Dystonia is a neurological disorder characterized by intermittent or sustained muscle contractions that cause abnormal, usually repetitive, movements and postures. Dystonic movements can be tremulous and twisting and often follow a pattern. They are frequently associated with overflow muscle activation and may be triggered or worsened by voluntary action. Most voluntary muscles can be affected and, in the case of the neck muscles, the condition is referred to as cervical dystonia (CD), the most common form of dystonia. The high incidence of pain distinguishes CD from other focal dystonias and contributes significantly to patient disability and low quality of life. Different degrees of pain in the cervical region are reported by more than 60% of patients, and pain intensity is directly related to disease severity. Botulinum toxin (BoNT) is currently considered the treatment of choice for CD and can lead to an improvement in pain and dystonic symptoms in up to 90% of patients. The results for BoNT/A and BoNT/B are similar. The complex relationship between pain and dystonia has resulted in a large number of studies and more comprehensive assessments of dystonic patients. When planning the application of BoNT, pain should be a key factor in the choice of muscles and doses. In conclusion, BoNT is highly effective in controlling pain, and its analgesic effect is sustained for a long time in most CD patients.
6 August 2019 in Movement Disorders Clinical Practice
Background: The frequency and presentation of each of the most common forms of spinocerebellar ataxias (SCAs) varies widely. In the case of the Americas, this diversity is particularly dynamic given additional social, demographic, and cultural characteristics.Objective: To describe the regional prevalence and clinical phenotypes of SCAs throughout the continent.Methods: A literature search was performed in both MEDLINE and LILACS databases. The research was broadened to include the screening of reference lists of systematic review articles for additional studies. Investigations dating from the earliest available through 2019. Only studies in English, Portuguese, and Spanish were included. We analyzed publications with genetically confirmed cases only, ranging from robust samples with epidemiological data to case reports and case series from each country or regions.Results: Overall, SCA3 is the most common form in the continent. Region‐specific prevalence and ranking of the common forms vary. On the other hand, region‐specific phenotypic variations were not consistently found based on the available literature analyzed, with the exception of the absence of epilepsy in SCA10 consistently described in a particular cluster of cases in South Brazil.Conclusion: Systematic, multinational studies analyzing in detail the true frequencies of SCAs across the Americas as well as distinct clinical signs and clues of each form would be ideal to look for these potential variations.
1 January 2018 in Parkinsonism & Related Disorders
Alzheimer’s disease (AD) is the most frequent cause of dementia. This work aims to assess the effectiveness of reality orientation (RO), a traditional, extensively documented cognitive enhancement technique, when combined with acetylcholinesterase inhibitors in the treatment of AD. Fourteen patients with AD having mild to moderate dementia receiving standard treatment with donepezil were randomly assigned to control and treatment groups. Patients in the treatment group were submitted to weekly RO sessions for 6 months. Cognitive outcomes were assessed based on scores in the Consortium to Establish a Registry for Alzheimer’s Disease (CERAD) neuropsychological battery and the Clock Drawing Test (CDT). Mean CERAD neuropsychological battery, Mini-Mental State Examination (MMSE), and CDT scores improved in the treatment group and worsened in the control group. A number of CERAD neuropsychological battery and MMSE scores were statistically significant. Our findings suggest that RO is a valuable long-term complementary intervention for dementia in AD.