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Friedrich Nietzsche (1844-1900) was a remarkable philologist-philosopher while remaining in a condition of ill-health. Issues about his wandering/disruptive behavior that might be a consequence and/or protection against his cognitive decline and multifaceted disease are presented. The life complex that raises speculations about its etiology is constituted by: insight, creativity and wandering behavior besides several symptoms and signs of disease(s), mainly neurological one. The most important issue to be considered at the moment is not the disease diagnosis (Lissauer's general paresis or CADASIL, e.g.), but the probable Nietzsche's great cognitive reserve linked to the multifactorial etiology (genetic and environmental), and shared characteristics both to creativity and psychopathology. This makes any disease seems especial regarding Nietzsche, and whichever the diagnostic hypothesis has to consider the Nietzsche's unique background to express any disease(s).

The year of 2014 is the birth centenary of Aristides Azevedo Pacheco Leão (1914-1993), and also marks seventy years of the publication of his discovery of the novel electrophysiological phenomenon, named by him "spreading depression" (SD), soon designated "Leão's wave" or "Leão's spreading depression". This was a remarkable scientific milestone, and the author must be celebrated for this achievement, as the studies he triggered proceeded worldwide, with new concepts, as spreading depolarization, until the present days. Robust experimental and clinical evidence emerged to suggest that these and related electrophysiological phenomena are involved in the mechanisms of migraine aura, acute cerebrovascular diseases, traumatic brain injury, transient global amnesia, epileptic seizures, and their pathophysiological characteristics come to offer new therapeutic perspectives. He was a remarkable and complex personality, and the authors remit the readers to a paper where his personal life is contemplated.

Some aspects of a dark period in the history of the modern neurology, that of the World War I (WWI), are here remembered, mainly by the neurological French School participation . Some personalities and their works related to the WWI are presented such as Joseph Babinski, Jules Froment, Clovis Vincent, Jules Joseph Dejerine, Augusta Déjérine-Klumpke, Jules Tinel, Pierre Marie, Achille Alexandre Souques, Charles Foix, and Georges Guillain.

The works of William Shakespeare (1564-1616), the greatest dramatist and poet of the English language, reflect several cultural values of the Western world which are also shared by other cultures. On his 450 th birthday, many of his concepts are admired as descriptions of human feelings and neurological phenomena, demonstrating his insights into what it is today considered cognitive neuroscience.

Andreas Vesalius (1514-1564) is considered the Father of Modern Anatomy, and an authentic representative of the Renaissance. His studies, founded on dissection of human bodies, differed from Galeno, who based his work on dissection of animals, constituted a notable scientific advance. Putting together science and art, Vesalius associated himself to artists of the Renaissance, and valued the images of the human body in his superb work De Humani Corporis Fabrica.This paper aims to honor this extraordinary European Renaissance physician and anatomist, who used aesthetic appeal to bind text and illustration, science and art. His achievements are highlighted, with an especial attention on neuroanatomy. Aspects about his personal life and career are also focused.

Unlabelled: Children with epilepsy present significant problems concerning attention and comorbidity with attention deficit hyperactivity disorder (ADHD)., Objective: To determine the prevalence of attention complaints, ADHD diagnosis and attention profile in a sample of children and adolescents with idiopathic epilepsy., Method: 36 children and adolescents with idiopathic epilepsy and 37 genre and age matched healthy controls underwent several procedures to diagnose their neuropsychological profile and comorbidity with ADHD., Results: The prevalence of ADHD was higher in patients with epilepsy [χ2= 4.1, p = 0.043, 6 (16.7%) vs 1 (2.7%)], with worse results in attention related WISC items and factors in patients with epilepsy comparing to the controls, but not between patients with and without ADHD. Clinical characteristics did not influence those results., Conclusion: This study found a greater prevalence of problems wih attention in pediatric patients with idiopathic epilepsy, but not a distinct profile between those with or without ADHD.

Initially the trajectory of the historical forerunners and conceptions of senile dementia are briefly presented, being highlighted the name of Alois Alzheimer who provided clinical and neuropathological indicators to differentiate a group of patients with Senile dementia. Alzheimer's examination of Auguste D's case, studied by him with Bielschowsky's silver impregnation technique, permitted to identify a pathological marker, the intraneuronal neurofibrillary tangles, characterizing a new disease later named after him by Kraepelin - Alzheimer's disease. Over the time this disorder became one of the most important degenerative dementing disease, reaching nowadays a status that may be considered as epidemic.

