Abstract Objective This study aimed to investigate the prevalence of autism spectrum disorder and its possible correlations with clinical characteristics in patients with infantile epileptic spasms syndrome in a single center in Brazil. Methods This retrospective cross-sectional study examined 53 children with the diagnosis of infantile epileptic spasms syndrome prior to an autism spectrum disorder assessment. Participants were divided into two groups based on the presence or absence of autism spectrum disorder. Available variables (sex, medications, median age at onset of infantile epileptic spasms syndrome, and presence of comorbidities) were compared using Mann-Whitney U or chi-square tests. Results Among the included patients, 12 (23 %) were diagnosed with autism spectrum disorder, corresponding to a relative risk of 0.29 (95 % confidence interval 0.174-0.492). The age at the first seizure ranged from 3 to 15 months, with a mean of 6.65 months. This age significantly differed between participants with autism spectrum disorder (10.58 months) and those without (5.43 months), p<0.001. Conclusion Children with infantile epileptic spasms syndrome have a higher risk of being diagnosed with autism spectrum disorder. Later age of onset and period of spasm occurrence might be predisposing risk factors. Brazil crosssectional cross sectional 5 assessment sex, sex (sex medications comorbidities MannWhitney Mann Whitney chisquare chi square tests 1 23 (2 029 0 29 0.2 95 (9 0.1740.492. 01740492 0.174 0.492 . 174 492 0.174-0.492) months 665 6 65 6.6 10.58 1058 10 58 (10.5 5.43 543 43 (5.4 , p0001 p 001 p<0.001 factors 2 ( 02 0. 9 1740 0.1740.492 0174049 0174 0.17 0492 0.49 17 49 0.174-0.492 66 6. 10.5 105 (10. 5.4 54 4 (5. p000 00 p<0.00 0.1740.49 017404 017 0.1 049 0.4 0.174-0.49 10. (10 5. (5 p00 p<0.0 0.1740.4 01740 01 04 0.174-0.4 (1 p0 p<0. 0.1740. 0.174-0. p<0 0.1740 0.174-0 p< 0.174-

Abstract Introduction Parkinson disease (PD) is a progressive degeneration characterized by motor disorders, such as tremor, bradykinesia, stiffness and postural instability. Objective To evaluate the independence, confidence and balance in the development of daily activities in patients with PD before and after rehabilitation. Methods A descriptive, retrospective cross-sectional study was carried out with 16 patients (mean 57.6 ± 18.7 years), submitted to anamnesis, otolaryngological evaluation and vestibular assessment. The Vestibular Disorders Activities of Daily Living (VADL) and the Activities-Specific Balance Confidence (ABC) scales were applied before and after rehabilitation with virtual reality. Results a) The instrumental subscale of the questionnaire showed statistically significant result (p = 0.022; 95 % CI 1.21; 2.21) between the first and second assessments; b) The correlation between the questionnaires showed statistically significant result in the ambulation subscale (p = 0.011; 95 % CI -0.85; -0.17) first and (p = 0.002, 95 % CI -0.88; -0.31) second assessments, and the functional subscale was only verified in the second assessment (p = 0.011, 95 % CI -0.85; -0.17); and c) The patients presented clinical improvement in the final assessment after rehabilitation with significant result for the tightrope walk (p = 0.034, 95 % CI -12.5; -0.3) and ski slalom games (p = 0.005, 95 % CI -34.8; -6.6). Conclusions Our results showed that the VADL and ABC questionnaires, applied before and after rehabilitation, were important tools to measure the independence, confidence and balance while developing daily activities. The VADL and ABC questionnaires may effectively contribute to quantify the effect of the applied therapeutics and, consequently, its impact on the quality of life of patients with PD.

