Factores asociados a epilepsia farmacorresistente en niños atendidos en un hospital de referencia nacional / Factors associated with drug-resistant epilepsy in children treated at a national referral hospital

Antecedentes: Según la Organización Mundial de la Salud (OMS) cerca de 70 millones de personas en el mundo padecen epilepsia. Los países de medianos y bajos ingresos presentan 70-80% de los casos; se estima que afecta 4-13% de los niños hasta los 16 años, de los cuales la epilepsia farmacorresistente (EFR) se desarrolla en 10-23%. Objetivo: Determinar factores de riesgo asociados a EFR en pacientes pediátricos atendidos en Hospital María, Especialidades Pediátricas (HMEP), Tegucigalpa, marzo 2017-marzo 2022. Métodos: Estudio de casos-controles. A partir del total de pacientes menores de 18 años con diagnóstico de epilepsia atendidos en el Servicio de Neurología HMEP, se definió Caso como pacientes con diagnóstico de EFR y Controles como pacientes con epilepsia no farmacorresistente (ENFR). A partir de expedientes clínicos, se evaluaron factores sociodemográficos, antecedentes personales y familiares, factores clínicos, estudios de imagen y electroencefalograma. El estudio fue aprobado por el Comité de Ética Institucional. Resultados: Se analizaron 81 casos y 162 controles. La edad más afectada en casos fue preescolar (35.8%), en controles fue edad escolar (41.4%). El sexo masculino presentó similar distribución en ambos grupos (51.8% y 51.2%). La procedencia rural fue más frecuente en los casos que en controles (58.0% versus 48.8%). Se identificaron los siguientes factores asociados a EFR: Antecedentes familiares de epilepsia (ORa 2.32, IC95%1.22­4.41, p=0.01), alteración focal en examen físico (ORa 2.23, IC95%1.10­4.55, p=0.02), neurodesarrollo anormal (ORa 2.78, IC95%1.18­6.54, p=0.02). Discusión: El control adecuado de las crisis epilépticas incide directamente en la calidad de vida y sobrevida de los pacientes. La identificación correcta de los niños con epilepsia con los factores asociados identificados en este estudio, que coinciden con lo descrito internacionalmente, permitirá hacer un mejor tamizaje y priorizar la referencia temprana a un neurólogo pediatra contribuyendo a mejorar la calidad de vida de los pacientes...(AU)

Bilateral fracture of the shoulders following epileptic seizures: a case report / Fratura bilateral de ombros após convulsões epilépticas: relato de caso

Convulsive seizures caused by hyponatremia occur when this condition is severe and develops quickly, resulting in a brain's adaptive inability to contain brain swelling. Seizures are rarely the cause of shoulder fractures. This is a case report of bilateral humerus fracture following a single epileptic seizure caused by drug hyponatremia, an unconventional event in medical practice. A 69-year-old woman was admitted to the emergency room after a single tonic-clonic seizure with spontaneously ceased sphincter relaxation, showing Glasgow 6. No falls or restraint were reported by observers. When alert, the patient reported pain and difficulty moving both arms. During examination, the movement was li- mited to the right and left. Anteroposterior radiographs revealed bilateral fracture at the neck of humerus. To complement inves- tigation for further lesions, a computed tomography confirmed bilateral fracture-dislocation with impaction of the humeral head with the glenoid. Atraumatic bilateral fracture-dislocation of the humerus after epileptic seizure is a very rare event. It is believed that some of these diagnoses have been neglected due to the difficulty of characterizing the patient's pain in a postictal state. The importance of a detailed physical examination shall be emphasized in risk groups such as the polymedicated elderly.

Convulsive seizures caused by hyponatremia occur when this condition is severe and develops quickly, resulting in a brain's adaptive inability to contain brain swelling. Seizures are rarely the cause of shoulder fractures. This is a case report of bilateral humerus fracture following a single epileptic seizure caused by drug hyponatremia, an unconventional event in medical practice. A 69-year-old woman was admitted to the emergency room after a single tonic-clonic seizure with spontaneously ceased sphincter relaxation, showing Glasgow 6. No falls or restraint were reported by observers. When alert, the patient reported pain and difficulty moving both arms. During examination, the movement was li- mited to the right and left. Anteroposterior radiographs revealed bilateral fracture at the neck of humerus. To complement inves- tigation for further lesions, a computed tomography confirmed bilateral fracture-dislocation with impaction of the humeral head with the glenoid. Atraumatic bilateral fracture-dislocation of the humerus after epileptic seizure is a very rare event. It is believed that some of these diagnoses have been neglected due to the difficulty of characterizing the patient's pain in a postictal state. The importance of a detailed physical examination shall be emphasized in risk groups such as the polymedicated elderly.