Objective: To compare the executive functions of children and adolescents with idiopathic epilepsy with a control group and to correlate with clinical data, intelligence and academic performance., Method: Cross-sectional, descriptive and analytical study. Thirty-one cases and thirty-five controls were evaluated by the WCST (Wisconsin Card Sorting Test).The results were compared with clinical data (seizure type and frequency, disease duration and number of antiepileptic drugs used), IQ (WISC-III) and academic performance (APT)., Results: Patients with epilepsy had poorer executive function scores. There was no positive linear correlation between test scores and epilepsy variables. There was a positive association between academic performance and some executive function results., Conclusion: Children with well controlled idiopathic epilepsy may show deficits in executive functions in spite of clinical variables. Those deficits may influence academic performance.

Unlabelled: The relationships among suicidal ideation, sleep, depression, anxiety, and effects on epilepsy require more research., Objective: The aim of this study was to estimate the prevalence of suicidal ideation in outpatients with epilepsy, and relate this to sleep quality, daytime sleepiness, depression, and anxiety., Method: Ninety-eight non-selected patients were evaluated. The subjects were classified as "suicidal ideators" or "non-ideators", based on their response to item 9 of the Beck Depression Inventory., Results: The prevalence of suicidal ideation was 13.3% (χ2=50.46, p<0.001). The differences between cases with or without suicidal ideation were statistically significant in relation to sleep quality (p=0.005) and symptoms of depression (p=0.001) and anxiety (p=0.002)., Conclusion: Our results revealed that depression and anxiety were associated with sleep quality, daytime sleepiness, and suicidal ideation and that depression and sleep disturbance were good predictors of suicide in subjects with epilepsy.

Claude Bernard (1813-1878) followed two main research paths: the chemical and physiological study of digestion and liver function, along with experimental section of nerves and studies on sympathetic nerves. Curare studies were, for example, of longstanding interest. His profound mental creativity and hand skillfulness, besides methodology quality, directed his experiments and findings, mainly at the Collège de France. His broader and epistemological concerns were carried out at the Sorbonne and later at the Muséum national d'Histoire naturelle. His insight gave clues to define the " milieu intérieur", later known as "homeostasis", and grasp the brain complexity. Bernard followed and surpassed his master François Magendie who also fought against dogmas and laid the foundations of experimental medicine, and its main heinous tool - vivisection. Bernard created the methodological bases of experimental medicine, and collected honors as a renowned researcher.

The main objective of this paper is to present the importance of hysteria on Babinski's oeuvre, and the conceptions of pithiatism from Babinski until the one of conversion disorder. Babinski gave a mental basis for hysteria in the place of Charcot's encephalopatic one, and several important semiotic tools to differentiate organic from hysterical manifestations based on studies from 1893-1917/8. His teachings were spread worldwide, and in Brazil they were also appreciated in the work on hysteria by Antonio Austregesilo, the first Brazilian neurology chairman. The neurobiological basis of hysteria conceived by Charcot is nowadays reappraised, and Babinski's neurosemiological contribution is everlasting. The patients believed to be hysterical, and the two outstanding neurologists, Charcot and Babinski, gave support for the development of the modern neurology.

Brown-Séquard, a remarkable medical personality of the 19 th century, was born in a small island of the Indian Ocean. He travelled over the world exerting his skills: a successful physician, and an innovative researcher, with a very ample range of interests. His favored subject was the nervous system. The spinal cord was studied extensively, with novel and important discoveries on the sensory pathways. He identified cases with spinal cord hemisection, and described the clinical presentation corresponding to a syndrome which bears his name (Brown-Séquard syndrome), for which he is best known among neurologists. Regarding the brain, he proposed nine mental and physical functions (organs) related to dynamically interconnected cell clusters, in harmony with the " réseau de cellules anastomosées ", " activités dynamogeniques et inhibitrices ", and " action à distance " concepts. Finally, he is considered by some as the "father" of endocrinology, due to his studies on glands and their secretions.

French medicine was of the utmost importance for the birth of modern medicine and neurology in the 19th century. Innovative approaches, such as examination at the bedside, the use of the stethoscope, techniques of auscultation, palpation, and close patient examination, besides emphasis on anatomical-clinical correlation and observation of the outcome of the disease, were put into practice. French medicine offered professional training and incentives for the beginnings of Brazilian neurology and psychiatry. Returning from France, many Brazilian physicians implemented what they had learned, mainly in Paris. The most important pupils of the French neurology schools in Brazil during the 19th century and first half of the 20th century include names like Antonio Austregesilo, Aloysio de Castro, Enjolras Vampré, and Deolindo Couto, founders of the leading Brazilian neurological schools, directly influenced by Dejerine, Pierre Marie, Guillain and Babinski.

