Otávio Gomes Lins

Congenital Zika syndrome with arthrogryposis: retrospective case series study

Objective To describe the clinical, radiological, and electromyographic features in a series of children with joint contractures (arthrogryposis) associated with congenital infection presumably caused by Zika virus. Design Retrospective case series study. Setting Association for Assistance of Disabled Children, Pernambuco state, Brazil. Participants Seven children with arthrogryposis and a diagnosis of congenital infection presumably caused by Zika virus during the Brazilian microcephaly epidemic. Main outcome measures Main clinical, radiological, and electromyographic findings, and likely correlation between clinical and primary neurological abnormalities. Results The brain images of all seven children were characteristic of congenital infection and arthrogryposis. Two children tested positive for IgM to Zika virus in the cerebrospinal fluid. Arthrogryposis was present in the arms and legs of six children (86%) and the legs of one child (14%). Hip radiographs showed bilateral dislocation in seven children, subluxation of the knee associated with genu valgus in three children (43%), which was bilateral in two (29%). All the children underwent high definition ultrasonography of the joints, and there was no evidence of abnormalities. Moderate signs of remodeling of the motor units and a reduced recruitment pattern were found on needle electromyography (monopolar). Five of the children underwent brain computed tomography (CT) and magnetic resonance imaging (MRI) and the remaining two CT only. All presented malformations of cortical development, calcifications predominantly in the cortex and subcortical white matter (especially in the junction between the cortex and white matter), reduction in brain volume, ventriculomegaly, and hypoplasia of the brainstem and cerebellum. MRI of the spine in four children showed apparent thinning of the cord and reduced ventral roots. Conclusions Congenital Zika syndrome should be added to the differential diagnosis of congenital infections and arthrogryposis. The arthrogryposis was unrelated to the abnormalities of the joints themselves, but was possibly of neurogenic origin, with chronic involvement of central and peripheral motor neurones leading to deformities as a result of fixed postures in utero. Based on the neurophysiological observations, we suggest two possible mechanisms: tropism of neurones, with involvement of peripheral and central motor neurones, or a relation with vascular disorders.

DISTÚRBIOS DO SONO NA EPILEPSIA DO LOBO TEMPORAL

The objective of this study was to evaluate sleep macrostructure and sleep disturbance in a group of 39 patients with temporal lobe epilepsy (TLE). Patients completed questionnaires to evaluate their sleep and subjective daytime sleepiness (Epworth Sleepiness Scale [ESS]) and undergone Polysomnography and Multiple Sleep Latency Test (MSLT). Daytime sleepiness was the most frequent complaint (85%), followed by wakefulness during sleep, history of seizures during sleep (75%) and initial insomnia (26%). Parassomnias (67%), obstructive sleep apneas (13%), restless legs syndrome (15%) and periodic limb movements (5%) were the most frequent sleep disorders. The most frequent changes of sleep patterns were: sleep architecture fragmentation (100%), decreased amount of REM sleep (92%) and increase in time awake after sleep onset (77%). There were significative correlations between the ESS and the MSLT (p<0,05). In conclusion, TLE patients have fragmented sleep with increased sleep stages shifts, increased number of awakenings and in time awake after sleep onset. REM sleep was decreased. Daytime sleepiness was the most frequent complaint in TLE patients.

Eletromiografia de superfície da musculatura supra-hióidea durante a deglutição de idosos sem doenças neurológicas e idosos com Parkinson

PURPOSE: to investigate differences between electromyographic samples of right and left suprahyoid muscles during deglutition of thickened liquids and liquid consistencies by elderly people with Parkinson disease and elderly people without any neurological disease. METHODS: 30 subjects, from both genders, took part in this study. Fifteen were elderly without neurological disease whereas the others showed Parkinson disease. All of them were submitted to a clinical evaluation protocol and surface electromyography. RESULTS: significant amplitude difference was found between subjects (f=14.4 and p=0.000) and between sides (t=4,4 and p=0.0037). As for muscular contraction time, the differences were just significant between the consistencies (f=8.2 and p=0.005). CONCLUSION: the electromyographic amplitude was shorter for subjects with Parkinson disease and this amplitude was higher in both groups at the right side. The duration of the muscular contraction did not display significant difference between the subjects with Parkinson disease and elderly without neurological disease. This duration was shorter during the deglutition of liquid consistencies.

