Encefalopatía de Wernicke y polineuropatía asociada a déficit de complejo B después de una cirugía bariátrica / Wernicke´s encephalopathy and polyneuropathy associated with vitamin B complex deficiency after a bariatric surgery

La cirugía bariátrica es un tratamiento que garantiza una pérdida de peso sustancial y duradera, y beneficios tangibles respecto a condiciones médicas asociadas a la obesidad. El aumento del número de cirugías bariátricas ha llevado también a un aumento de las complicaciones relacionadas con ella, incluyendo la encefalopatía de Wernicke y la polineuropatía por deficiencia de vitaminas del complejo B. En este artículo se reporta un caso de encefalopatía de Wernicke siete semanas después de la cirugía, enfatizando en la importancia de reconocer el espectro de la sintomatología para hacer un diagnóstico temprano, que permita intervenir en la fase reversible de esta enfermedad potencialmente letal.

Bariatric surgery is a treatment that guarantees a substantial and lasting weight loss in addition to the tangible benefits relating to obesity-associated medical conditions. The increasing number of bariatric surgeries has revealed an increasing number of complications related to this procedure, including Wernicke´s encephalopathy and vitamin B deficiency polyneuropathies. Herein, a 7-week post-surgery case of Wernicke´s encephalopathy is presented that emphasizes the importance of an early recognition of these symptoms so as to initiate intervention during the reversible phase of these potentially lethal pathologies.

Inhibitory action of antioxidants (ascorbic acid or α-tocopherol) on seizures and brain damage induced by pilocarpine in rats / Ação inibitória de antioxidantes (ácido ascórbico e α-tocoferol) nas convulsões e dano cerebral em ratos induzidos pela pilocarpina

Temporal lobe epilepsy is the most common form of epilepsy in humans. Oxidative stress is a mechanism of cell death induced by seizures. Antioxidant compounds have neuroprotective effects due to their ability to inhibit free radical production. The objectives of this work were to comparatively study the inhibitory action of antioxidants (ascorbic acid or α-tocopherol) on behavioral changes and brain damage induced by high doses of pilocarpine, aiming to further clarify the mechanism of action of these antioxidant compounds. In order to determinate neuroprotective effects, we studied the effects of ascorbic acid (250 or 500 mg/kg, i.p.) and α-tocopherol (200 or 400 mg/kg, i.p.) on the behavior and brain lesions observed after seizures induced by pilocarpine (400 mg/kg, i.p., P400 model) in rats. Ascorbic acid or α-tocopherol injections prior to pilocarpine suppressed behavioral seizure episodes. These findings suggested that free radicals can be produced during brain damage induced by seizures. In the P400 model, ascorbic acid and α-tocopherol significantly decreased cerebral damage percentage. Antioxidant compounds can exert neuroprotective effects associated with inhibition of free radical production. These results highlighted the promising therapeutic potential of ascorbic acid and α-tocopherol in treatments for neurodegenerative diseases.

A epilepsia de lobo temporal é a mais comum forma de epilepsia em humanos. O estresse oxidativo é um dos mecanismos de morte celular induzida pelas crises convulsivas. Os compostos antioxidantes apresentam efeitos neuroprotetores devido à sua capacidade de inibir a produção de radicais livres. Os objetivos do presente trabalho foram estudar de forma comparativa a ação inibitória de antioxidantes (ácido ascórbico e α-tocoferol) sobre as alterações comportamentais e histopatológicas no hipocampo de ratos após convulsões induzidas pela pilocarpina. A fim de determinar os efeitos neuroprotetores destas drogas, o presente trabalho estudou os efeitos do ácido ascórbico (250 ou 500 mg/kg, i.p.) e do α-tocoferol (200 ou 400 mg/kg, i.p.) sobre o comportamento e as lesões cerebrais observados após convulsões induzidas pela pilocarpina (400 mg/kg, i.p., P400), em ratos. As injeções de ácido ascórbico ou α-tocoferol antes da administração de pilocarpina reduzem o número de animais que convulsionam. Estes achados sugerem que os radicais livres podem induzir o desenvolvimento de lesão cerebral durante as crises epilépticas. No modelo P400, o ácido ascórbico e o α-tocoferol, diminuem significativamente os danos cerebrais. Os compostos antioxidantes podem exercer efeitos neuroprotetores, e esses resultados podem estar associados à inibição da produção de radicais livres. Estes resultados sugerem um promissor potencial terapêutico tanto para o ácido ascórbico quanto para o α-tocoferol no tratamento de doenças neurodegenerativas.