Evacuación de hematoma subdural crónico: hemorragia de Duret postoperatoria y restitución ad-integrum. A propósito de un caso / Evacuation of chronic subdural hematoma: postoperative Duret hemorrhage and ad-integrum replacement. About a case

El tratamiento de elección para la evacuación de hematoma subdural crónico es la evacuación mediante orificio de trépano. Las complicaciones más frecuentes son recurrencia, neumoencefalo, convulsiones, hemorragia intracraneal y en otros sitios. La hemorragia del tronco cerebral secundaria a la cirugía es extremadamente rara. Aquí presentamos un paciente masculino de 72 años, que ingresa con GSC 9/15, hemiparesia izquierda 2/5. Con HSD crónico bilateral con desplazamiento de línea media de 1.5 cm. hacia izq. Al cual se realiza evacuación de HSD derecho. Y en el POP se detecta GSC 12/15, lado izquierdo fuerza 5/5, con hemiparesia derecha a predominio braquial. TC control POP: adecuada evacuación de HSD y lesión hiperdensa protuberancial. Es externado al 6to día POP con GSC 14/15 sin déficit motor. Este reporte, además de abordar la bibliografía actual y fisiopatología; agrega otro caso para reafirmar la posibilidad de pronóstico favorable en ésta patología

ABSTRACT The treatment of choice for the evacuation of chronic subdural hematoma is evacuation through a trepan orifice. The most frequent complications are recurrence, re-accumulation of the hematoma, pneumoencephalus, seizures, intracranial hemorrhage and elsewhere. Brainstem hemorrhage secondary to surgery is extremely rare. Here we present a 72-year-old male patient, admitted with GSC 9/15, left hemiparesis 2/5. Without obeying orders. With bilateral chronic HSD with midline displacement of 1.5 cm. to left; to which evacuation of right HSD is performed. And in the POP GSC 12/15 is detected, left side force 5/5, with right hemiparesis to brachial predominance. POP control CT: adequate evacuation of HSD and hyperdense pontine lesion. It is extership to the 6th day POP with GSC 14/15 without motor deficit. This report, besides addressing the current bibliography and physiopathology; adds another case to reaffirm the possibility of favorable prognosis in this pathology

Síndrome de Turcot / Turcotꞌs syndrome

RESUMEN Se presenta a una paciente de 27 años de edad, con varios ingresos en el Hospital Provincial Clínico Quirúrgico Docente “José Ramón López Tabrane”, de Matanzas. Por presentar clínica compatible con pólipos de colon, corroborados por videocolonoscopia, diagnosticados en marzo de 2015. Posteriormente reingresa a los 14 meses por convulsiones, al inicio generalizado y luego limitado al hemicuerpo derecho, cefalea universal y vómitos. Falleció a los 23 días de su ingreso, en los antecedentes patológicos familiares destaca madre fallecida a los 52 años por cáncer de colon y hermana a los 21 años por neoplasia maligna colorectal (AU).

ABSTRACT The case of a female patient, aged 27 years is presented. She was in-patient in the Teaching Clinical Surgical Provincial Hospital “José Ramón López Tabrane”, of Matanzas for several times presenting clinical characteristics compatible polyps in the colon, corroborated by video colonoscopy, and diagnosed in March 2015. She was readmitted 14 months later because of convulsions, generalized firstly and lately limited to the right side of the body, universal headache and vomits. She died 23 days after the admittance. The family history shows that her mother died when she was 52, due to colon cancer and her sister died at twenty one due to colorectal malignant neoplasia (AU).

Encefalitis autoinmunitaria asociada a anticuerpos contra el receptor N-metil-D-aspartato: presentación de dos casos / Autoimmune encephalitis associated to antibodies against the N-methyl-D-aspartate receptor: Report of two cases

Resumen La encefalitis asociada a anticuerpos contra receptores N-metil-D-aspartato es un síndrome neurológico que se presenta más comúnmente en mujeres jóvenes y frecuentemente se asocia al teratoma de ovario. Se caracteriza por un cuadro clínico agudo con síntomas generales inespecíficos que evoluciona hacia deterioro neurológico, psicosis y convulsiones; en su etapa más avanzada, se asocia con movimientos anormales y disautonomía. Se reportan dos casos en mujeres de 23 y 12 años. Dada su baja incidencia, se explica el proceso clínico que llevó a su diagnóstico y las opciones de tratamiento empleadas.

