Combined ocular and cervical vestibular evoked myogenic potential in individuals with vestibular hyporeflexia and in patients with Ménière's disease / Potencial evocado miogênico vestibular ocular e cervical simultâneo em indivíduos com hiporreflexia vestibular e em indivíduos com doença de Ménière

Abstract Introduction: The vestibular evoked myogenic potential is a potential of mean latency that measures the muscle response to auditory stimulation. This potential can be generated from the contraction of the sternocleidomastoid muscle and also from the contraction of extraocular muscles in response to high-intensity sounds. This study presents a combined or simultaneous technique of cervical and ocular vestibular evoked myogenic potential in individuals with changes in the vestibular system, for use in otoneurologic diagnosis. Objective: To characterize the records and analyze the results of combined cervical and ocular VEMP in individuals with vestibular hyporeflexia and in those with Ménière's disease. Methods: The study included 120 subjects: 30 subjects with vestibular hyporeflexia, 30 with Ménière's disease, and 60 individuals with normal hearing. Data collection was performed by simultaneously recording the cervical and ocular vestibular evoked myogenic potential. Results: There were differences between the study groups (individuals with vestibular hyporeflexia and individuals with Ménière's disease) and the control group for most of wave parameters in combined cervical and ocular vestibular evoked myogenic potential. For cervical vestibular evoked myogenic potential, it was observed that the prolongation of latency of the P13 and N23 waves was the most frequent finding in the group with vestibular hyporeflexia and in the group with Ménière's disease. For ocular vestibular evoked myogenic potential, prolonged latency of N10 and P15 waves was the most frequent finding in the study groups. Conclusion: Combined cervical and ocular vestibular evoked myogenic potential presented relevant results for individuals with vestibular hyporeflexia and for those with Ménière's disease. There were differences between the study groups and the control group for most of the wave parameters in combined cervical and ocular vestibular evoked myogenic potential.

Resumo Introdução: O potencial evocado miogênico vestibular é um potencial de média latência que avalia a resposta muscular decorrente de estimulação auditiva. Pode ser gerado a partir da contração do músculo esternocleidomastóideo e também a partir da contração de músculos extraoculares em resposta a sons de elevada intensidade. Este estudo apresenta uma técnica combinada ou simultânea de potencial evocado miogênico vestibular cervical e ocular em indivíduos com alterações no sistema vestibular para que possa ser usada no diagnóstico otoneurológico. Objetivo: Caracterizar o registro e analisar os resultados do potencial evocado miogênico vestibular cervical e ocular combinado em indivíduos com hiporreflexia vestibular e em indivíduos com doença de Ménière. Método: Participaram do estudo 120 indivíduos, 30 com hiporreflexia vestibular, 30 com doença de Ménière e 60 com audição dentro dos padrões de normalidade. A coleta de dados foi feita por meio do potencial evocado miogênico vestibular cervical e ocular registrados simultaneamente. Resultados: Houve diferença entre o grupo de estudo (indivíduos com hiporreflexia vestibular e indivíduos com doença de Ménière) e o grupo controle para a maioria dos parâmetros das ondas no potencial evocado miogênico vestibular cervical e ocular combinado. Para o potencial evocado miogênico vestibular cervical observou-se que o prolongamento da latência das ondas P13 e N23 foi a alteração mais encontrada no grupo de indivíduos com hiporreflexia vestibular e no grupo de indivíduos com doença de Ménière. Para o potencial evocado miogênico vestibular ocular o prolongamento da latência das ondas N10 e P15 foi a alteração mais encontrada no grupo de estudo. Conclusão: O potencial evocado miogênico vestibular cervical e ocular combinado apresentou resultados relevantes para os indivíduos com hiporreflexia vestibular e para os indivíduos com doença de Ménière. Houve diferença entre o grupo de estudo e o grupo controle para a maioria dos parâmetros das ondas no potencial evocado miogênico vestibular cervical e ocular combinado.