Jean-Martin Charcot was a pioneer in a variety of subjects, including nervous system diseases; anatomy; physiology; pathology; and diseases of ageing, joints, and lungs. His medical achievements were mainly based on his anatomopathological proficiency, his observation, and his personal thoroughness that favored the delineation of the nosology of the main neurological diseases, including multiple sclerosis, amyotrophic lateral sclerosis, Parkinson's disease, peroneal muscular atrophy, and hysteria/epilepsy. The link of this anatomoclinical method with iconographic representations and theatrical lessons, and the rich bibliographical documentations, carried out in a crowded barn for diseased people--Salpetrière Hospital, were the basis of his achievements, which are still discussed 120 years after his death.

We present the common milestones of the neurology and psychiatry in Brazil, but, previously, they were summarized in the European matrix. The main psychiatric and neurological historical interceptions may be recognized by the terms neurology, psychiatry, alienism, neurosis and insanity; its organogenesis or socio- and psychogenetic basis; and its proximity or distance from internal medicine.

Objective: It was to evaluate the relationships between fatigue and excessive daytime sleepiness, sleep quality, depression and anxiety., Methods: This was a cross-sectional study on 98 unselected adult patients with epilepsy (PWE) at a tertiary center. It used clinical-sociodemographic characteristics, fatigue measured by the SF-36 vitality subscale (VsSF-36) and the Epworth Sleepiness Scale, Pittsburgh Sleep Quality Index and Beck Depression and Anxiety Inventories., Results: We observed that our patients presented vitality or fatigue similar to drivers with chronic headaches evaluated in the same city. Fatigue was related to depression, anxiety and sleep quality, but not to daytime sleepiness. A multiple linear regression analysis was conducted and we observed that fatigue was independently correlated with depression and quality of sleep. However, no significant difference was observed regarding seizure frequency or number of antiepileptic drugs., Conclusion: Fatigue needs to be studied more in PWE, and its risk factors need to be controlled, along with sleep quality and psychiatric disorders.

The concept of shock apparently emerged in the middle of the 18th century (Whyett) as an occurrence observed experimentally after spinal cord transection, and identified as "shock" phenomenon one century later (Hall). The concept was extended (Brown-Séquard) and it was suggested that brain lesions caused functional rupture in regions distant from the injured one ("action à distance"). The term "diaschisis" (von Monakow), proposed as a new modality of shock, had its concept broadened, underpinned by observations of patients, aiming at distinguishing between symptoms of focal brain lesions and transitory effects they produced, attributable to depression of distant parts of the brain connected to the injured area. Presently, diaschisis is related mainly to cerebrovascular lesions and classified according to the connection fibers involved, as proposed by von Monakow. Depression of metabolism and blood flow in regions anatomically separated, but related by connections with the lesion, allows observing diaschisis with neuroimaging.

The intersection between infectious diseases, poverty and mental disease was an important subject to the Brazilian Neuropsychiatry in the early 19th century. José Martins da Cruz Jobim (1802-1878) was engaged in a hygienist approach based on symptomatological and anatomopathological studies. He wrote "Insânia loquaz" (Loquacious insanity), 1831, the first written text about mental illness in Brazil, founded on clinical and pathological data, compatible with tuberculous meningitis. Thus, Jobim deserves the title of the first neuropsychiatrist in Brazil. The authors critically studied the clinical history and the autopsy findings of his cases, and the main health policies at the time.

It is reported the Brazilian Neurology birth (1912), that has as the hallmark its first Neurology Cathedra of Rio de Janeiro, and the links between Neurology and Psychiatry, besides the main medical protagonists at that time in Rio de Janeiro: João Carlos Teixeira Brandão (1854-1921), first professor of the cathedra of Clinical Psychiatry and Nervous Diseases (1883-1921); Juliano Moreira (1873-1933), the founder of the Brazilian scientific Psychiatry and director of the Hospício Nacional de Alienados (National Hospice for the Insane) (1903-1930); Antônio Austregésilo Rodrigues de Lima (1876-1960), first professor of the cathedra of Neurology, considered the father of the Brazilian Neurology. Aloysio de Castro (1881-1959) was a great Brazilian neurosemiologist at that time. Austregésilo practiced both disciplines, Neurology and Psychiatry, and like Jean-Martin-Charcot, he was very interested in a typically psychiatric disorder, the hysteria. It is also considered in this paper the first Brazilian authors of Neurology and/or Psychiatric texts and the places where Neurology was initially developed by the main founders: Hospício Nacional de Alienados, Santa Casa de Misericórdia do Rio de Janeiro and Policlínica Geral do Rio de Janeiro.