Abstract Anti-N-methyl-D-aspartate receptor encephalitis is a neurological syndrome that is more common in young women and is often associated with ovarian teratoma. It is characterized by acute general unspecific symptoms that evolve to neurological deterioration, psychosis and seizures. In its more advanced stage it is associated with abnormal movements and dysautonomia. We report two cases in women of 23 and 12 years of age. Given its low incidence, we present the clinical exercise that led to their diagnoses and the treatment options employed.

Tumor fibroso solitario meníngeo / Meningeal fibrous solitary tumor

Se presenta caso de paciente masculino de 28 años, sin antecedentes médicos de importancia, con síndrome convulsivo primer evento. Se le realiza estudio de imagen donde se documenta lesión ocupativa extraaxial temporo ­ occipito ­ cerebelar derecha. Paciente es llevado a resección de tumor. Se obtiene hallazgo histopatológico de tumor fibroso solitario meningeo. Además se documenta foco irritativo cortical a este mismo nivel. El tumor fibroso solitario meningeo representa un espectro de tumores mesenquimales, agrupado actualmente por la Clasificación de la OMS como Tumor fibroso solitario/ Hemangiopericitoma grado 1. El tratamiento se basa en resección quirúrgica amplia y vigilancia a largo plazo...(AU)

Psychogenic non-epileptic seizures in the post-anesthesia recovery unit / Convulsões não epilépticas psicogênicas em sala de recuperação pós-anestésica

Abstract Introduction: Psychogenic non-epileptic seizures (PNES or “pseudoseizures”) remain an obscure topic in the peri-operative setting. They are sudden and time-limited motor and cognitive disturbances, which mimic epileptic seizures, but are psychogenically mediated. Pseudoseizures occur more frequently than epilepsy in the peri-operative setting. Early diagnosis and management may prevent iatrogenic injury. Case: 48 year-old female with a history of depression and “seizures” presented for gynecologic surgery. She described her seizure history as “controlled” without anticonvulsant therapy. The patient underwent uneventful general anesthesia and recovered neurologically intact. During the first two postoperative hours, the patient experienced 3 episodes of seizure-like activity with generalized shaking of extremities and pelvic thrusting; her eyes were firmly closed. No tongue biting or incontinence was noted. The episodes lasted approximately 3 min each, one of which resolved spontaneously and the other two following intravenous lorazepam. During these episodes, the patient had stable hemodynamics and adequate ventilation such that endotracheal intubation was deemed unwarranted. Post-ictally, the patient was neurologically intact. Computed axial tomography of the head, metabolic assay, and electroencephalogram showed no abnormalities. A presumptive diagnosis of PNES was made. Discussion: Psychogenic non-epileptic seizures mimic shivering, and should be considered early in the differential diagnosis of postoperative shaking, as they may be more likely than epilepsy in this setting. Pseudoseizure patterns include asynchronous convulsive episodes lasting more than 90 s, forced eye closure with resistance to opening, and retained pupillary responses. Autonomic manifestations such as tachycardia, cyanosis and incontinence are usually absent. A psychiatric background is common. Knowledge and correct diagnosis of pseudoseizures is of great importance for anesthesiologists to prevent morbidity and iatrogenic injury such as respiratory arrest caused by anticonvulsant therapy, in addition to the risks associated with endotracheal intubation and prolonged hospital stays. The diagnosis of pseudoseizures must be thoroughly documented and relayed in transfer of care to avoid misdiagnosis and iatrogenic complications. Treatment recommendations are anecdotal; psychiatric interventions are the hallmark of treatment. Anesthetic recommendations include techniques involving the minimum required short-acting agents, along with high levels of peri-operative psychological support and reassurance.