Crianças com encefalopatia crônica não evolutiva: avaliação audiológica e próteses auditivas / Children with chronic non-progressive encephalopathy: hearing evaluation and hearing aids

OBJETIVO: avaliar a capacidade auditiva de crianças com encefalopatias crônicas não evolutivas (ECNE) independentemente de suspeita de perda auditiva e da etiologia e caracterizar o benefício do uso de prótese auditiva em crianças com ECNE que apresentaram perda auditiva. MÉTODO: avaliação neurológica, otorrinolaringológica e audiológica e aplicação do protocolo Parent's Evaluation of Aural / Oral Performance of Children (Peach). RESULTADOS: Das 46 crianças avaliadas, encontraram-se 22 (48 por cento) sem perdas e 24 (52 por cento) com algum grau de perda auditiva sensorioneural. Quanto às etiologias encontradas nas 46 crianças, a maior porcentagem é de encefalopatia hipóxica isquêmica seguida de processos infecciosos e kernicterus. Quanto à suspeita de perda auditiva, nas 16 (35 por cento) crianças cujos pais tiveram suspeita, o percentual de algum grau de perda auditiva foi de 56 por cento, e nas 30 (65 por cento) cujos pais não a tiveram, a avaliação audiológica revelou que 50 por cento apresentaram algum grau de perda auditiva. O protocolo Peach se mostrou um instrumento eficaz para avaliar o benefício da prótese auditiva. CONCLUSÃO: das crianças avaliadas, mais da metade apresentou perda auditiva, no entanto, não houve relação estatisticamente significante entre a etiologia e a suspeita de perda auditiva. Assim, consideramos que não é possível prever qualquer perda auditiva a partir da suspeita e recomendamos a avaliação auditiva em todas as crianças com ECNE, pois todas as crianças com perda auditiva examinadas neste estudo revelaram benefícios importantes com o uso da prótese auditiva.

AIM: to assess the auditory abilities of children with non-progressive chronic encephalopathy (NPCE), independently of the presence or not of hearing loss, and of the etiology of the encephalopathy; to characterize the benefit of hearing aids in children with NPCE and hearing loss. METHOD: neurologic, otorhinolaryngologic and auditory assessments. Application of the Parent's Evaluation of Aural/Oral Performance of Children (PEACH) protocol. RESULTS: out of the 46 assessed children, 22 (48 percent) presented no hearing loss and 24 (52 percent) presented some level of sensorineural hearing loss. Regarding the encephalopathy etiology, most of the participants presented ischemic hypoxic encephalopathy followed by infectious process and kernicterus. The results also indicate that 16 (35 percent) parents suspected that their child had hearing loss; out of this total, 56 percent had the hearing loss confirmed. Thirty parents (65 percent) did not have any hearing complaints about their children. For these children the auditory evaluation indicated that 50 percent presented some level of hearing loss. The PEACH protocol proved to be effective to assess the benefit of hearing aids. CONCLUSION: the results indicate that over half of participants presented hearing loss. No correlation was observed between etiology and complaints of hearing loss. This means that it is not possible to predict hearing loss based on complaints. All children who presented hearing loss benefited from the use of hearing aids.

Cárie dentária e higiene bucal em crianças com paralisia cerebral tetraparesia espástica com alimentação por vias oral e gastrostomia / Dental caries and oral hygiene in children with spastic tetraparetic cerebral palsy receiving either oral feeding or nutrition via gastrostomy tube

Objetivo: Avaliar as condições bucais quanto à experiência de cárie dentária e índice de higiene bucal em crianças com paralisia cerebral tetraparesia espástica (PCTE), com alimentação por vias oral e gastrostomia. Método: Foram avaliadas 36 crianças na faixa etária de 2 a 7 anos (5,2 ±1,8), que procuraram tratamento odontológico no Lar Escola São Francisco Centro de Reabilitação UNIFESP/EPM, e que apresentavam valores de motricidade oral ≤19 (severamente comprometidos), segundo a Orofacial Motor Function Assessment Scale. As crianças foram reunidas em dois grupos, segundo a via de alimentação oral (G1; n=20) e sonda gástrica (G2; n=16). Ambos os grupos foram avaliados quanto à experiência de cárie dentária e o Índice de Higiene Oral Simplificado (IHOS). Os resultados foram analisados estatisticamente, utilizando-se teste t Student, Quiquadrado e de Mann-Whitney, com nível de significância fixado em p<0,05. Resultados: Observou-se uma diferença estatisticamente significante (p=0,0415) entre os dois grupos quanto à experiência de cárie dentária, apresentando o G1 maiores valores. Quanto ao IHOS, observaram-se valores significantemente maiores (p=0,0401) para o G2. Conclusão: As crianças com PCTE e comprometimento motor oral severo, que se alimentam por via oral, apresentam maior risco da doença cárie dentária que os gastrostomizados de doença periodontal, embora ambos requeiram medidas preventivas para a manutenção da saúde bucal, bem como o tratamento odontológico individualizado, sempre respeitando as condições sistêmicas e odontológicas do paciente.