Migraine patients consistently show abnormal vestibular bedside tests / Pacientes enxaquecosos consistentemente apresentam testes vestibulares de beira-de-leito anormais

Migraine and vertigo are common disorders, with lifetime prevalences of 16% and 7% respectively, and co-morbidity around 3.2%. Vestibular syndromes and dizziness occur more frequently in migraine patients. We investigated bedside clinical signs indicative of vestibular dysfunction in migraineurs. Objective To test the hypothesis that vestibulo-ocular reflex, vestibulo-spinal reflex and fall risk (FR) responses as measured by 14 bedside tests are abnormal in migraineurs without vertigo, as compared with controls. Method Cross-sectional study including sixty individuals – thirty migraineurs, 25 women, 19-60 y-o; and 30 gender/age healthy paired controls. Results Migraineurs showed a tendency to perform worse in almost all tests, albeit only the Romberg tandem test was statistically different from controls. A combination of four abnormal tests better discriminated the two groups (93.3% specificity). Conclusion Migraine patients consistently showed abnormal vestibular bedside tests when compared with controls.

Enxaqueca e vertigem são desordens comuns, com prevalência de 16% e 7% respectivamente, e comorbidade em torno de 3,2%. Síndromes vestibulares e tonturas ocorrem mais frequentemente em enxaquecosos. Pesquisamos alterações vestibulares utilizando testes de beira-de-leito em enxaquecosos. Objetivo Verificar se as respostas dos reflexos vestíbulo-ocular, vestíbulo-medular e risco de quedas medidas por 14 testes de beira-de-leito são diferentes comparando-se enxaquecosos sem vertigem, e controles. Método Estudo transversal com sessenta pessoas, 30 enxaquecosos; 25 mulheres, 19-60 anos; e trinta controles saudáveis pareados por sexo e idade. Resultados Houve tendência de pior desempenho entre enxaquecosos em quase todos testes, porém apenas o teste de Romberg tandem foi estatisticamente diferente dos controles. Uma combinação de quatro testes anormais discrimina os grupos com especificidade de 93,3%. Conclusão O grupo de enxaquecosos mostrou consistentemente testes vestibulares de beira-de-leito anormais quando comparados a controles.

The vascular and neurogenic factors associated with erectile dysfunction in patients after pelvic fractures

ABSTRACT Erectile dysfunction (ED) is a common complication of pelvic fractures. To identify the vascular and neurogenic factors associated with ED, 120 patients admitted with ED after traumatic pelvic fracture between January 2009 and June 2013 were enrolled in this study. All patients answered the International Index of Erectile Function (IIEF-5) questionnaire. Nocturnal penile tumescence (NPT) testing confirmed the occurrence of ED in 96 (80%) patients on whom penile duplex ultrasound and neurophysiological testing were further performed. Of these ED patients 29 (30%) were demonstrated only with vascular abnormality, 41 (42.7%) were detected only with neural abnormality, 26 (27.1%) revealed mixed abnormalities. Of the 55 patients (29+26) with vascular problems, 7 patients (12.7%) with abnormal arterial response to intracavernous injection of Bimix (15mg papaverine and 1mg phentolamine), 31 (56.4%) with corporal veno-occlusive dysfunction and 17 (30.9%) had both problems. Of the 67 (41+26) patients with abnormal neurophysiological outcomes, 51 (76.1%) with abnormal bulbocavernosus reflex (BCR), 20 (29.9%) with pathological pudendal nerve evoked potentials (PDEPs) and 25 (37.3%) with abnormal posterior tibial somatosensory nerve evoked potentials (PTSSEPs). Our observation indicated that neurogenic factors are important for the generation of ED in patients with pelvic fracture; venous impotence is more common than arteriogenic ED.