Abstract Introduction Spinocerebellar ataxia (SCA) is part of a genetic and clinical heteroge- neous group of neurodegenerative diseases characterized by progressive cerebellar ataxia. Objective To describe the results of audiological and electrophysiological hearing evaluations in patients with sporadic ataxia (SA). Methods A retrospective cross-sectional study was carried out with 11 patients submitted to the following procedures: anamnesis, otorhinolaryngological evaluation, tonal and vocal audiometry, acoustic immittance and brainstem auditory evoked potential (BAEP) tests. Results The patients presented with a prevalence of gait imbalance, of dysarthria, and of dysphagia; in the audiometric and BAEPs, four patients presented with alterations; in the acoustic immittance test, five patients presented with alterations, predominantly bilateral. Conclusion The most evident alterations in the audiological evaluation were the prevalence of the descending audiometric configuration between the frequencies of 2 and 4 kHz and the absence of the acoustic reflex between the frequencies of 3 and 4 kHz bilaterally. In the electrophysiological evaluation, the patients presented changes with a prevalence of increased I, III and V wave latencies and the interval in the interpeak I-III, I-V and III-V. In the present study, it was observed that auditory complaints did not have a significant prevalence in this type of ataxia, which does not occur in some types of autosomal recessive and dominant ataxia.

RESUMO Objetivo: Analisar características perinatais de crianças com transtorno do espectro autista (TEA). Métodos: Revisão retrospectiva dos prontuários medicos de 75 crianças com TEA, entre janeiro de 2008 e janeiro de 2015. Os critérios de inclusão foram o diagnóstico de TEA baseado no DSM-5 e o termo de consentimento assinado pelo responsável legal. O critério de exclusão foi ausência de todos os dados no prontuário médico. As variáveis analisadas foram: idade materna, prematuridade (idade gestacional menor que 37 semanas), baixo peso ao nascer (<2.500 g) e asfixia perinatal (Apgar menor que 7 no quinto minuto). Os dados foram avaliados por meio do teste de diferença entre as proporções (nível de significância de p<0,05). Resultados: Setenta e cinco pacientes foram incluídos no estudo. A idade materna variou de 21,4 a 38,6 anos (29,8±4,1 anos). O parto prematuro ocorreu em 14 (18,7%) pacientes, asfixia perinatal em 6 (8,0%) e baixo peso ao nascer em 32 (42,6%). As prevalências de prematuridade, asfixia perinatal e baixo peso ao nascer entre as crianças com TEA neste estudo foram maiores do que as prevalências gerais dessas condições entre todos os nascidos vivos em nosso país, região e estado, as quais são, respectivamente, 11,5, 2,3 e 8,5% no Brasil, 11,0; 2,2 e 8,5% na região Sul e 10,5, 2,0 e 8,4% no estado do Paraná. Conclusões: Nossos achados mostraram maior prevalência de prematuridade, baixo peso ao nascer e asfixia perinatal em crianças com TEA. Algumas limitações são o desenho retrospectivo do estudo e a amostra de pequeno tamanho. Grandes estudos prospectivos são necessários para esclarecer a possível associação entre intercorrências perinatais e TEA.

ABSTRACT Objective: To analyze perinatal features of children with autism spectrum disorder (ASD). Methods: Retrospective review of the medical records of 75 children with ASD, between January 2008 and January 2015. Inclusion criteria were diagnosis of ASD based on DSM-5 criteria, and the informed consent form signed by the person who is legally responsible. The exclusion criterion was missing on the medical record. The variables analyzed were maternal age, prematurity (gestational age under 37 weeks), low birth weight (<2,500 g), and perinatal asphyxia (5th minute Apgar score <7). Data were analyzed using the difference between proportions test, being significant p<0.05. Results: Seventy-five patients were included. Maternal age ranged from 21.4 to 38.6 years (29.8±4.1 years). Premature birth occurred in 14 (18.7%) patients, perinatal asphyxia in 6 (8.0%), and low birth weight in 32 (42.6%) patients. The prevalence of prematurity, low birth weight, and perinatal asphyxia among the children in our study was higher than the general prevalence of these conditions among all live births in our country, region, and state, which are, respectively, 11.5, 2.3, and 8.5% in Brazil; 11.0, 2.2, and 8.5% in Southern Brazil; and 10.5, 2.0, and 8.4% in the state of Paraná. Conclusions: Our findings show a higher prevalence of prematurity, low birth weight, and perinatal asphyxia among children with ASD. Some limitations are the retrospective study design, and the small sample size. Large prospective studies are needed to clarify the possible association between perinatal complications and ASD.