Culture- and antigen-negative meningitis in Guatemalan children / Meningitis negativa a pruebas antigénicas y de cultivo en niños guatemaltecos

OBJECTIVE: To compare children with confirmed bacterial meningitis (CBM) and those with culture- and latex-negative meningitis (CLN). METHODS: Children 1 to 59 months of age admitted to three major referral hospitals in Guatemala City with clinical signs compatible with bacterial infections were evaluated prospectively between 1 October 1996 and 31 December 2005. Bacterial cultures and latex agglutination antigen testing were performed on samples of cerebrospinal fluid (CSF). RESULTS: The case-fatality rate was significantly higher in the 493 children with CBM than in the 528 children with CLN (27.6 percent and 14.9 percent, respectively; P < 0.001). Children with CBM were less likely to have received antibiotics and more likely to have seizures, shock, or coma on admission than children with CLN. Among the 182 CBM survivors and 205 CLN survivors studied between October 2000 and December 2005, clinically observed sequelae were present at discharge in a higher percentage of the CBM than of the CLN group (78.6 percent and 46.8 percent, respectively; P < 0.0001). CSF glucose < 10 mg/dL, peripheral neutrophils < 2 000 cells/mm³, coma or shock at admission, and concurrent sepsis or pneumonia were risk factors for mortality in children with CBM; only coma or shock at admission predicted mortality in children with CLN. CONCLUSIONS: The high case-fatality and sequelae rates suggest that many children with CLN may have had bacterial meningitis. Estimates based on confirmed meningitis alone underestimate the true vaccine-preventable disease burden. Additional studies to determine etiologies of CLN in this population are indicated.

OBJETIVO: Comparar los casos infantiles de meningitis bacteriana confirmada (MBC) y meningitis negativa a pruebas de látex y de cultivo (MNLC). MÉTODOS: Se evaluaron los niños de 1 a 59 meses de edad ingresados en tres grandes hospitales de referencia de la Ciudad de Guatemala entre el 1 de octubre de 1996 y el 31 de diciembre de 2005 con signos clínicos de infección bacteriana. Se realizaron cultivos bacterianos y pruebas de aglutinación antigénica con látex en muestras de líquido cefalorraquídeo (LCR). RESULTADOS: La tasa de letalidad fue significativamente mayor en los 493 niños con MBC que en los 528 niños con MNLC (27,6 por ciento y 14,9 por ciento, respectivamente; P < 0,001). Los niños con MBC tuvieron menor probabilidad de recibir antibióticos y mayor de sufrir convulsiones, choques o entrar en coma al ser ingresados que los niños con MNLC. Se observó un mayor porcentaje de manifestaciones clínicas de secuelas al alta hospitalaria en los 182 niños sobrevivientes con MBC que en los 205 sobrevivientes con MNLC estudiados entre octubre de 2000 y diciembre de 2005 (78,6 por ciento y 46,8 por ciento, respectivamente; P < 0,0001). Los factores de riesgo de muerte en los niños con MBC fueron: glucosa en LCR < 10 mg/dL, neutrófilos periféricos < 2 000 células/mm³, coma o choque al ingreso, y sepsis o neumonía concurrentes; solo el coma y el choque al ingreso predijeron la muerte en niños con MNLC. CONCLUSIONES: Las altas tasas de letalidad y de secuelas indican que muchos niños con MNLC pueden haber tenido meningitis bacteriana. Las estadísticas basadas solamente en los casos confirmados de meningitis subestiman la verdadera carga de enfermedad prevenible mediante vacuna. Se deben emprender estudios adicionales para determinar las etiologías de la MNLC en esta población.