Resumo Introdução: As convulsões não epilépticas psicogênicas (CNEP ou “pseudoconvulsões”) permanecem como tema obscuro no cenário perioperatório. Trata-se de distúrbios motores e cognitivos súbitos, mas por tempo limitado, que imitam as convulsões epilépticas, mas que são psicogenicamente mediados. Pseudoconvulsões ocorrem com mais frequência do que epilepsia em cenário perioperatório. O diagnóstico e o tratamento precoces podem evitar lesões iatrogênicas. Caso: Paciente do sexo feminino, 48 anos, com história de depressão e “convulsões”, apresentou-se para cirurgia ginecológica. A paciente descreveu sua história de convulsões “controladas” sem o uso de terapia anticonvulsivante. Foi submetida à anestesia geral sem intercorrências e recuperou-se neurologicamente intacta. Durante as duas primeiras horas de pós-operatório, apresentou três episódios semelhantes à convulsão, com tremores generalizados das extremidades e impulso pélvico; seus olhos estavam bem fechados. Não observamos mordedura da língua ou incontinência. Os episódios duraram cerca de três minutos cada; um dos episódios resolveu espontaneamente e os outros dois após a administração de lorazepam por via intravenosa. Durante os episódios, a condição hemodinâmica da paciente era estável e a ventilação adequada, de modo que a intubação traqueal foi considerada injustificável. Após a convulsão, a paciente estava neurologicamente intacta. Tomografia axial da cabeça, teste metabólico e eletroencefalograma não mostraram alterações. O diagnóstico de provável CNEP foi feito. Discussão: As convulsão não epilépticas psicogênicas imitam o tremor e devem ser inicialmente consideradas no diagnóstico diferencial de tremor pós-operatório, pois podem ser mais prováveis do que a epilepsia nesse cenário. Os padrões da pseudoconvulsão incluem episódios convulsivos assíncronos que duram mais de 90 segundos, olhos forçadamente fechados com resistência à abertura e respostas pupilares mantidas. Manifestações autonômicas, como taquicardia, cianose e incontinência, normalmente estão ausentes. Uma história psiquiátrica é comum. O conhecimento e o diagnóstico correto de pseudoconvulsões são muito importantes para os anestesiologistas para a prevenção de morbidade e lesões iatrogênicas, como a parada respiratória causada por terapia anticonvulsivante, além dos riscos associados à intubação orotraqueal e internação prolongada. O diagnóstico de pseudoconvulsões deve ser cuidadosamente documentado e retransmitido nas trocas de equipes médicas para evitar erros de diagnóstico e complicações iatrogênicas. As recomendações de tratamento são anedóticas; intervenções psiquiátricas são o pilar do tratamento. As recomendações anestésicas incluem técnicas que envolvem o uso de agentes de ação curta, juntamente com altos níveis de apoio e amparo psicológico no período perioperatório.

Absence seizure associated with coloprep consumption in colonoscopy / Convulsão de ausência com o uso de Coloprep em colonoscopia

Coloprep is a bowel preparatory solution given before endoscopic procedures to get a unobscured internal vision. It has among its constituent's sodium sulphate, potassium sulphate and magnesium sulphate which produce an osmotic effect in the bowel. However, the use of such agents in hyponatremic and patients predisposed to seizures can have adverse ramifications. The current case outlines manifestation of absence seizure in a 52-year-old male patient who was administered Coloprep for colonoscopy. There was absence of other predisposing factors and the symptoms were ameliorated using timely identification and rectification of the underlying derangements.

Coloprep é uma solução preparatória intestinal administrada antes de procedimentos endoscópicos, com o objetivo de se ter uma visão interna não obscurecida. Entre os constituintes de Coloprep, observa-se sulfato de sódio, sulfato de potássio e sulfato de magnésio, que provocam efeito osmótico no intestino. Mas o uso de tais agentes em pacientes hiponatrêmicos e com predisposição para convulsões pode ter ramificações adversas. O caso em tela delineia uma manifestação de convulsão de ausência em paciente do gênero masculino com 52 anos e que recebeu Coloprep para colonoscopia. Não havia outros fatores predisponentes e os sintomas melhoraram graças à oportuna identificação e correção dos transtornos subjacentes.

Diagnóstico tardio de síndrome de DiGeorge em criança hipocalcêmica: relato de caso / Late diagnosis of DiGeorge syndrome in hypocalcemic child: case report

A Síndrome de DiGeorge (SDG) decorre de uma microdeleção 22q11.2, sendo considerada uma das microdeleções mais frequentes em humanos. Caracteriza-se por espectro fenotípico bastante amplo, incluindo dificuldade de aprendizado, fácies dismórfica, anomalias cardíacas, hipocalcemia, hipoparatireoidismo, fenda palatina, anomalias do timo, insuficiência imunológica e problemas de fala e alimentação. Contudo, nenhum achado é patognomônico ou mesmo obrigatório. Este relato de caso pretende chamar a atenção para essa síndrome como causa potencial de hipocalcemia e convulsões hipocalcêmicas mesmo após o período neonatal. Reporta-se a história clinico-laboratorial e manejo de um menino de 12 anos, diagnosticado aos sete com SDG em decorrência de facies típica e crise convulsiva hipocalcêmica. O paciente apresentava diagnóstico prévio de transtorno do déficit de atenção e hiperatividade, atraso no desenvolvimento neuropsicomotor e fácies suspeita (micrognatia, orelhas de implantação baixa, hipertelorismo, nariz angular). A hipocalcemia que deflagrou a crise convulsiva foi secundária ao hipoparatireoidismo, sendo tratado com carbonato de cálcio e calcitriol. Houve melhora clínica, porém se manteve hipocalcêmico, apesar de dose otimizada da medicação. O caso é atípico, já que o diagnóstico de SDG foi feito tardiamente, visto que a maioria dos casos é diagnosticada no período neonatal. Além disso, o quadro demonstra a variabilidade de achados clínicos que podem ser encontrados nessa síndrome e a importância de se investigar a SDG em pacientes que apresentem hipocalcemia, mesmo em idades mais avançadas. Salienta-se que o diagnostico tem relevância na implicação dos cuidados à saúde, devido aos riscos imunológicos e cardiológicos apresentados pelos pacientes portadores, devendo ser realizado o mais precocemente possível.(AU)