Clinical Heterogeneity in Korean Patients with Nemaline Myopathy

PURPOSE: Nemaline myopathy (NM) is a clinical heterogeneous congenital myopathy characterized by the presence of subsarcolemmal or cytoplasmic rod-like structures that call nemaline bodies in the muscle fibers. The purpose of this study was to investigate the clinical diversity and pathological features of Korean patients with NM. MATERIALS AND METHODS: Eight patients underwent analyses of clinical manifestations by a structured protocol. Diagnoses were established by a muscle biopsy. RESULTS: Two patients had the typical congenital type, which exhibited neonatal hypotonia and delayed motor milestone, and five patients had the childhood onset type, which exhibited mild gait disturbance as a first symptom. One patient had the adult onset type, which showed acute respiratory failure. Limb weakness was proximal-dominant occurred in six patients. Hyporeflexia was observed in most patients. Elongated faces and high arched palates and feet were also observed. On light microscopy, the nemaline bodies were observed in type 1 and 2 fibers. All patients showed type 1 predominance and atrophy. In the two cases in which ultrastructural studies were performed, typical nemaline rods and disorganized myofibrillar apparatus were detected. CONCLUSION: In conclusion, the eight Korean patients in this study with NM shared common clinical expressions such as proximal limb weakness, reduced deep tendon reflex, and dysmorphic features. This study, however, showed that clinical heterogeneity ranged from typical congenital, mildly affected childhood to the adult onset form with acute respiratory failure. The pathological findings in this study were in accordance with those of other previous reports.

Presbivertigem como causa de tontura no idoso / Presbyvertigo as a cause of dizziness in elderly

INTRODUÇÃO: tontura é queixa freqüente na população geriátrica e interfere na qualidade de vida desses indivíduos. OBJETIVO: descrever as causas de tontura, correlacionar sintomas e alterações na prova calórica e verificar o impacto da presbivertigem como fator primário de tontura em população de idosos. MÉTODO: revisão do prontuário de 132 pacientes com mais de 60 anos e distúrbio do equilíbrio corporal, atendidos no ambulatório de Otoneurologia do Hospital das Clínicas da UFMG, no período de 1998 a 2007. As variáveis analisadas foram: dados epidemiológicos, história clínica, doenças associadas e resultado da prova calórica. Casos de vertigem de posição e suspeita de lesão central foram excluídos da análise. RESULTADOS: A amostra constou de 120 pacientes. A idade média foi de 70 anos, sendo 87 (71 por cento) mulheres. Dentre os tipos de tontura, vertigem de alguns minutos de duração e freqüência diária foi mais freqüente. Em relação ao resultado da prova calórica, exame normal foi observado em 73 por cento e, dentre os resultados alterados, hiporreflexia bilateral (presbivertigem) foi observada em 8 por cento. Correlacionando-se com o tipo de tontura, hiporreflexia bilateral associou-se com instabilidade postural (p = 0,006; IC = 2 - 419). CONCLUSÃO: tontura no idoso tem causa multifatorial. Perda da função vestibular periférica pode estar relacionada à presbivertigem e deve ser considerada em pessoas idosas com desequilíbrio. Distúrbios metabólicos, psíquicos, disautonômicos, ortopédicos, visuais e de propriocepção podem ser causa de tontura em idosos com exame vestibular normal.

BACKGROUND: dizziness is a frequent complaint in the geriatric population and has a negative impact in the life quality of these individuals. AIM: to correlate the types of dizziness and alterations in the caloric test and to verify the frequency of presbyvertigo in an elderly population with dizziness. METHOD: The records of 132 patients aged over 60 and with balance disturbance were reviewed. These patients were evaluated in the Otoneurology Service of the Clinical Hospital of the Federal University of Minas Gerais between the years of 1998 and 2007. The variables considered for analysis were: epidemiologic data, clinical history, associated diseases and the result of the caloric test. Patients with positional and central vertigo were excluded from the analysis. RESULTS: The research sample consisted of 120 patients, with an average age was of 70 years, being 71 percent (n=87) of sample women. Vertigo with the duration of a few minutes and of a daily frequency was the most frequent type of dizziness. In relation to the caloric test, normal results were observed in 73 percent of the sample. Altered results included unilateral weakness (14 percent), bilateral weakness (10 percent) and hyperactive caloric response (3 percent). Correlating the caloric test with the type of dizziness, bilateral weakness was associated with postural instability (p=0.006; IC=2 - 419). CONCLUSION: dizziness in elderly has many causes. Bilateral weakness of the vestibular function can be related to presbyvertigo and must be considered in aged individuals who present unbalance. Metabolic, psychiatric, disautonomic, orthopedic, visual and proprioceptive disturbances may be the cause of dizziness in aged individuals who present normal vestibular evaluation.