Estudio neuroepidemiológico nacional (EPINEURO) colombiano / National neuroepidemiological study in Colombia (EPINEURO)

OBJETIVO: Determinar la prevalencia de ocho problemas neurológicos -migraña, enfermedad cerebrovascular, enfermedad de Parkinson, neuropatía periférica, trastornos del desarrollo neurológico, epilepsia, demencia y secuelas de traumatismo craneoencefálico- en Colombia como parte del estudio neuroepidemiológico nacional (EPINEURO). MÉTODOS: Se realizó una encuesta transversal en dos fases entre septiembre de 1995 y gosto de 1996. En la primera fase se identificó a las personas que podían estar padeciendo alguna enfermedad neurológica y en la segunda se hizo el diagnóstico específico. Se xaminó una muestra de 8 910 personas distribuidas en proporción con la población de cada una de las cinco zonas geográficas de Colombia, que son las regiones entral, sudoccidental, noroccidental, oriental y la costa caribe. La evaluación se realizó según una versión odificada del protocolo de neuroepidemiología de la Organización Mundial de la Salud, además de un examen físico simplificado. A los posibles pacientes de demencia identificados se les aplicó un examen neuropsicológico, diseñado para este estudio, con el fin de profundizar en los aspectos cognoscitivos. Se aplicaron las escalas de Hachinski, Yesavage y Blessed y los criterios iagnósticos del NINCDS-ADRDA para la enfermedad de Alzheimer, los del NINDS-AIREN para la demencia de origen vascular y los del DSM-IV para todo tipo de demencia. Se realizó una ncuesta para determinar si había secuelas de traumatismo craneoencefálico. Las personas mayores de 12 años fueron evaluadas por neurólogos de adultos y los menores de 12 años por un neuropediatra. Se calcularon las prevalencias de trastornos neurológicos por edad y sexo, tanto por egión como para el país en su totalidad, y sus intervalos de confianza de 95 por ciento. RESULTADOS: La prevalencia por 1 000 habitantes de los trastornos investigados ueron las siguientes: migraña, 71,2 (intervalo de confianza de 95 por ciento [IC95 por ciento]: 65,5 a 76,8); trastornos del desarrollo neurológico, 46,1 (IC95 por ciento: 35,5 a 58,9); enfermedad cerebrovascular, 19,9 (IC95 por ciento: 14,3 a 27,4); demencia, 13,1 (IC95 por ciento: 8,5 a 9,3); epilepsia, 10,3 (IC95 por ciento: 8,5 a 13,0); neuropatía periférica, 8,5 (IC95 por ciento: 6,8 a 10,7); secuelas de traumatismo craneoencefálico, 6,4 (IC95 por ciento: 5,0 a 7,8) y enfermedad de Parkinson 4,7 (IC95 or ciento: 2,2 a 8,9)...

Cortical, brainstem and autonomic nervous system dysfunction in infants with post-hemorrhagic hydrocephalus

OBJECTIVE: The aim of this study was to evaluate the degree of central and autonomic dysfunction by means of electroencephalographic (EEG) and cardio-pneumographic recording (CPGR) in infants with Post-Hemorrhagic Hydrocephalus (PHH). METHODS: Nineteen infants with PHH were studied, born from October 1998 to January 2000. Clinical diagnosis was confirmed by cephalic perimeter measurement and ultrasonographic study. Eight channel bipolar EEG and CPGR were performed after sleep deprivation, EEG features studied were: Temporal organization, symmetry, synchrony, frequency of normal and abnormal patterns. CPGR features studied were: Frequency of apnea and temporal organization by means of beat to beat variability. RESULTS: Averages of birth's age and weight, Apgar scores at one and five minutes, and cephalic perimeter showed lower values in PHH infants. PHH infants showed abnormalities in EEG and CPGR such as: Higher discontinuous activity (63), asymmetry (73), asynchrony (89), abnormal EEG patterns (89), apnea (26) and absence of heart rate variability (63). CONCLUSIONS: EEG and CPGR recordings are a useful tools in the examination of infants with PHH. These findings suggest cortical-subcortical, and brainstem dysfunction with severe encephalopathy and autonomic nervous dysfunction related to ventricular dilatation in infants with PHH.

Globus Pallidus Lesions Associated with High Mountain Climbing

Acute mountain sickness (AMS) occurs commonly in hikers who are rapidly exposed to high altitude environments. Despite the numerous reports of AMS, few studies have reported pallidal lesions associated with altitude sickness. A previously healthy 49-yr-old Korean patient, after ascent to 4,700 m, suffered symptoms consistent with AMS. After returning home, the patient showed changes in personality characterized by abulia, indifference, and indecisiveness. T2 weighted brain magnetic resonance imaging showed high signal lesions involving bilateral globus pallidus. Our case suggests that globus pallidus injury should be included in the differential diagnosis of patients with personality or cognitive change after recovery from AMS.