The DiGeorge Syndrome (DGS) stems from a 22q11.2 microdeletion and is considered one of the most frequent microdeletions in humans. It is characterized by very wide phenotypic spectrum, including learning disability, dysmorphicfacies, cardiac abnormalities, hypocalcemia, hypoparathyroidism, cleft palate, thymus abnormalities, immune impairment and speech and feeding problems. However, any finding is pathognomonic or even mandatory. This case report aims to draw attention to this syndrome as a potential cause of hypocalcemia and hypocalcemic seizures even after the neonatal period. Refers to clinical and laboratory history and management of a boy of 12, diagnosed at 07 with DGS due to typical facies and hypocalcemic seizure. The patient had a previous diagnosis of attention deficit hyperactivity disorder, developmental delay and suspected facies (micrognathia, low-set ears, hypertelorism, angular nose). Hypocalcemia that triggered the seizure was secondary to hypoparathyroidism, being treated with calcium carbon- ate and calcitriol. There was clinical improvement, but hypocalcemic remained despite optimal medication dose. The case is atypical, since the diagnosis DGS was made later, as the majority of cases are diagnosed in the neonatal period. In addition, the table shows the variability of clinical findings that can be found in this syndrome and the importance of investigating the DGS in patients who have hypocalcaemia, even at older ages. Please note that the diagnosis is relevant in the involvement of health care due to immunological and cardiac risks posed by patients and should be done as early as possible.(AU)

Multiple Endocrine Neoplasia Type 1 Presenting as Hypoglycemia due to Insulinoma

Multiple endocrine neoplasia (MEN) mutation is an autosomal dominant disorder characterized by the occurrence of parathyroid, pancreatic islet, and anterior pituitary tumors. The incidence of insulinoma in MEN is relatively uncommon, and there have been a few cases of MEN manifested with insulinoma as the first symptom in children. We experienced a 9-year-old girl having a familial MEN1 mutation. She complained of dizziness, occasional palpitation, weakness, hunger, sweating, and generalized tonic-clonic seizure that lasted for 5 minutes early in the morning. At first, she was only diagnosed with insulinoma by abdominal magnetic resonance images of a 1.3 × 1.5 cm mass in the pancreas and high insulin levels in blood of the hepatic vein, but after her father was diagnosed with MEN1. We found she had familial MEN1 mutation, and she recovered hyperinsulinemic hypoglycemia after enucleation of the mass. Therefore, the early genetic identification of MEN1 mutation is considerable for children with at least one manifestation.

Seizure Outcome after Anterior versus Complete Corpus Callosotomy in Children: A Systematic Review with Meta-Analysis / Controle das crises epilépticas após calosotomia anterior versus completa em crianças: uma revisão sistemática com metanálise

Introduction Refractory epilepsy is a debilitating and challenging condition to manage. Corpus callosotomy (CC) seems to be an effective treatment option for patients with seizures not amenable to focal resection. The aim of the present study is to compare seizure outcome of pediatric patients following anterior CC, compared with complete CC. Method The authors performed a systematic review and meta-analysis of the English literature involving comparative studies. Results The present investigation includes four retrospective case-controlled studies and authors perform a pooled analysis of the surgical results. Seizure outcome presented favorable results in patients who underwent complete CC (Odds Ratio, M-H, Fixed, 95% CI: 3.02 [1.43, 6.387], p-value: 0.005). Clinical and neurological complications occurred independently when a complete or anterior CC was performed. Conclusion Complete CC seems to be the most effective treatment option to control intractable seizure in children not amenable to focal resection.

Introdução Epilepsia refratária é uma condição debilitante e desaadora para lidar. Calosotomia parece ser uma opção de tratamento ecaz para pacientes com convulsões não passíveis de ressecção focal. O objetivo do presente estudo é comparar o resultado de convulsões em pacientes pediátricos de acordo com calosotomia anterior e completa. Métodos Uma revisão sistemática e metanálise da literatura médica em inglês envolvendo estudos comparativos. Resultados Quatro casos retrospectivos foram incluídos na presente investigação e uma análise dos resultados cirúrgicos foi realizada. Convulsões decorrentes tiveram resultados favoráveis em pacientes submetidos a calosotomia complete (odds ratio, M-H, xo, 95% IC: 3,02 [1,43; 6,387], valor de p: 0,005). Complicações clínicas e neurológicas ocorreram independentemente de se calosotomia complete ou anterior. Conclusão Calosotomia completa parece ser a opção de tratamento mais ecaz para controlar convulsões não rastreáveis e não passíveis de ressecção focal em crianças.