Background. Sleep complaints are common in patients with epilepsy (PWE). Excessive daytime sleepiness (EDS) is one of the most reported complaints and its impact is still a matter of debate. Objective. Evaluate the relationship between EDS and epilepsy, with emphasis on prevalence, assessment, and causes. Methods. A systematic review on PubMed database in the last 10 years (2002 to 2012). The search returned 53 articles and 34 were considered relevant. After citation analysis, 3 more articles were included. Results. Most studies were cross-sectional and questionnaire based. 14 papers addressed EDS as the primary endpoint. 14 adult and 3 children studies used subjective and objective analysis as methodology. The number of studies increased throughout the decade, with 21 in the last 5 years. Adult studies represent almost three times the number of children studies. EDS prevalence in PWE varies from 10 to 47.5%. Prevalence was higher in developing countries. Conclusion. EDS seems to be related more frequently to undiagnosed sleep disorders than to epilepsy-related factors, and although it affects the quality of life of PWE, it can be improved by treating comorbid primary sleep disorders.

The 50th anniversary of the Brazilian Academy Neurology (BAN) is being celebrated. BAN was founded on May 5th, in 1962. The BAN initial leaders and members are treated with reverence, as well as the honorable place where its foundation took place: the Institute of Neurology, first in Brazil, at the backyards of the ancient National Hospice for the Insane, which is an important and fruitful birthplace of the Brazilian Neurology.

Theodor Hermann Meynert (1833-1892), a German-Austrian neuropathologist and anatomist, is known as one of the founders of the Brain Psychiatry. He was the most arduous defender of the bridge between psychological and neurophysiological events, both dependent on specific neuroanatomical structures. Juliano Moreira (1873-1933), the founder of the Brazilian scientific psychiatry, is also mentioned, as well as the influence he received from the German psychiatry, mainly Emil Kraepelin's (1856-1926). Finally, the rapprochement of Psychiatry and Neurology is considered mostly in common areas as Neuropsychiatry.

The modern Brazilian Neurology was born in the campus of Praia Vermelha, Universidade Federal do Rio de Janeiro (UFRJ). The former National Hospice for the Insane (NHI) as well as some of its facilities were fundamental for the teaching about diseases of the nervous system to the students of the UFRJ's Medical School, caring for patients with neurosyphilis and epilepsy, as well as children with neurological impairment. We highlight the role of Juliano Moreira, director of the NHI (1903-1930), and his team, including Antonio Austregésilo Rodrigues Lima, the "father" of the Brazilian Neurology, in the construction of the modern Brazilian Psychiatry and Neurology.

Objective: To evaluate the prevalence of risk of having obstructive sleep apnea (OSA) and its determinants in patients with epilepsy (PE)., Method: 98 adult PE were prospectively screened for risk of OSA by Berlin questionnaire. Data was also collected about excessive daytime sleepiness, depression, anxiety, clinical and socio-demographic characteristics., Results: The PE main characteristics: 59-men/39-women, mean age=39.97, SD=12.3, range 18-66. The prevalence of the risk of OSA was 55.1% (CI 95%, 0.45-0.65). The high risk for OSA was related with body mass index (BMI) (p=0.000), neck circumference (NC) (p=0.000), arterial hypertension (AH) (p=0.000), and anxiety (p=0.006), without relationship with number of seizures, number of antiepileptic drugs, age or depression. The NC was statistically significant regarding risk of OSA, mainly in men., Conclusion: We found a high risk of OSA in this sample. The main implicated measures were the large NC, high BMI and anxiety. The anthropometric variables were more relevant than those related to epilepsy itself and similar to those of the general population.

The main aim of this paper was to present some pioneer women neurologist, their struggle to pursue their career and the barriers mainly encountered at the beginning of their professional lives. It was also presented the progressive increasing of the feminine participation in medicine and in the neurology with study based on data of the School of Medicine of the Federal University of Rio de Janeiro and Brazilian Academy of Neurology. Their composition were analyzed according to gender, class and academic rank. In spite of this feminization, there is lack of a women's parallel advancement into leadership positions despite no visible barriers ("glass ceiling").

