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Background: Swallowing and feeding problems may occur with the progression of behavioral variant frontotemporal dementia (bvFTD) and can impair the anticipatory and oral preparatory phases of swallowing., Objective: To characterize swallowing problems and the feeding situation of patients with bvFTD and to correlate the swallowing problems with functionality, executive functions, cognitive and behavioral features., Methods: Consecutive outpatients with bvFTD in mild, moderate and severe dementia stages were recruited along with their caregivers. Patients and caregivers were screened with the following scales: "Mini-Mental State Examination", "Severe Mini-Mental State Examination", "FTLD-modified Clinical Dementia Rating", "Neuropsychiatric Inventory", "Frontal Assessment Battery", "Index of Independence in Activities of Daily Living", "Swallowing Rating Scale" and "Assessment of Feeding and Swallowing Difficulties in Dementia"., Results: Overall, thirty patients with bvFTD were included along with their caregivers. Patients with bvFTD showed feeding and swallowing difficulties such as: messy to eat, passivity, coughing and choking, difficulty with some food consistencies and with specific food. Swallowing problems in bvFTD correlated with impaired functionality (p<0.05) and cognition (p<0.05), executive dysfunction (p<0.01) and behavioral features (p<0.01). Caregivers had great difficulty in managing the feeding situation during mealtime, with different characteristics in each dementia stage., Conclusion: Patients with bvFTD had inappropriate speed eating, passivity, coughing and choking starting in the mild dementia stage, and these problems worsen in the severe stage. Such difficulties affected caregiver performance during mealtime. The correlations indicated that swallowing difficulties tend to follow cognitive and behavioral decline in patients with bvFTD.

Objective: Few studies have quantified the prevalence of restless legs syndrome (RLS) in patients with post-polio syndrome (PPS). Our objective was to assess the prevalence and severity of RLS in patients with PPS and to examine the demographic characteristics of this population., Method: This was a cross-sectional study conducted from April 2010 to May 2012 at the outpatient Neuromuscular Disorders clinic of Universidade Federal de São Paulo, São Paulo, Brazil. We evaluated 119 patients with PPS, consecutively recruited, and investigated for RLS based on the diagnostic criteria established by the International Restless Legs Syndrome Study Group (IRLSSG). Patients were evaluated with the Brazilian version of the IRLSSG severity scale., Results: The prevalence of RLS was 36% (n = 43; 32 women and 11 men). The ages at onset of RLS (median = 41 years) and PPS (median = 41 years) were concurrent, and the correlation between onset of symptoms of RLS and onset of symptoms of PPS was positive and very strong (Spearman r = 0.93, p = 0.01). The median RLS severity was 23 (range, 20-28). Low educational achievement and depression were predictive of RLS development., Conclusion: In the largest population of patients with PPS studied to date, our results indicate a high prevalence of RLS, marked disease severity, and concomitant onset of both conditions in many patients with PPS. Further studies are needed to elucidate a possible pathophysiologic mechanism linking these two conditions. We suggest that all post-polio patients with sensory and motor complaints in the legs be investigated for RLS., (Copyright © 2017 Elsevier B.V. All rights reserved.)

Background: Few studies have described characteristics of swallowing in primary progressive aphasia (PPA) and its variants., Objective: To describe and characterize swallowing and eating behaviors of patients with PPA, as well as their correlates with neuropsychiatric symptoms and patterns of communication., Methods: We studied 16 patients with PPA and 16 their caregivers. PPA was subdivided in agrammatic variant (PPA-G), semantic variant (PPA-S) and logopenic variant (PPA-L). All patients and their caregivers were screened with the following scales: "Assessment of Feeding and Swallowing Difficulties in Dementia", "Neuropsychiatric Inventory", and "Functional Outcome Questionnaire for Aphasia"., Results: Patients with PPA-S had diverse swallowing problems such as drooling of saliva or food, multiple swallows, delayed swallow and choking, all of which correlated with anxiety, apathy and aberrant motor behavior. Patients with PPA-G and PPA-L had choking and delayed swallow, respectively. Disturbances in eating behaviors were more frequent in the group with PPA-L, and they correlated with difficulties in patterns of communication., Conclusions: All variants showed swallowing difficulties and they were more frequent in PPA-S. Further studies with larger samples of patients are needed to better characterize swallowing problems and their consequences in the different variants of PPA.