Déficit adquirido de factor X en leucemia mielomonocítica crónica: reporte de un caso / Isolated factor X deficiency in chronic myelomonocytic leukemia: Report of one case

Bleeding disorders are commonly associated with hemato-oncologic diseases. We report a 68 years old male with a chronic myelomonocytic leukemia derived from a long lasting mielodysplastic syndrome that did not respond to treatment with Azacitidine. The patient was hospitalized due to tonic clonic seizures. A CAT scan showed a hematoma in the frontal lobe. A new assessment of hemostasis revealed an isolated deficiency of Factor X. We speculate that this deficit could be secondary to consumption due to the chronic Myelomonocytic Leukemia.

Multiorgan disorder syndrome (MODS) in an octagenarian suggests mitochondrial disorder / Síndrome mitocondrial no sindrómico con fallas de múltiples sistemas. Presentación de un caso

Non-syndromic, multi-organ mitochondrial disorders (MIDs) are frequently missed if treating physicians are not aware of them. We report a 85 years old Caucasian male, referred for tonic-clonic seizures, presenting with a plethora of abnormalities, including neurodermitis, atopic dermatitis, diabetes, hypertension, renal insufficiency, non-specific colitis, urine bladder lithiasis, bilateral cataracts, atrial fibrillation, diverticulosis, polyneuropathy, vitamin-D-deficiency, renal cysts, left anterior hemi-block, right bundle branch block, pulmonary artery hypertension, and heart failure. Neurological investigations revealed ptosis, quadriparesis, fasciculations, dysarthria, dysdiadochokinesia, tremor, hyperkinesia, ataxia, leukoencephalopathy, and basal ganglia calcification. Based upon this combination of abnormalities a non-syndromic mitochondrial multi-organ disorder syndrome (MIMODS, encephalo-myo-cardiomyopathy) was diagnosed.

Las alteraciones mitocondriales no sindrómicas y mutisistémicas pueden ser pasadas por alto si no se está consciente de su existencia. Presentamos un hombre de 85 años, referido por convulsiones tónico clónicas, que presentaba una plétora de anomalías tales como neurodermatitis, dermatitis atópica, diabetes, hipertensión, insuficiencia renal, colitis no específica, litiasis vesical, cataratas bilaterales, fibrilación auricular, diverticulosis, polineuropatía, deficiencia de vitamina D, quistes renales, hemibloqueo izquierdo anterior y bloqueo de rama derecha, hipertensión pulmonar e insuficiencia cardíaca. El estudio neurológico reveló la presencia de ptosis, cuadriparesia, fasciculaciones, disartria, disdiadocoquinesia, temblor, hiperquinesia, ataxia, leucoencefalopatía y calcificación de ganglios basales. Basados en estos hallazgos, se diagnosticó un síndrome mitocondrial no sindrómico con fallas de múltiples sistemas.

Primera convulsión en niños / First seizure in children

Introducción: Las convulsiones son la urgencia neurológica más frecuente en pediatría. 10% de la población tendrá un episodio convulsivo en algún momento de su vida. Objetivo: Determi- nar mediante la relación clínica, laboratorial, elec- troencefalográfica e imagenológica las causas de la primera convulsión en niños del Instituto Hon- dureño de Seguridad Social (I.H.S.S.) de San Pedro Sula, Honduras, durante el período junio 2013 a septiembre 2014. Pacientes y Métodos: Se realizó un estudio descriptivo longitudinal en el I.H.S.S., con un universo de 16930 niños atendi- dos en área de emergencia en el período descri- to, la muestra fueron 40 niños que asistieron por primera convulsión, con edades comprendidas entre un mes y 12 años. Los pacientes fueron valorados por neurólogo pediatra o pediatra, en emergencia y/o sala de Hospitalización. Resulta- dos: La primera convulsión se presentó en menores de dos años en el 72.5% de los casos (n=29). Los principales diagnósticos fueron el síndrome convulsivo febril en 45% (n=18) y epilepsia en 35% (n=14) de los casos. Se realiza- ron 19 electroencefalogramas obteniendo 4 con hallazgos anormales y 20 tomografías cerebrales, 5 con hallazgos anormales. Se hospitalizaron 85% de los niños con primera convulsión y en 65% (26) de los niños se inició terapia anticomi- cial oral de mantenimiento. Conclusiones: La población con riesgo de presentar primera convulsión son los menores de 2 años. Las causas principales de primera convulsión son las convulsiones febriles y la epilepsia...(AU)