Evidências da existência de um processo encefalopático crônico pós-isquêmico: perspectivas para a efetividade dos métodos fisioterápicos e para o tratamento de pacientes afásicos / Evidences of a post-ischemic chronic encephalopathic process: implications to efficacy of physical therapy and treatment of aphasia

Experimentalmente, a ocorrência de hipertermia após isquemia cerebral determinou o desencadeamento de um processo inflamatório crônico, associado à continuada perda de novos neurônios. Por outro lado, a antipirexia durante a fase inicial de recuperaçäo evitou a neurodegeneraçäo crônica posterior. A afirmaçäo desses fenômenos em pacientes vítimas de acidente cérebro-vascular isquêmico - freqüentemente acometido por complicaçöes clínicas associada à hipertermia - tem profundas implicaçöes para a recuperaçäo das seqüelas próprias dessa situaçäo, entre as quais se inclui a afasia. O desenvolvimento de terapêuticas farmacológicas capazes de bloquear tal processo neurodegenerativo pode ensejar a desejada reorganizaçäo estrutural do tecido nervoso em resposta à fisioterapia e aos métodos fonoaudiológicos

Rett Syndrome: a difficult differential diagnosis in prelingual non-verbal children

Rett's disease, is a condition rarely met with in clinical phoniatric practice. The present work deals with 2 girls [3 and 3.8 years] met with during a period of 17 years. The routine scheme applied on brain damaged motorly handicapped children was applied on both girls. Both cases showed severe and nearly equal affection of all abilities motoric, manual reflexive, mental, social and language abilities. Although, no special therapeutic program was reported in the literature yet language and physical therapy were presented as a trial. The results of applying both lines of therapy are unsatisfactory regarding the period of follow up. Rett's disease should be suspected by the treating team when the response to language and physical therapy is rather slow, a point of prognostic significance more work is still needed to collect and study these cases in order to understand the pathophysiology of this rare condition

A study on nesidioblastosis in hyperinsulinemic hypoglycemia: diagnosis, treatment, and neurologic sequelae

The medical records of six cases of nesidioblastosis were examined to determine the diagnostic approach, treatment, and neurologic sequelae. All six patients were male, and their ages at the onset of the disease ranged from one day to six months (mean 3.36 +/- 2.5 mo.). Initial clinical features were seizure, cyanosis, poor feeding, and apnea. Other subsequent symptoms were developmental delay, hyperactivity, and cold sweating. The Birth weight of the neonatal onset group was heavier than the postneonatal onset group (4.4 +/- 0.3 vs 3.26 +/- 0.04 kg). Before the diagnosis of hyperinsulinism, steroids of ACTH proved effective for seizure control. Initially, hyperinsulinemia (serum insulin greater than 10 microU/ml) was detected in four cases, but another two cases also showed hyperinsulinism by insulin/glucose(I/G) ratio greater than 0.3 during the fasting test. The glucagon response performed in 2 cases, showed normal and partial responses. Euglycemia was obtained by near total pancreatectomy (95% pancreatic resection)without malabsorption or persistent diabetes. In one case, nesidioblastoma coexisted with nesidioblastosis. Developmental delay was noted in three cases. In this group, the mean duration between symptom onset and operation was longer than the group without developmental delay (1.25 +/- 0.47 vs 0.38 +/- 0.19 yr).

Birth asphyxia and neurodevelopmental outcome.

Thirty six neonates with severe birth asphyxia (Apgar score less than or equal to 3 at 1 min), 32 with moderate birth asphyxia (Apgar score 4 to 6 at 1 min) and 35 controls (Apgar score greater than or equal to 7 at 1 min) matched for weight and gestation were followed up prospectively for neurodevelopmental outcome. Fetal distress occurred more frequently in babies with severe birth asphyxia when compared to controls (p less than 0.05). Six neonates with severe birth asphyxia had abnormal neurological signs such as delayed sucking, hypo or hypertonia, apneic spell or seizures. Of these, only two had delayed developmental milestones (Developmental Quotient less than 70) and features of cerebral palsy. Both of these babies developed seizures during first 24 hours, did not suck and required gavage feeding. The study highlights the fact that a vast majority of survivors of birth asphyxia enjoy good quality of life thus emphasizing the need for vigorous management of asphyxiated babies at birth.