Objective: It was to evaluate the degree of fatigue in patients with paralytic poliomyelitis (PP) and with post-polio syndrome (PPS), and correlate it with parameters of sleep and the circadian cycle., Methods: Thirty patients, 17 female (56.7%), participated in the study: they answered the Revised Piper Fatigue Scale and performed a nocturnal polysomnographic study. Eleven had PP (mean age±standard deviation of 47.9±6.4 years), and 19 had PPS (mean age±standard deviation of 46.4±5.6 years)., Results: Our study showed that fatigue was worse in the afternoon in the PP Group and had a progressive increase throughout the day in the PPS Group. We also observed compromised quality of sleep in both groups, but no statically significant difference was found in the sleep parameters measured by polysomnography., Conclusion: Fatigue has a well-defined circadian variation, especially in PPS Group. Poor sleep quality is associated with fatigue and, therefore, sleep disturbances should be evaluated and treated in this group of PPS.

Objective: To determine the frequency and clinical manifestations of patients with post-poliomyelitis syndrome (PPS) in a Brazilian division of neuromuscular disorders., Methods: A total of 167 patients with prior history of paralytic poliomyelitis was investigated for PPS, based on international diagnostic criteria. Other variables analyzed were: gender, race, age at poliomyelitis infection, age at PPS onset, and PPS symptoms., Results: One hundred and twenty-nine patients presented PPS, corresponding to 77.2% of the studied population. 62.8% were women and 37.2% were men. Mean age of patients with PPS at onset of PPS symptoms was 39.9±9.69 years. Their main clinical manifestations were: new weakness in the previously affected limbs (69%) and in the apparently not affected limbs (31%); joint pain (79.8%); fatigue (77.5%); muscle pain (76%); and cold intolerance (69.8%)., Conclusions: Most patients of our sample presented PPS. In Brazil, PPS frequency and clinical features are quite similar to those of other countries.

Objective: To determine the clinical correlates of the restless legs syndrome (RLS) in a Brazilian sleep disorders center., Methods: We retrospectively studied 118 patients with RLS from January, 2004, to December, 2010. The analyzed variables were: age at disease onset, gender, race, years of school instruction, primary and secondary RLS, and treatment options., Results: Among the studied patients, 83.9% were women with a female/male sex ratio of 5:1. Mean age of the patients at symptom onset ± standard deviation was 41.7±17.9 years-old. The primary RLS was found in 85% of patients. The other 15% remainders consisted of secondary forms, and they were associated with neuropathy, iron deficiency anemia, end-stage renal disease, or Parkinson's disease. Drug therapy for RLS was introduced in 67% of patients., Conclusions: Most patients presented primary RLS with an early disease onset. Further epidemiological studies are welcomed to provide better information on secondary RLS in Brazil.

Background: Few studies have described the occurrence of restless legs syndrome in post-polio syndrome., Methods: We studied 10 consecutive patients with post-polio syndrome and symptoms of restless legs syndrome. We look at demographic, clinical and laboratorial data., Results: A remarkable finding was the concomitant onset of symptoms of both diseases, suggesting a possible underlying mechanism. Severity of restless legs symptoms was moderate to very severe., Conclusion: Epidemiological studies with larger samples are needed to better establish the relationship and the incidence of restless legs syndrome in post-polio syndrome., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Clinical features of pontine infarction depend on the topography of vascular lesion and most remarkably sometimes the same topographic region can lead to different clinical syndromes (e.g., dorsal pontine tegmentum). In this report we describe an elderly patient with acute dorsal pontine infarction leading to a unique syndrome of bilateral horizontal gaze palsy and unilateral peripheral facial paralysis. We propose that this syndrome could be included as a part of a continuum that involves one-and-a-half syndrome, eight-and-a-half syndrome, and other variants of pontine tegmentum infarction.

Objective: To analyze the demographic features of the population sample, the time of headache complaint until first consultation and the diagnosis of primary and secondary headaches., Method: 3328 patients were analyzed retrospectively and divided according to gender, age, race, school instruction, onset of headache until first consultation and diagnosis(ICHD-II, 2004)., Results: Sex ratio (Female/Male) was 4:1, and the mean age was 40.7+/-15 years, without statistical differences between sexes. Approximately 65% of the patients were white and 55% had less than eight years of school instruction. Headache complaint until first consultation ranged from 1 to 5 years in 32.99% patients. The most prevalent diagnosis were migraine (37.98%), tension-type headache-TTH (22.65%) and cluster headache (2.73%)., Conclusion: There are few data on epidemiological features of headache clinic populations, mainly in developing countries. According to the literature, migraine was more frequent than TTH. It is noteworthy the low school instruction of this sample and time patient spent to seek for specialized attention. Hypnic headache syndrome was seen with an unusual frequency.