Severe root resorption resulting from orthodontic treatment: Prevalence and risk factors

OBJECTIVE: To assess the prevalence of severe external root resorption and its potential risk factors resulting from orthodontic treatment. METHODS: A randomly selected sample was used. It comprised conventional periapical radiographs taken in the same radiology center for maxillary and mandibular incisors before and after active orthodontic treatment of 129 patients, males and females, treated by means of the Standard Edgewise technique. Two examiners measured and defined root resorption according to the index proposed by Levander et al. The degree of external apical root resorption was registered defining resorption in four degrees of severity. To assess intra and inter-rater reproducibility, kappa coefficient was used. Chi-square test was used to assess the relationship between the amount of root resorption and patient's sex, dental arch (maxillary or mandibular), treatment with or without extractions, treatment duration, root apex stage (open or closed), root shape, as well as overjet and overbite at treatment onset. RESULTS: Maxillary central incisors had the highest percentage of severe root resorption, followed by maxillary lateral incisors and mandibular lateral incisors. Out of 959 teeth, 28 (2.9%) presented severe root resorption. The following risk factors were observed: anterior maxillary teeth, overjet greater than or equal to 5 mm at treatment onset, treatment with extractions, prolonged therapy, and degree of apex formation at treatment onset. CONCLUSION: This study showed that care must be taken in orthodontic treatment involving extractions, great retraction of maxillary incisors, prolonged therapy, and/or completely formed apex at orthodontic treatment onset. .

OBJETIVO: avaliar a prevalência de reabsorções radiculares externas severas e identificar prováveis fatores de risco decorrentes do tratamento ortodôntico. MÉTODOS: utilizou-se uma amostra selecionada aleatoriamente, composta de radiografias periapicais de incisivos superiores e inferiores, obtidas no mesmo centro radiológico, de pré- e pós-tratamento ortodôntico ativo, de 129 pacientes, de ambos os sexos, tratados por meio da técnica Edgewise Standard. Dois examinadores mensuraram e definiram a reabsorção radicular de acordo com índice proposto por Levander et al., e o grau de reabsorção foi registrado, definindo a reabsorção em quatro graus de severidade. Para avaliar a reprodutibilidade intra- e interexaminadores, adotou-se o índice de coeficiente kappa ponderado. O teste chi-quadrado (χ2) foi adotado para avaliar a relação entre a quantidade de reabsorção radicular e o sexo dos pacientes, arcada dentária (superior ou inferior), tratamentos com ou sem extrações, duração do tratamento, forma radicular, estágio do ápice radicular (aberto ou fechado), overjet e overbite no início do tratamento. RESULTADOS: os incisivos centrais superiores apresentaram a maior porcentagem de reabsorção radicular severa, seguidos dos incisivos laterais superiores e dos incisivos laterais inferiores. Entre 959 dentes avaliados, 28 (2,9%) apresentaram reabsorção radicular severa. Os fatores de risco relacionados foram: dentes localizados na região anterossuperior, overjet maior ou igual a 5mm ao início do tratamento, tratamentos envolvendo extrações dentárias, tempo prolongado de terapia e formação radicular completa à época do início do tratamento ortodôntico. CONCLUSÃO: o estudo demonstrou que cuidados devem ser tomados em tratamentos ortodônticos envolvendo extrações, com grande retração de incisivos superiores, tratamentos prolongados e/ou ápice radicular completamente formado no início da terapia ortodôntica. .

Attention Deficit Hyperactivity Disorder in Epileptic Children

It is well-known that the prevalence of attention deficit hyperactivity disorder (ADHD) is higher in epileptic children than in the general pediatric population. The aim of this study was to compare the accompaniment of ADHD in epileptic children with well-controlled seizures and no significant intellectual disability with that in healthy controls. We included epileptic children between the ages of 6 and 12 yr visiting our clinic for six consecutive months and controls without significant medical or psychiatric illnesses. We excluded patients with intellectual disability or persistent seizures during the recent three months. The diagnosis of ADHD was based on the criteria of the Diagnostic and Statistical Manual of Mental Disorders-IV (DSM-IV). After exclusion of 84 patients, we enrolled 102 (54.8%) children (mean age, 9.4 +/- 2.0 yr). Seven (7 of 102, 6.9%) were diagnosed with ADHD. As compared to control group (4 of 110, 3.6%), there was no difference in ADHD accompaniment (P = 0.29). No difference was observed in ADHD accompaniment according to seizure type and epilepsy syndrome. In conclusion, the accompaniment of ADHD in epileptic children with well-controlled seizures and no intellectual disability may not differ from that of the general pediatric population.