Abstract Introduction The discrete wavelet transform is used in many studies as signal preprocessor for EEG spike detection. An inherent process of this mathematical tool is the recursive wavelet convolution over the signal that is decomposed into detail and approximation coefficients. To perform these convolutions, firstly it is necessary to extend signal borders. The selection of an unsuitable border extension algorithm may increase the false positive rate of an EEG spike detector. Methods In this study we analyzed nine different border extensions used for convolution and 19 mother wavelets commonly seen in other EEG spike detectors in the literature. Results The border extension may degrade an EEG spike detector up to 44.11%. Furthermore, results behave differently for distinct number of wavelet coefficients. Conclusion There is not a best border extension to be used with any EEG spike detector based on the discrete wavelet transform, but the selection of the most adequate border extension is related to the number of coefficients of a mother wavelet.

Introduction  Fishing, one of the oldest productive activities, is an important sector of the national and world economy. Aim  To evaluate the vestibular behavior in a population of fishermen. Methods  In a retrospective and cross-sectional study, 13 fishermen (mean 45.0), between 33 and 62 years of age, were submitted to anamnesis, otorhinolaryngological evaluation, and vestibular exam through the vector electronystagmography. Results  The most evident otoneurologic symptoms were hearing loss (76.9%), tinnitus (61.7%), dizziness (46.1%), and headache (46.1%). The most evident clinical symptoms were fatigue (46.1%), depression (23.0%), anxiety (15.3%), insomnia (7.7%), and agitation during sleep (7.7%). There were alterations in the vestibular exam in 5 fishermen (38.5%) discovered in the caloric test. There was a prevalence of alteration in the peripheral vestibular system. There was a major frequency of the peripheral vestibular irritative syndrome. Conclusion  The otoneurologic complaints were frequent in the population studied to verify the importance of allowing labyrinth exams and the need for adopting preventive measures relating to noise exposure as well as carbon monoxide exposure, because they can cause and/or enhance various manifestations of labyrinthine vestibular impairment that can affect the quality of life of these workers.

Introduction Exposure to music is the subject of many studies because it is related to an individual's professional and social activities. Objectives Evaluate the vestibular behavior in military band musicians. Methods A retrospective cross-sectional study was performed. Nineteen musicians with ages ranging from 21 to 46 years were evaluated (average = 33.7 years and standard deviation = 7.2 years). They underwent anamnesis and vestibular and otolaryngologic evaluation through vectoelectronystagmography. Results The most evident otoneurologic symptoms in the anamnesis were tinnitus (84.2%), hearing difficulties (47.3%), dizziness (36.8%), headache (26.3%), intolerance to intense sounds (21.0%), and earache (15.7%). Seven musicians (37.0%) showed vestibular abnormality, which occurred in the caloric test. The abnormality was more prevalent in the peripheral vestibular system, and there was a predominance of irritative peripheral vestibular disorders. Conclusion The alteration in vestibular exam occurred in the caloric test (37.0%). There were changes in the prevalence of peripheral vestibular system with a predominance of irritative vestibular dysfunction. Dizziness was the most significant symptom for the vestibular test in correlation with neurotologic symptoms. The present study made it possible to verify the importance of the labyrinthine test, which demonstrates that this population should be better studied because the systematic exposure to high sound pressure levels may cause major vestibular alterations.