Objective: The main objective of the study was to evaluate excessive daytime sleepiness (EDS) in patients with epilepsy from a tertiary outpatient clinic in Rio de Janeiro, Brazil., Methods: Ninety-nine unselected patients from the epilepsy outpatient clinic were invited and their sleep-wake habits were assessed by means of a clinical interview and four validated instruments: Epworth Sleepiness Scale, Beck Depression Inventory, Beck Anxiety Inventory, and the Basic Nordic Sleep Questionnaire. Sociodemographic information and characteristics of the epilepsy syndrome were collected., Results: Complaints about EDS (Epworth Sleepiness Scale score >10) were reported by 47.5% (95% CI: 37.3-57.7) of the patients. In the sample, 52.6% (95% CI: 0.41-0.60) were overweight or obese, and 53.1% (95% CI: 0.43-0.62) had less than 8 years of formal education. Mainly anxiety, but also neck circumference, was correlated with EDS. Our patients did not exhibit quantitative sleep deprivation. No other epilepsy-related variable showed relationship with EDS. The prevalence of EDS in our population was higher than in similar studies performed in other countries. This finding does not seem to be related to epilepsy itself, but rather to other clinical factors, such as neck circumference, and mainly psychiatric factors, such as anxiety., Conclusions: Psychiatric comorbidities such as anxiety are linked to EDS and should be recognized and treated. More studies based on objective sleep quality analysis are needed to help elucidate these relationships in the Brazilian population., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Classifications for neurological disorders have evolved from following the theory of the humors to modern anatomical pathology and, recently, to the germ theory that stared the etiological era, as seen in book content lists. The symptomatic approach towards neuronosology was widely used until the middle of the 19(th) century. The following books are representatives of this: "De Cerebri Morbis (1549)"; "De anima brutorum (1672)"; and "A Treatise on Nervous Disease (1820-1823)". During the Enlightenment, "Synopsis Nosologiae Methodicae (1769)" had a great repercussion subsequently on neuronosology. "Lehrbuch der Nerven-Krankheiten des Menschen (1840-1846)" was the first systematic treatise in neurology, and "A Treatise on the Diseases of the Nervous System (1871)" was the first American textbook of neurology, and probably the first comprehensive one. The first Brazilian textbook was "Lições sobre as moléstias do sistema nervoso, feitas na Faculdade de Medicina do Rio de Janeiro (1878)". A presentation going from causes of death to the International Classification of Diseases and their importance for neurology is also made.

Objective: To provide data about age, gender, time and space variation by means of mortality statistics from epilepsy in Brazil., Method: It was used demographic and mortality data obtained from the Brazilian Ministry of Health, regarding Brazil (capitals) over the calendar years 1980-2007. For the description of the data it was used Microsoft Office Excel 2007 spreadsheet, and figuring of trends by linear regression, GraphPad Prism 5.0., Results: There is a tendency to elderly mortality, and male mortality in almost age group, although regarding the relative frequency rates by age, 20-39 age strata is more representative. The mortality at home is proportionally predominant at 20-29 years of age, 47.52%. Adjusted death rates/100,000, 2007, do not show clear regional predominance., Conclusion: We can infer that various deaths coded as epilepsy, may be due to an acute symptomatic seizures. Sudden unexplained death in epilepsy (SUDEP) may be misdiagnosed and probably more represented at the group with more deaths at home.

Unlabelled: Members of the Brazilian Royal Family carry a rich medical history of epileptic seizures and alike., Objective: To present the medical knowledge about epilepsy by the time of the Brazilian Empire, as reported by the royal family., Method: Narrative review of historical facts about D. Pedro I's family health., Results: The Royal Family, since D. João VI's generation is full of members with epilepsy or acute symptomatic seizures of different etiologies., Conclusion: The reported cases suggest that Dom Pedro I's family presented epilepsy with tonic-clonic generalized seizures, besides psychogenic, organic non epileptic events and acute symptomatic seizures. As a whole, this familial epilepsy could fit the diagnosis of generalized epilepsy with febrile convulsion plus.

Unlabelled: Intrauterine seizure is a rare event. Genetic predisposition and trauma are possible risk factors., Objective: To review and comment on the historical description of intrauterine events of a bastard daughter of Dom Pedro I (Maria Isabel Alcântara Brasileira - 1830-1896)., Method: Review of historical facts about the health of Dom Pedro I's daughter according to primary and secondary historical data., Results: According to historical accounts, Dom Pedro I's daughter suffered trauma during the intrauterine period that provoked intrauterine seizures. At the age of eight years, she developed self-limited and benign generalized epilepsy. Like her father, she had mood problems and also learning difficulties., Conclusion: Dona Maria Isabel's own report does not shown sufficient evidence to support the diagnosis of post-traumatic intrauterine seizures. Nevertheless, her family history suggests a genetic basis for her epilepsy.