A resposta auditiva de estado estável na avaliação auditiva: aplicação clínica

TEMA: a resposta auditiva de estado estavel (RAEE) e um procedimento eletrofisiologico que permite avaliar ao mesmo tempo os limiares auditivos de varias frequencias em ambas as orelhas, reduzindo assim o tempo de teste, e permite estimular ate niveis proximos a 125dB HL, caracterizando assim a audicao residual. OBJETIVO: verificar a aplicabilidade da RAEE para determinar os limiares auditivos nos diferentes graus de perda auditiva neurossensorial coclear. METODO: foram avaliados 48 individuos com idade entre sete e trinta anos e diferentes graus de perdas auditivas. A Audiometria Tonal Liminar (ATL) e a RAEE foram avaliadas nas seguintes frequencias portadoras, 0,5; 1; 2 e 4k Hz. As frequencias portadoras na RAEE foram moduladas em amplitude e frequencia, com estimulacao multipla e dicotica nas perdas auditivas de grau leve e moderado. Estimulacao simples foi utilizada nos outros graus de perdas auditivas. RESULTADOS: houve associacao significante (p < 0,01) entre os limiares obtidos na ATL e RAEE para todas as frequencias testadas, principalmente para perdas auditivas de grau profundo. Contudo em alguns pacientes o grau da perda auditiva pode ser super-estimado. CONCLUSAO: a RAEE pode ser utilizada para predizer os limiares auditivos da ATL, porem nao deve ser analisada de forma isolada, mas de forma complementar a avaliacao audiologica comportamental.BACKGROUND the auditory steady state response (ASSR) is an electrophysiological procedure that evaluates, at the same time, the hearing thresholds of both ears at various frequencies. This procedure reduces the time of testing and enables the stimulation of levels close to 125dB HL, characterizing residual hearing. AIM to verify the applicability of the ASSR to determine the hearing thresholds in different levels of sensorineural hearing loss. METHOD 48 individuals aged 7-30 years, with different levels of hearing loss were assessed. The Pure tone Audiometry (PTA) and the ASSR were carried out in following carrier frequencies 0.5; 1; 2 and 4k Hz. The carrier frequencies in the ASSR were modulated in amplitude and frequency, with multiple dicotic stimulation for mild and moderate hearing loss. Simple stimulation was used to test the other hearing deficit levels. RESULTS there was a significant association (p<0.01) between the thresholds obtained in the PTA and in the ASSR for all of the tested frequencies, especially for the severe levels. However, for some patients, the hearing loss level was overestimated in the ASSR. CONCLUSION the ASSR can be used to estimate the PTA threshold; nevertheless, it should not be analyzed in an isolated way, but seen as a complementary procedure to the behavioral auditory assessment.

Quality of Life Related to Swallowing in Parkinson’s Disease

Swallowing difficulties in Parkinson’s disease can result in decreased quality of life. The swallowing quality of life questionnaire (SWAL-QOL) is an instrument for specifically assessing quality of life with respect to swallowing, which has been little explored in patients with Parkinson’s disease (PD). The goal of this study was to evaluate the quality of life with respect to swallowing in persons with PD compared to controls and at several stages of the disease using the SWAL-QOL. The experimental group was composed of 62 persons with PD at stages 1–4. Forty-one age-matched healthy subjects constituted the control group. The SWAL-QOL scores were significantly lower for the patients with PD than for the controls in all SWAL-QOL domains. Eating duration had the largest difference in score between persons with PD and the controls and the lowest mean score, followed by communication, fatigue, fear, sleep, and food selection. The scores of most domains were lower at later stages of the disease. The scores for eating duration, symptom frequency, and sleep were significantly lower at stage 4 than stages 1 and 2. In conclusion, patients with PD have significantly lower scores in all domains of the SWAL-QOL than normal controls. This means swallowing difficulties occurring in patients with PD negatively affect their QOL. Progression of the disease worsens swallowing QOL, more specifically in the domains of eating duration, symptom frequency, and sleep. This occurs mostly at later stages of the disease.

Monitorando a deglutição através da eletromiografia de superfície

OBJETIVO: descrever o metodo de registro da eletromiografia de superficie atraves da utilizacao de um protocolo desenvolvido para o estudo da degluticao e demonstrar a degluticao de um paciente com doenca de Parkinson e de um sujeito normal atraves do registro da eletromiografia de superficie (EMGs). METODOS: para ilustrar os parâmetros eletrofisiologicos registrados apos execucao do protocolo foram utilizados dois voluntarios do sexo feminino, sendo um sem doenca e outro apresentando doenca de Parkinson (DP) no estagio III de acordo com a escala de Hoehn e Yahr. Os parâmetros analisados pelo foram: a duracao da atividade eletrica durante a degluticao, a amplitude (rms) e o limite de disfagia. RESULTADOS: os resultados mostram diferencas entre os eletromiogramas ilustrativos. CONCLUSAO: a EMGs pode ser utilizada como metodo de avaliacao e monitorizacao da degluticao de sujeitos sem doenca e com DP.