O objetivo do estudo foi verificar os benefícios da reabilitação vestibular (RV) com realidade virtual, por meio de avaliação pré e pós-aplicação da Escala de Equilíbrio de Berg (EEB), em quatro casos de ataxia espinocerebelar (AEC). Os casos foram submetidos aos seguintes procedimentos: anamnese, inspeção otológica, avaliação vestibular e aplicação da EEB pré e pós-RV, com a realidade virtual representada por meio da utilização de jogos do equipamento Wii Fit. Os casos retratam quatro pacientes com diagnóstico genético de AEC (dois tipo 2, um tipo 3 e um em investigação), sendo três do gênero feminino e um do gênero masculino, na faixa etária de 30 a 62 anos. Os pacientes referiram sintomas otoneurológicos e, no exame vestibular, observou-se a presença de nistagmo semiespontâneo com características centrais, ausência de nistagmo pós-rotatório, hiporreflexia e preponderância direcional do nistagmo assimétrica à prova calórica. Nos casos 1 e 2, os pacientes referiram melhora na coordenação dos movimentos e do equilíbrio corporal, independente do escore na EEB ter demonstrado médio risco para queda, antes e após a realização dos exercícios. No caso 3, o paciente apresentou melhora do escore na EEB, bem como do equilíbrio, apresentando baixo risco para queda. O caso 4 não evidenciou melhora na avaliação após a execução dos exercícios. Este estudo de caso demonstrou a possibilidade da aplicação dos exercícios de RV com estímulos virtuais na AEC, com melhora da coordenação motora e do equilíbrio postural.

The purpose of this study was to verify the benefits of the vestibular rehabilitation (VR) with virtual reality through the assessment before and after the application of the Berg Balance Scale (BBS) in four cases of spinocerebellar ataxia (SCA). The cases were underwent the following procedures: anamnesis, ear inspection, vestibular assessment and application of the BBS before and after VR with virtual reality using games from Wii Fit device. The cases describe four patients that were diagnosed with genetically inherited SCA (two type 2, one type 3 and one still under investigation), three of them were female and one was male, with ages ranging from 30 to 62 years. The patients presented otoneurological symptoms and the vestibular test showed the presence of semi-spontaneous nystagmus, absence of post-rotational nystagmus, hyporeflexia, and asymmetric directional preponderance of the nystagmus in the caloric test. Patients from cases 1 and 2 have showed an improvement in motor coordination and in body balance, even though the score presented by the BBS had demonstrated medium risk for falling before and after the exercises. In case 3, the patient's loss of balance and BBS score have improved, presenting low risk of falling; whereas the patient in case 4 did not show any improvement in the assessment after the exercises. This case study shows the applicability of VR exercises with virtual stimuli in SCA with improved motor coordination and postural balance.

ObjetivoAvaliar os dados clínicos, o eletroencefalograma, a etiologia, a classificação, o tratamento, a morbidade e a mortalidade do estado de mal epiléptico.MétodosQuinze pacientes, idade média de 41,3 anos, seis masculinos, foram avaliados retrospectivamente. Todos eles foram acompanhados por eletroencefalogramas seriados ou monitoração eletrencefalográfica contínua.ResultadosA comorbidade mais comum foi hipertensão arterial. Sete (46,7%) pacientes tinham epilepsia focal sintomática prévia. Mais de uma etiologia foi identificada em 40,0% dos casos. O estado de mal epiléptico parcial complexo foi o mais frequente (n=14; 93,3%) e discrete seizures foram os padrões ictal inicial mais observados. Midazolam contínuo foi usado em nove (60,0%) pacientes e tiopental contínuo em três (20,0%). Nove (60,0%) participantes morreram, um (6,6%) teve sequelas neurológicas e cinco (33,3%) não apresentaram sequelas.ConclusõesAlta taxa de mortalidade foi associada com idade avançada e com a presença de descargas periódicas epileptiformes lateralizadas. Midazolam provou ser uma droga segura. Estado de mal epiléptico refratário está associado à alta mortalidade.