Posterior sagittal anorectoplasty in anorectal anomalies: clinical, manometric and profilometric evaluation

CONTEXT AND OBJECTIVE: Anorectal malformations comprise a spectrum of anomalies that continue to be difficult to treat, even today. The aim was to evaluate the fecal continence of children who underwent posterior sagittal anorectoplasty due to anorectal malformations, via computerized anorectal manometry and profilometry. DESIGN AND SETTING: Prospective study at Universidade Federal de São Paulo. METHOD: 82 patients (56.1 percent boys; 43.9 percent girls) of mean age 85.5 months were evaluated. They were divided into continent, partially continent and incontinent groups. Age, sex, manometric variables and profilometric parameters were studied. The results were statistically analyzed. RESULTS: Among the 82 patients, 37.8 percent were continent, 25.6 percent were partially continent and 36.6 percent were incontinent. The overall mean resting pressure was 22 mmHg, and the means for the continent, partially continent and incontinent groups were, respectively, 30.7 mmHg, 23 mmHg and 14.7 mmHg. The overall mean pressure response to voluntary contraction was 56 mmHg, and the means for the groups were 65.4 mmHg, 55.8 mmHg and 46.6 mmHg, respectively. The rectosphincteric reflex was absent in 82.9 percent of the cases. In the profilometry analysis for all patients together, blue (20 to 50 mmHg) and yellow (50 to 80 mmHg) were predominant, and there was a similar distribution for the continent and partially continent patients. However, among the incontinent patients, green (< 20 mmHg) and blue prevailed. CONCLUSIONS: Manometric and computerized profilometric analyses were an excellent method for postoperative evaluations on patients with intermediate and high anorectal anomalies, and for therapeutic planning.

CONTEXTO E OBJETIVO: As anomalias anorretais correspondem a um espectro de malformações de tratamento difícil mesmo nos dias de hoje. O objetivo foi avaliar crianças portadoras de anomalias anorretais altas e intermediárias, operadas pela anorretoplastia sagital posterior quanto à continência fecal através da manometria anorretal e profilometria computadorizadas. TIPO DE ESTUDO E LOCAL: Trabalho prospectivo, na Universidade Federal de São Paulo. MÉTODO: Avaliamos 82 pacientes agrupados em continentes, parcialmente continentes e incontinentes quanto a idade, sexo e variáveis padronizadas na manometria anorretal e profilometria. Os resultados foram analisados estatisticamente. RESULTADOS: Dos 82 pacientes 37,8 por cento eram continentes, 25,6 por cento parcialmente continentes e 36,6 por cento incontinentes. A média da pressão de repouso à manometria anorretal foi de 22 mmHg, sendo entre os continentes, parcialmente continentes e incontinentes, respectivamente de 30,7 mmHg, 23 mmHg e 14,7 mmHg. A média da resposta pressórica à contração voluntária foi de 56 mmHg, sendo entre os continentes 65,4 mmHg, parcialmente continentes 55,8 mmHg e incontinentes 46,6 mmHg. O reflexo reto-esfincteriano encontrava-se ausente em 82,9 por cento dos casos. Predominaram na profilometria as cores azul (20 a 50 mmHg) e amarela (50 a 80 mmHg), quando todo o grupo foi analisado conjuntamente, com padrão semelhante entre os continentes e parcialmente continentes; nos incontinentes, destacaram-se as cores verde (< 20 mmHg) e azul. CONCLUSÕES: A manometria anorretal computadorizada e a profilometria mostraram-se úteis na avaliação do comportamento pressórico esfincteriano, assim como no acompanhamento pós-operatório e planejamento terapêutico dos pacientes.