Psychiatric comorbidities in patients with systemic lupus erythematosus: a systematic review of the last 10 years

OBJECTIVE To analyze the frequency of psychiatric comorbidities in patients with systemic lupus erythematosus (SLE) using the systematic review method. METHODS A systematic literature search was performed between April and July 2011 in the following databases: BIREME, PubMed and CAPES thesis database. This search prioritized studies published over the last ten years (2001-2011), involving the presence of psychiatric comorbidities in patients with SLE. RESULTS Out of 314 articles published in scientific journals (PubMed) and 29 (BIREME), previously identified ones, 13 articles on psychiatric disorders and SLE were selected so they could be submitted to the systematic review methodological approach. The articles indicated high frequency of psychiatric comorbidities, especially mood and anxiety disorders. There is no consensus between the disease activity and psychiatric disorders. Patients with active SLE showed a higher risk of developing mood disorders than patients with inactive SLE. CONCLUSION Patients with SLE had a higher suicide risk than the general population. More thorough studies to evaluate the psychological and genetic role, specific and non-specific autoimmune inflammatory mechanisms in mood and anxiety disorders are needed.

Comorbidades psiquiátricas em pacientes com lúpus eritematoso sistêmico: uma revisão sistemática dos últimos 10 anos

OBJECTIVE: To analyze the frequency of psychiatric comorbidities in patients with systemic lupus erythematosus (SLE) using the systematic review method. METHODS: A systematic literature search was performed between April and July 2011 in the following databases: BIREME, PubMed and CAPES thesis database. This search prioritized studies published over the last ten years (2001-2011), involving the presence of psychiatric comorbidities in patients with SLE. RESULTS: Out of 314 articles published in scientific journals (PubMed) and 29 (BIREME), previously identified ones, 13 articles on psychiatric disorders and SLE were selected so they could be submitted to the systematic review methodological approach. The articles indicated high frequency of psychiatric comorbidities, especially mood and anxiety disorders. There is no consensus between the disease activity and psychiatric disorders. Patients with active SLE showed a higher risk of developing mood disorders than patients with inactive SLE. CONCLUSION: Patients with SLE had a higher suicide risk than the general population. More thorough studies to evaluate the psychological and genetic role, specific and non-specific autoimmune inflammatory mechanisms in mood and anxiety disorders are needed.

Interleukin-6 promoter polymorphisms -174 G/C in Brazilian patients with systemic lupus erythematosus.

To investigate the association of the IL-6 gene promoter polymorphism (-174 G/C) with SLE susceptibility and disease features in Brazilian patients, a case-control study of 80 lupus cases and 60 volunteer healthy women was performed. Genotyping was carried out by polymerase chain reaction and PCR product was digested by HSP92II restriction enzyme, being after visualized in polyacrylamide gel. There were significant differences in the distribution of the IL-6 gene C/C polymorphism between the SLE and control groups (χ(2) = 8.668; P = 0.0032). Individual carriers of the variant allele G had a 1.98 (95% CI: 1.0844-3.6353)-fold increased risk for SLE. Besides, association was observed in frequency of C allele (P = 0.0247; OR = 0.5036) between SLE patients and control groups. This study presents preliminary evidence for association between IL-6 polymorphism and SLE susceptibility in a Northeast population from Brazil.

Valores referenciais da eletromiografia de músculos envolvidos na deglutição: uma revisão sistemática

BACKGROUND: normal surface electromyography (sEMG) of muscles involved in swallowing. PURPOSE: to investigate if the literature indicates normal parameters for duration, amplitude and characteristics of the electromyograms of the muscles involved in swallowing (orbicularis oris, masseter, muscles of the suprahyoid muscles and the infra-hyoid region covered by the platysma muscle). CONCLUSION: the search resulted in 33 references, of which only five were within the inclusion criteria. Only one reference was classified with good level of quality by the Jadad scale with modifications. The selected articles cannot point reliable reference values mainly for EMG amplitude and morphology, because they used a inappropriate sampling rate for the EMG recordings that maximizes the achievement of distorted data for the muscle activity. Literature suggests the normalization of techniques for the electromyographic signal due to the variability between and among subjects.