ObjectiveTo evaluate clinical data, electroencephalogram, etiology, classification, treatment, morbidity, and mortality in acute refractory status epilepticus.MethodsFifteen patients, mean age of 41.3 years-old, six males, with refractory status epilepticus, were retrospectively studied. All of them were followed by serial electroencephalogram or continuous electroencephalographic monitoring.ResultsThe most common comorbidity was hypertension. Seven (46.7%) patients were diagnosed with previous symptomatic focal epilepsy. More than one etiology was identified in 40.0% of the cases. Status epilepticus partial complex was the most common (n=14, 93.3%), and discrete seizures were the most observed initial ictal pattern. Continuous intravenous midazolam was used in nine (60.0%) patients and continuous thiopental in three (20.0%). Nine (60.0%) participants died, one (6.6%) had neurological sequelae, and five (33.3%) presented no neurological sequelae.ConclusionsHigher mortality rate was associated with advanced age and periodic lateralized epileptiform discharges. Midazolam proved to be a safe drug. The refractory status epilepticus is related to high mortality.

OBJETIVO: Investigar o comportamento auditivo de pacientes com insuficiência renal crônica submetidos ao transplante renal. MÉTODOS: Foram avaliados 30 pacientes, 10 do gênero feminino e 20 do gênero masculino, na faixa etária de 13 a 26 anos (média de idade 16,97 anos). Os sujeitos foram submetidos a anamnese, avaliação otorrinolaringológica, avaliação audiológica convencional e de altas frequências, medidas de imitância acústica e avaliação do processamento auditivo central. Para os resultados da audiometria de altas frequências foi utilizado um grupo controle. RESULTADOS: Os sujeitos não apresentaram queixas auditivas na anamnese. Os resultados da audiometria convencional demonstraram predomínio da normalidade; na audiometria de altas frequências, os pacientes apresentaram resultados piores do que os sujeitos do grupo controle. Na imitanciometria houve predomínio de curva tipo A bilateral. Na avaliação do processamento auditivo central, 14 sujeitos (46,67%) apresentaram resultados alterados no Staggered Spondaic Word Test (SSW). Houve diferença significativa entre a variável idade e o resultado da audiometria tonal limiar: quanto maior a idade, menor a sensibilidade auditiva nos limiares de 250 Hz a 8 kHz. Houve relação entre o tipo de doador (cadáver ou vivo) e o resultado do teste SSW: os índices de resultados alterados foram maiores quando o doador era cadáver, em comparação com casos de doador vivo. CONCLUSÃO: Houve alterações na avaliação audiológica convencional e de altas frequências e no processamento auditivo central de sujeitos com insuficiência renal crônica submetidos ao transplante renal, sugerindo a necessidade de orientação à equipe envolvida quanto aos cuidados, prevenção e identificação precoce de acometimentos audiológicos.

PURPOSE: To investigate the auditory behavior of patients with chronic renal failure (CRF) undergoing kidney transplantation. METHODS: Thirty patients were evaluated, 10 (33.33%) females and 20 (66.67%) males, aging from 13 to 26 years (average, 16.97 years; standard deviation, 3.60 years). Patients underwent the following procedures: anamnesis, otolaryngological examination, audiological evaluation (pure tone and high frequency), acoustic impedance measurements and central auditory processing evaluation. A control group was used to compare the high-frequency audiometry results. RESULTS: The following observations were made: absence of auditory complaints at the time of anamnesis; pure-tone audiometry was predominantly normal; patients presented lower hearing levels at the high-frequency audiometry, when compared to the control group, and as for the acoustic impedance measurements, curves of the type A were predominant; there was a change of the central auditory processing for 14 patients (46.67%) in the Staggered Spondaic Word Test (SSW); there was a significant difference between the age variable and the result of the pure-tone audiometry, that is, hearing sensitivity in thresholds from 250Hz to 8,000Hz decreased with advancing age; and the relation between the type of donor and the SSW test result was significant. Rates were higher when the patients had been transplanted from deceased donors compared to living donors. CONCLUSION: There were no changes in conventional audiological and high-frequency evaluation, or in the central auditory processing. Professionals involved in the care of kidney transplantation recipients must be better informed about the care, prevention, and early identification of auditory disorders.