Primitive reflexes and cognitive function

CONTEXTO: A prevalência e significado patológico dos reflexos primitivos (RP) no adulto, bem como sua relação com a idade e a cognição, são questões controversas. OBJETIVO: Estudar a relação entre RP e cognição em 30 pacientes com doença de Alzheimer (DA) e 154 sujeitos controles normais. MÉTODO: O diagnóstico de DA baseou-se nos critérios DSM-IV, NINCDS-ADRDA e CAMDEX. Os RP foram quantificados de 0 (ausente) a 1 (leve) ou 2 (acentuado). CASI-S (Cognitive Abilities Screening Instrument -Short Form) foi usado para avaliar o registro, orientação temporal, fluência verbal e evocação. Um teste de cópia de pentágonos foi acrescentado. RESULTADOS: Os RP mais frequentes nos pacientes e controles foram o de sucção (77% e 62%, respectivamente) e "snout" (60% e 27%), seguidos do glabelar (30% e 19%), paratonia (37% e 5%) e palmomentoniano (23% e 5%). Nenhum sujeito controle teve mais que três RP. A frequência dos RP tendeu a aumentar com a idade e a deterioração cognitiva. O reflexo de preensão e o sinal de Babinski foram encontrados apenas nos pacientes com DA. Os RP não se correlacionaram uns com os outros, exceto o reflexo "snout" com o de sucção e com o glabelar.CONCLUSÃO: O achado de reflexo de preensão e sinal de Babinski, ou a presença de mais de três sinais primitivos, particularmente a combinação de paratonia e reflexos "snout", sucção e palmomentoniano, são sugestivos de disfunção cerebral, especialmente quando esses sinais são acentuados e acompanhados de déficits de orientação, evocação, fluência verbal e praxia construcional.

Manifestaçoes neurológicas em crianças e adolecentes infectados e expostos ao HIV-1 / Neurological findings in a group of children and adolescents exposed and infected by HIV-1

The CNS infection by HIV-1 in infancy could be present immediately after infection or became manifest later. Microcephalia, mental retardation, pyramidal signs, humor and behavioral disorders and antiretroviral therapy complications are common. This is an observational, sectional and descriptive study about findings on neurological examination of 173 patients in a group of children and adolescents infected and exposed to HIV-1 in perinatal period. Most of them had more than one neurological finding or different diagnosis. The more common findings were: encephalopathy, mental retardation, language delay, pyramidal signs, hyporeflexia. The neurological examination was abnormal in 67% of all patients even in seroreverters. We suggest that this group has a high risk to neurological disease and the development of co-morbidity is directly correlated to clinical deterioration by HIV-1 infection.

O envolvimento do sistema nervoso central SNC na infecção pelo HIV-1 em crianças pode estar evidente desde o início ou demorar muitos anos para se manifestar. Microcefalia, rebaixamento cognitivo, sinais piramidais, distúrbios do humor e do comportamento e complicações pelo uso da terapia antiretroviral são comuns. Este é um trabalho observacional, descritivo e seccional cuja finalidade é descrever as alterações do exame neurológico em um grupo de crianças e adolescentes expostos pelo HIV-1 durante o período perinatal. Foram avaliados 173 pacientes. Muitos pacientes tinham superposição de alterações de exame neurológico e/ou mais de um diagnóstico. As alterações mais comuns foram: retardo do desenvolvimento neuropsicomotor, atraso de linguagem, deficiência mental, síndrome piramidal, hiporreflexia. O exame neurológico foi alterado em 67% dos casos, mesmo naqueles pacientes soro-revertidos. Sugerimos que existe alto risco para doença neurológica nesse grupo de pacientes e que a progressão da infecção pelo HIV-1 acentua o aparecimento de co-morbidades e comprometimento de seu prognóstico.

