parts, focus on the implications of recent clinical trials, systematic reviews, and meta-analyses in stroke rehabilitation literature. The main objective was to guide physicians, physiotherapists, speech therapists, occupational therapists, nurses, nutritionists, and other professionals involved in post-stroke care. Recommendations and levels of evidence were adapted according to the currently available literature. Part I discusses topics on rehabilitation in the acute phase, as well as prevention and management of frequent conditions and comorbidities after stroke.

The Guidelines for Stroke Rehabilitation are the result of a joint effort by the Scientific Department of Neurological Rehabilitation of the Brazilian Academy of Neurology aiming to guide professionals involved in the rehabilitation process to reduce functional disability and increase individual autonomy. Members of the group participated in web discussion forums with predefined themes, followed by videoconference meetings in which issues were discussed, leading to a consensus. These guidelines, divided into two parts, focus on the implications of recent clinical trials, systematic reviews, and meta-analyses in stroke rehabilitation literature. The main objective was to guide physicians, physiotherapists, speech therapists, occupational therapists, nurses, nutritionists, and other professionals involved in post-stroke care. Recommendations and levels of evidence were adapted according to the currently available literature. Part I discusses topics on rehabilitation in the acute phase, as well as prevention and management of frequent conditions and comorbidities after stroke.