OBJETIVO: Estado de mal epiléptico não convulsivo (EMENC) é atualmente considerado uma das formas mais frequentes de estado de mal epiléptico. O objetivo deste estudo foi identificar a história natural da evolução eletrográfica do EMENC refratário, bem como estabelecer relações entre padrões ictais e o prognóstico. MÉTODOS: Foram analizados, retrospectivamente, 14 pacientes com comprometimento da consciência e EMENC. Os padrões ictais foram classificados em crises isoladas (CI), crises subintrantes (CS), descarga ictal contínua (DIC), descarga ictal contínua com períodos de atenuação (DIC-A) e descargas epileptiformes periódicas lateralizadas (PLEDs). RESULTADOS: Os padrões ictais observados foram CI (n=7; 50,0%), PLEDs (n=3; 1,4%), DIC (n=2; 14,3%), CS (n=1; 7,1%) e DIC-A (n=1; 7,1%). CONCLUSÕES: Achados eletrográficos no EMENC refratário são heterogêneos e não obedecem a uma sequência estereotipada. As PLEDs estão associadas à maior probabilidade de morbidade e mortalidade neurológica.

OBJECTIVE: Nonconvulsive status epilepticus (NCSE) is currently considered as one of the most frequent types of status epilepticus (SE). The objective of the present study was to identify the natural history of the electrographical evolution of refractory NCSE and to establish the relationship between ictal patterns and prognosis. METHODS: We analyzed, retrospectively, 14 patients with loss of consciousness and NCSE. The ictal patterns were classified as discrete seizures (DS), merging seizures (MS), continuous ictal discharges (CID), continuous ictal discharges with flat periods (CID-F), and periodic lateralized epileptiform discharges (PLEDs). RESULTS: The ictal patterns were DS (n=7; 50.0%), PLEDs (n=3; 1.4%), CID (n=2; 14.3%), MS (n=1; 7.1%), and CID-F (n=1; 7.1%). CONCLUSIONS: NCSE electrographic findings are heterogeneous and do not follow a stereotyped sequence. PLEDs were related to a higher probability of neurological morbidity and mortality.

INTRODUÇÃO: hamartoma hipotalâmico é uma malformação congênita rara, que pode se manifestar através de crises epilépticas, principalmente as gelásticas, atraso do desenvolvimento neuropsicomotor, retardo mental, distúrbios comportamentais e puberdade precoce. As crises têm início precoce e são clinicamente refratárias. A etiologia e os mecanismos fisiopatogênicos não são totalmente conhecidos. O eletrencefalograma pode apresentar desde desorganização e alentecimento da atividade de base até paroxismos epileptogênicos multifocais e/ou generalizados. OBJETIVO: relatar as dificuldades e desafios do tratamento neurocirúrgico em um caso de hamartoma hipotalâmico em um lactente. RELATO DO CASO: lactente com crises epilépticas desde oito meses de idade. A investigação revelou a presença de uma lesão em túber cinerium sugestiva de hamartoma. As crises tornaram-se refratárias, sendo indicado procedimento cirúrgico. A ressecção endoscópica não pôde ser realizada, pois o hamartoma encontrava-se totalmente aderido ao hipotálamo. Atualmente, a criança mantém crises tônicas, clônicas e atônicas. DISCUSSÃO: a lesionectomia realizada por microcirurgia ou radiocirurgia parece ser o tratamento mais efetivo para o controle das crises em pacientes com hamartoma hipotalâmico. Calosotomia pode ser eficaz em casos selecionados e lobectomias/ressecções corticais não tem efetividade no controle das crises. Em alguns pacientes, particularmente nos lactentes, lesionectomia e radiocirurgia podem ser tecnicamente inviáveis. Quando o tratamento neurocirúrgico não é possível a epilepsia deve ser classificada como intratável.