Background: The relationship between obstructive sleep apnea (OSA) and depressive symptoms is ambiguous in the literature., Purpose: To investigate if there is a correlation between depressive symptoms and the severity of OSA., Method: A retrospective, cross-sectional study of data from 123 consecutive adults patients with neither mental illness nor psychotropic drugs intake, referred to a sleep laboratory for an evaluation of OSA. For the statistical analysis (uni- and multivariate), we used the following variables: gender and age, as well as scores based on several scales and indexes such as Beck Depressive Inventory (BDI), Epworth Sleepiness Scale (ESS), Body Mass Index (BMI) and Apnea-Hypopnea Index (AHI)., Results: Univariate analysis found a weak but statistically significant negative correlation between BDI and AHI. However, with the multivariate logistic regression analysis model, the inverse relation between AHI and BDI no longer has statistical significance., Conclusion: There is no causal relationship between OSA and depressive symptoms in the population studied.

Objective: To show that the obstructive sleep apnea syndrome (OSAS) was the probable cause of D. Pedro II's excessive daytime sleepiness., Method: Research of historical documents and bibliographical., Results: The excessive daytime sleepiness of D. Pedro II (1825-1891) was well known and bitterly criticized behavior by oppositionist magazines; it was also recognized by his peers. He would fall asleep in public places such as the theater and while attending lectures. As a youth, he was of normal complexion, putting on weight (obesity) at middle years., Conclusion: The possibility of this diagnosis is particularly relevant in this case because it points to an organic cause for D. Pedro II daytime naps and excessive daytime sleepiness. It could be the result of OSAS and not "disinterest" as erroneously assumed at that time.

Introduction: Dom Pedro II, the Prince Heir and Emperor under regency, in a delicate period of the construction of the Brazilian nation, had convulsive seizures., Objective: To investigate the convulsive seizures and related syndromes of Dom Pedro II and his family, besides the physicians in charge of the health care., Method: Narrative review based on primary and secondary sources., Conclusion: The scattered and self-limited convulsive seizures associated with physical and mental integrity favored a benign prognosis. Dom Pedro and his family presented rich history of epileptic seizures and febrile convulsion. This variety resembles the diagnosis of generalized epilepsy with febrile seizures plus that seems to be a combination of several syndromes with shared genetic susceptibility.

Dom João VI's death occurred in adverse political conditions that led to the regicide hypothesis. The main aim of this paper is to mention life style and conditions of the death of the king by means of narrative review based on primary and secondary sources. Dom João VI died in the way of convulsions and gastrointestinal symptoms. This could be the result of the pathological chain of genetic determination, accentuated by the inappropriate life style, obesity and sedentary habits, and/or poisoning. The finding of arsenic in high doses in his viscera favors the last hypothesis as the basic cause of death, but one can not discard the atherosclerosis predisposing risk factors for a final stroke.

The main objective of this paper is to know the medical doctors of the Emperor, the preconized treatment, and the knowledge about diabetes at that time, its repercussions on the Emperor's nervous system, and the related political implications. A narrative revision was made, based on primary and secondary sources. Dom Pedro II was examined by the aristocracy of the medicine at the time, especially Jean-Martin Charcot, amongst the doctors of international reputation, and Cláudio Velho da Motta Maia, amongst the Brazilian doctors. Charcot diagnosed in the Monarch: mental stress, diabetic neuropathy, and a cerebral vascular lesion, probably a stroke, that he differentiated from other vascular obliterations elsewhere. He demonstrated his knowledge about diabetic neuropathy, possible topographical alternatives to justify the urinary incontinence, and Dom Pedro's weakness in the legs. Throughout his illness, Dom Pedro II presented others manifestations that contributed to his physical fragility, and that, certainly too, to his political decline, deposition and the proclamation of the Brazilian Republic.

We present medical issues related to Dom Pedro de Alcântara Bragança e Bourbon (1798-1834), first Emperor of Brazil. This is made by means of narrative revision on historical facts starting from primary and secondary sources. Dom Pedro presented familiar incidence of epilepsy. His seizures were relatively benign and scattered, supposedly started at the age of 13: idiophatic epilepsy with generalized tonic-clonic seizures only or juvenile myoclonic epilepsy. He also had behavioral disorder characterized by hypersexuality, agitation and impulsiveness. It could have facilitated his opportunistic behavior necessary for daring to transgress the conservatism of the Portuguese crown, to create the constitutional monarchism in Brazil and in Portugal.