Sinais de Babinski e Chaddock sem disfunção piramidal aparente / Babinski and Chaddock signs without apparent pyramidal disfunction

OBJETIVO: Verificar a presença dos sinais de Babinski e de Chaddock em cem pacientes sem história ou indícios clínicos de comprometimento da via piramidal, internados no Serviço de Clínica Médica do Hospital Universitário Clementino Fraga Filho da Universidade Federal do Rio de Janeiro. Como objetivos secundários, observar a possível prevalência de um sinal sobre o outro, assim como a influência da posição da cabeça sobre as respostas obtidas. MÉTODO: Cada um dos sinais foi pesquisado por um único autor, utilizando o mesmo instrumento, estando os pacientes em decúbito dorsal e com a cabeça em três posições. RESULTADOS: Em dez pacientes (10 por cento) obteve-se a resposta de extensão do hálux uni ou bilateral. O sinal de Babinski apresentou-se 18 vezes (40 por cento) e o sinal de Chaddock 27 vezes (60 por cento). CONCLUSÃO: O sinal de Chaddock foi o mais freqüente. As respostas anormais ocorreram duas vezes mais à esquerda e, aparentemente, não houve interferência da posição cefálica em relação às respostas obtidas.

Assessment of sensorial oral stimulation in infants with suck feeding disabilities.

A non-randomized single blind study was undertaken to determine the clinical and physiological changes in suck feeding after sensorial oral stimulation, in fourteen patients age 9 to 210 days old with sucking alterations. Patients lacked at least one of the five oral reflexes, plus two or more abnormal sucking sings or at least one abnormal sucking sign, plus two or more abnormal oral reflexes. Oral sensorial therapy was performed thrice daily for five days. The number of absent oral reflexes, number of abnormal sucking signs, volume of milk for nursing and sucking rate, were registered. Differences of medians were tested using Freidman's test and differential of proportions using Cochran's Q test. After therapy, oral reflexes were recovered (2, 0-4 vs. 5,5-5, p = 0.0000, median rank of absence oral reflexes) and the number of abnormal sucking signs decreased (6,1-9 vs. 1, 0-4; p = 0.0000). There were statistically significant improvements in patients who had lost launch up nipple ability (p = 0.005), delay at the beginning of sucking (p = 0.0022), drawing of milk from the mouth (p = 0.0001), cyanosis (p = 0.0084), weaning (p = 0.0004) and prolonged sucking (p = 0.0038). Even in patients with moderate improvement, no statistical differences were observed in nipple rooting (p = 0.09) and coughing (p = 0.09). No changes were observed in patients who had cried (p = 0.31) and spitted (p = 0.51) during feeding. At the end of therapy, volumes of consumed milk were increased at each feeding (10 ml, 0-40 vs. 50 ml, 25-60; p = 0.0001). Sucking rates also increased (22 sucks/minute, 10-35 vs. 40.5, 35-48; p = 0.0044). Oral sensorial and motor stimulation normalise oral motor reflexes, diminish the clinical abnormal sucking signs and increase milk volumes ingested for nursing.

The Guillain-Barré syndrome following dengue fever

A 44 year old female presented with fever, muscle aches, rash and a low platelet count. IgM antibody to dengue virus was positive. Two weeks later she developed a flaccid areflexic quadriparesis. Nerve conduction studies showed a predominantly demyelitinating sensory motor polyneuropathy consistent with Guillain-Barré syndrome. Despite the relatively common occurrence of dengue fever, an associated polyradiculoneuropathy is distinctly uncommon.

rational treatment of eclampsia built on proper understanding of the pathophysiology of the nervous system

In normal pregnant women, a normal external or internal stimulus reaching the brain produces a physiological response. In eciamptic patients, normal or even subnormal stimuli produce a massive response: namely a convulsion. A correlation between the pathophysiology of the nervous system in eclampsia and the state of neuroal hype; excitability is discussed. A rational for treatment in 20 eclamptic cases is outlines