INTRODUCTION: Hypothalamic hamartoma is a rare congenital malformation, characterized by epilepsy, especially gelastic seizures, psychomotor developmental delay, mental retardation, behavioral disorders and precocious puberty. Epilepsy has early onset and is usually medically refractory. Etiology and pathophysiological mechanisms are unclear. The EEG can present disorganization and slowing of background activity and multifocal and/or generalized epileptogenic discharges. OBJECTIVE: To report the difficulties and challenges of neurosurgical treatment of a hypothalamic hamartoma in an infant. CASE REPORT: Infant with seizures since eight months old of age. The neurological investigation revealed a lesion in tuber cinereum suggestive of hamartoma. The epilepsy evolved with resistance to antiepileptic drugs, requiring neurosurgical procedure. The endoscopic resection could not be performed because the hamartoma was firmly attached to the hypothalamus. Currently, the child remains with tonic, clonic and atonic seizures. DISCUSSION: Lesionectomy performed by microsurgery or radiosurgery seems to be the most effective treatment for seizure control in patients with hypothalamic hamartomas who do not respond to clinical treatment. Callosotomy may be effective in selected cases, and lobectomy/cortical resections are not related to seizure control. In some patients, particularly in infants, lesionectomy and radiosurgery may be technically unfeasible.

INTRODUÇÃO: síndrome de Down (SD) é a anormalidade cromossômica que mais comumente causa deficiência mental e sua associação com epilepsia é muito variável na infância. Embora as descrições iniciais da síndrome não relatassem crises, sua associação com epilepsia é relativamente comum. MÉTODOS: foram avaliados 68 indivíduos com SD e 83 com retardo mental não sindrômico (RMNS). Todos os pacientes foram submetidos à EEG digital, com duração mínima de 30 minutos e com eletrodos posicionados segundo o sistema internacional 10-20 de posicionamento de eletrodos. Dados foram analisados usando estatística descritiva e proporções foram comparadas com o teste t de Student e teste de Diferença entre Proporções com p<0,05 sendo considerado estatisticamente significativo. RESULTADOS: SD: 27,9% tinham epilepsia (primeira crise 2,2±3,7 anos). Quinze (22,1%) pacientes tinham descargas, 5 (7,4%) hipsarritmia, 5 (7,4%) padrão focal, 3 (4,4%) padrão generalizado, 2 (2,9%) padrão multifocal. N-SMR: 33,7% pacientes tinham epilepsia (primeira crise com 1,2±4,5 anos). Vinte e três (27,7%) pacientes tinham descargas, 10 (12,0%) padrão focal, 5 (6,0%) padrão generalizado e 8 (9,6%) padrão multifocal. CONCLUSÕES: a diferença entre a ocorrência de epilepsia no grupo SD e RMNS não foi estatisticamente significativa, assim como o EEG normal, com padrão focal, generalizado e multifocal. No grupo SD, 7,4% apresentaram hipsarritmia. A comparação com o grupo RMNS não foi possível por que ninguém neste grupo apresentou esta anormalidade no EEG.