Patients with epilepsy were reported to have concomitant sleep apnea, but it has been rarely linked to the epilepsy itself. We present a case of a 28-year-old, obese man with secondary medically resistant partial complex epilepsy due to a brain trauma, with progressive snoring, and sleep agitation, apneas, and important daytime somnolence. It was noticed in the polysomnographic study that he had several sleep respiratory events, probably due both to the epileptic seizures and the sleep apnea syndrome as a co-morbidity. Apnea and epilepsy will be discussed. A careful video-EEG-polysomnography study is important in evaluating refractory epileptic patients and/or epileptic patients with snoring.

Purpose: To validate a Portuguese version of a generic HRQL instrument already used for children with epilepsy. To analyze differences of scores among children with epilepsy and co-morbidities in contrast to other neurological and neuropsychiatric diagnosis, especially Attention Deficit and Hyperactivity Disorder (ADHD)., Methods: A total of 194 children (64 with epilepsy, 94 with ADHD and 38 with other neurological or neuropsychiatric diagnosis) were consecutively seen in a neuropaediatric ambulatory unit. Parents or caregivers responded to a Portuguese version of ICIS (Impact of Childhood Illness Scale). The questionnaire was analyzed for internal consistency and face validity. Clinical and socio-demographic variables were also analyzed., Results: Internal consistency, analyzed by Cronbach's alpha showed good results for total and combined scores and all sections of ICIS, except for the section "impact of illness and its treatment". The diagnostic categories differed in several ICIS sections, with the worst scores for children with epilepsy and co-morbidities. Children who only had epilepsy had a worse score on the section about treatment impact compared with children with ADHD, but better scores in relation to disease impact in development and parents/family. In the epilepsy group, HRQL scores were worst for symptomatic epilepsy, whilst seizure control and socioeconomic factors affected each section of the instrument in different ways., Conclusions: ICIS Portuguese version has good psychometric properties. It is a useful tool for analyzing HRQL in children with epilepsy and other neurodevelopmental and psychiatric disorders. Epilepsy and psychiatric co-morbidity seems to have an additive impact on a child's HRQL. This result is clinically important since epilepsy management must include not only seizure control but also mental health support.

Purpose: To construct a multidimensional questionnaire that analyses the epileptic child quality of life from the parental point of view., Method: The pilot questionnaire was composed of 157 questions distributed in several dimensions. Fifty-one epileptic children's parents answered the questionnaire. The instrument was tested in its diverse properties: frequency of endorsement, homogeneity (Cronbach alpha), criterion and face validity, and later it was reduced., Results: Endorsement frequency excluded 65 questions that did not attain a minimum of 5% response per item. Cronbach alpha was as follows: physical (0.93), psychological (0.91), social (0.91), familiar (0.70), cognitive (0.92), medical (0.30) and economical (0.37). Patient groups, in relation to seizure control, significantly differed only in physical domain and total score, although there was a trend to differences in other domains. The final questionnaire (QVCE50) has 50 items, with good homogeneity in the physical, psychological and cognitive domains., Conclusion: QVCE-50 is a promissing Brazilian HRQL questionnaire for children with epilepsy. It needs to be applied in a larger population to confirm its psychometric properties.

Objective: To demonstrate the epidemiological importance of the different types of non-epileptic events (NEE) in a low-income urban community., Method: The patients suspected of having epilepsy, who were detected in the first phase (screening one) of this prevalence study, were interviewed by a neurologist in a non-structured neurological interview. These NEE were classified as physiological and psychogenic, subdivided by various types. The psychogenic NEE were classified according to the DSM-IV criteria., Results: We compared the cases suspected of having epilepsy (n=176) with those not suspected (n=806) and discovered that those cases suspected of having epilepsy had a greater median age (<0.01) and female predominance (p<0.01). Among the cases suspected of having epilepsy there were different diagnosis: epileptic events without identifiable cause (n=20) or with identifiable causes (e.g., febrile convulsions and eclampsia). The most prevalent diagnosis for those suspected of having epilepsy was syncope (n=63; 35.8%). In terms of physiological events, the most frequent were: epileptic seizures, paroxysmal toxic phenomena (including alcoholism) and brain trauma, besides syncope; in terms of psychogenic events the most frequent were: dissociative and anxiety disorders. Regarding gender differences, paroxysmal toxic problems were significantly more prevalent in men (p= 0.02), and dissociative disorders (p=0.01) in women., Conclusion: This survey confirms the epidemiological importance of syncope in a populational sample with NEE. However, among the psychogenic disorders of this NEE sample, the most frequent were dissociative and anxiety phenomena. This finding contrasts with the literature based on samples from tertiary epileptic centers with video-EEG resources, which found somatoform disorders to be more prevalent than dissociative and anxiety phenomena.