INTRODUCTION: Down syndrome (DS) is the most common chromosomal abnormality causing mental retardation and its association with epilepsy is highly variable in childhood. Although the first descriptions of the syndrome did not report seizures, their association with epilepsy is relatively common. METHODS: were evaluated 68 individuals with DS and 83 with non-syndromic mental retardation (N-SMR). All patients underwent digital EEG, lasting at least 30 minutes and electrodes positioned according to the International 10-20 System of Electrode Placement. Data were analyzed using descriptive statistics and proportions were compared with Student's t-test and test of Differences between Proportions with p<0.05 considered statistically significant. RESULTS: DS: 27.9% had epilepsy (first seizure with 2.2±3.7 years). Fifteen (22.1%) patients had epileptiform discharges, 5 (7.4%) hypsarrhythmia, 5 (7.4%) focal pattern, 3 (4.4%) generalized pattern and 2 (2.9%) multifocal pattern. N-SMR: 33.7% patients had epilepsy (first seizure with 1.2±4.5 years). Twenty-three (27.7%) patients had epileptiform discharges, 10 (12.0%) focal pattern, 5 (6.0%) generalized pattern and 8 (9.6%) multifocal pattern. CONCLUSION: The difference between the occurrence of epilepsy in DS and N-SMR was not statistically significant, as well as between normal EEG, EEGs with focal pattern, generalized pattern and multifocal pattern. In SD group 7.4% have shown hypsarrhythmia.The comparison with N-SMR was not possible because none of these has shown this EEG abnormality.

TEMA: a polimiosite é uma enfermidade sistêmica idiopática caracterizada por um processo inflamatório não supurativo que acomete a musculatura esquelética e manifesta-se clinicamente por fraqueza muscular proximal e simétrica. Possui incidência variável, cerca de um caso para cada 100 mil habitantes, havendo predominância no sexo feminino. PROCEDIMENTOS: avaliou-se no Setor de Otoneurologia de uma Instituição, um paciente com diagnóstico de polimiosite, com 60 anos, do sexo feminino, que referiu dificuldade para escutar no ouvido direito, zumbido, ansiedade, insônia, dificuldade nos movimentos do pescoço e dor irradiada para ombros e braços. Foram realizados os seguintes procedimentos: anamnese, inspeção otológica, avaliação audiológica e vestibular. RESULTADOS: a) na avaliação audiológica apresentou, perda auditiva neurossensorial de grau moderado na orelha direita e limiares auditivos dentro dos padrões de normalidade na orelha esquerda; b) na imitanciometria apresentou, curva timpanométrica do tipo "A" e presença dos reflexos estapedianos bilateralmente; c) no exame vestibular observou-se, nistagmo semi-espontâneo do tipo múltiplo e ausência do efeito inibidor da fixação ocular. CONCLUSÃO: observou-se alteração nos exames realizados, principalmente, no exame labiríntico que demonstrou a importância de sua realização e a sensibilidade na captação de alteração que sugeriu envolvimento do sistema nervoso central contribuindo dessa forma, para uma intervenção e acompanhamento terapêutico mais rápido e eficaz.

BACKGROUND: polymyositis is an idiopathic systematic disease characterized by a non-suppurative inflammatory process which attacks the skeletal musculature and manifests itself clinically by proximal and symmetrical muscular debility. It shows a variable incidence, approximately one in every hundred thousand inhabitants, predominately in females. PROCEDURES: a patient, 60 years, female and diagnosed with polymyositis was evaluated in the Otoneurology Sector of an Institution, referred having hearing difficulties in the right ear, tinnitus, anxiety, insomnia, difficulties in neck movement and shoulders and arms disseminated pain. The following procedures were carried out: anamnesis, otological inspection, audiological evaluation and vestibular exam. RESULTS: a) in the audiological evaluation there was sensorineural hearing loss from moderate degrees in the right ear and normal hearing thresholds in the left ear; b) in the imitanciometry there was a type "A" tympanometric curve and the presence of the stapedial reflexes, bilaterally; c) in the vestibular exam, semi-spontaneous multiple nystagmus and an absence of the inhibiting effect for ocular fixation were observed. CONCLUSION: alterations were observed in the accomplished exams, mainly in the vestibular exam, which demonstrated the importance for being carried out; and the sensitivity in the acquisition of the alteration which suggested an involvement of the central nervous system, contributing, thus, to a faster and more effective intervention and therapeutic accompaniment.