We present the clinical, electroencephalographic, neuroimaging (brain magnetic resonance image - MRI and spectroscopy by MRI) and cytogenetic findings of a young male patient with a rare cytogenetic anomaly characterised by a de novo 46,XY,r(20)(p13q13.3) karyotype. He presents with mental retardation, emotional liability, and strabismus, without any other significant dysmorphies. There are brain anomalies characterised by corpus callosum, uvula, nodule and cerebellum pyramid hypoplasias, besides arachnoid cysts in the occipital region. He had seizures refractory to pharmacotherapy and long period of confusional status with or without a motor component. The authors recognised that the EEG pattern was not fixed but changed over time, specially for bursts of slow waves with great amplitude accompanied or not by sharp components, and bursts of theta waves sharply contoured. Previously, epilepsy solely has been assigned to region 20q13. However, the important structural cerebral alterations present in our case has not been reported associated to such chromosomal abnormality and may indicate possible new chromosomal sites where such atypical neurological characteristics could be mapped.

Objective: To study the most important characteristics of antiepileptic drug (AED) taking behavior in epileptic people., Methods: In a cross-sectional study, 45 consecutively seen patients answered a standardized questionnaire including questions about drug intake behavior., Results: Both genders were equally represented (22M x 23F). The mean age was 30.2 years. No specific characteristic were presented in all patients. The self-reported non-use of the drug at any moment one week before (self-reported non-adherence) was 40.0%. Patients took the drug more than once in most cases (75.0%), and the only precipitating factor of seizures more frequently avoided was alcohol intake (66.7%). Forty-four percent said to be afraid of becoming addicted to the medicine, 61.4% reduced or stopped the medicine just to see what would happen, and 47.7% changed the prescription with the same purpose. There is no relationship among socio-demographic, behavior aspects or treatment characteristics, and self-reported non-adherence., Conclusions: Several patient's aspects do not seem to be strongly correlated with self-reported adherence. Nevertheless, drug self-regulation is probably related to the drug-intake behavior, and it is important for the physician to understand this parallel influence on treatment for a more realistic approach.

It was evaluated the patient antiepileptic drug (AED) intake adherence in a pilot cross-sectional study carried out at a neurologic out-patient clinic of a university hospital. Ninety-three AED blood concentration (phenobarbital, phenytoin, carbamazepine) were analyzed from 24 patients. The variability of the AED blood level was measured (in the steady state period by means of the variation coefficient) and compared with the self-reported antiepileptic medication non-adherence, AED blood level according to the range (therapeutic or not), and the seizure control. It was not observed any strong correlation between the higher value of variability and the other three parameters of no adherence. The highest correlation was with the blood drug level (therapeutic or not). The evaluation of blood drug measurement alone, except in cases of extreme low adherence and variability of drug intake, is not enough for the recognition of incorrect drug intake, but the clinical markers and the self-reported adherence have to be also considered for this sort of evaluation.

Objective: To evaluate the accuracy of clinical unstructured and structured diagnosis of acute stroke subtypes--cerebral haemorrhage (CH), cerebral infarction (CI), subarachnoid haemorrhage (SAH)., Methods: Sixty consecutive patients with acute stroke admitted to the Emergency Ward of a Brazilian University Hospital were examined by emergency physicians and computerised tomography (CT). We also compared it (physician's unstructured diagnosis) to two published clinical scoring systems (structured diagnosis--Guy's Hospital and Siriraj Hospital) applied to three other populations--regarding the operational characteristics of the tests., Results: In our personal data, among 9 variables that could discriminate CH and CI, three have statistically significant difference (p < 0.05): headache (p = 0.0002) and vomiting (p = 0.02) occurred more frequently in CH patients, but previous stroke in those with CI (p = 0.04). Unstructured diagnosis proved valid for SAH, with a +LHR = 39.7; and to a smaller degree for CI (-LHR = 0.1). However, it exhibited low sensitivity for the diagnosis of CH. Structured tests (Guy's Hospital and Siriraj Hospital) also failed to confidently diagnose stroke subtypes, especially CH., Conclusions: Both clinical diagnosis (made by emergency physicians) and the available diagnostic tests fail to confidently discriminate CH and CI.