Epilepsia en niños con esclerosis tuberosa: experiencia en el instituto autónomo hospital universitario de los Andes. Mérida 2005-2011 / Epilepsy in children with tuberous sclerosis autonomous institute experience in the university hospital of the Andes. Merida 2005-2011

La esclerosis tuberosa (ET) es una anomalía genética, multisistémica, susceptible de originar tumores del sistema nervioso central. Las crisis epilépticas son manifestaciones comunes y constituyen el principal problema terapéutico. Describir lascaracterísticas epilépticas de los pacientes pediátricos con diagnóstico de complejo de esclerosis tuberosa (CET), controlados en el Servicio de Neurología Pediátrica del Instituto Autónomo Hospital Universitario de los Andes. Se realizó un estudio observacional, retrospectivo, tipo serie de casos con ET y Epilepsia. Se describió sexo, edad de diagnóstico e inicio de crisis, motivo de consulta, tipo de crisis epiléptica, hallazgos electroencefalográficos y de imagen, asociación a trastornos conductuales, severidad de compromiso intelectual y manifestaciones dermatológicas. Doce pacientes cumplieron criterios diagnósticos de CET, 10 (83%) fueron epilépticos, deéstos el 50% cursó con epilepsia de difícil control, 60% tuvo crisis parciales, 40% generalizadas. El 100% mostró alteraciones electroencefalográficas, 30% con patrón hipsarrítmico. 50% tenían alteraciones estructurales, tipo túber cortical en 80%. En 70% secontrolaron las crisis con Acido Valpróico y en un caso se requirió dieta cetogénica estricta. El signo extraneurológico más constante fueronmáculas hipocrómicas (100%). Aunque las convulsiones no forman parte de los criterios diagnósticos, son el motivo másfrecuente de consulta, que en asociación con máculas hipocrómicas, hace sospechar diagnóstico de ET. La variedad, refractariedad e inicio temprano de crisis requieren en muchos casos politerapia para el control, lo cual favorece el pronóstico del paciente

Tuberous sclerosis (TS) is a genetic, multisystemic, likely to cause central nervous system tumors. Seizures are common manifestations are the main therapeutic problem. To describe the epileptic characteristics, of pediatric patients with diagnosed with tuberous sclerosis complex (TSC), controlled in the Pediatric Neurology Department University Hospital Institute of Los Andes. Was performed an observational, retrospective, case series, with ET and Epilepsy. Described: sex, age of the diagnosis and initiation of crisis, reason for visit, seizure type, electroencephalographic findings and images, behavioral disorders, severity ofintellectual engagement and dermatologic manifestations. Twelve patients met the criteria diagnostic CET, 10 (83%) were epileptic, of these 50% passed with epilepsy of difficult control. 60% had partial seizures (40%) generalized. The 100% showed EEGabnormalities, hypsarrhythmic pattern 30% . The 50% of cases had structural abnormalities, 80% cortical tuber type. In 70% was achieved crisis control with valproic acid and in one case was required strict ketogenic diet .The extraneurological sign more constantwere the hypochromic macules (100%). Conclusion: Although seizures are not part of the diagnostic criteria, are the most frequent reason for consultation in partnership with hypochromic macules to suspect a diagnosis of ET. The variety, refractoriness and early onset of crisis, often require polytherapy to control, which favors the patient's prognosis.

No toda convulsión es epilepsia. caso clínico de neurocisticercosis múltiple en pediatría / Not every seizure is epilepsy. clinical case of multiple neurocysticercosis in pediatrics

En el mundo se considera que hay aproxi - madamente m ás de 50 millones de infecta dos con neurocisticercosis, teniendo como zonas end émicas M éxico, Centro y Sur Am érica, África, Asia e India (1). Se considera la enfermedad infecciosa neu- rol ógica m ás importante por sus manifesta ciones cl ínicas. El agente causal en cerdos y humanos es el cisticerco de la Taenia solium que puede alojarse en diferentes partes de cuerpo humano como ser mus -culo, cerebro y ventr ículos (2). El ser humano es el único hospedero de la solitaria y el cerdo es el inte rmediario, por lo que la prevalencia de la teniasis - cisticercosis depende de este vinculo. En zonas end émicas se diagnostica por la presencia de crisis convulsivas, cefalea, sí ntomas neurol ógicos focales, trastornos de visi ón, hipertensi ón endocraneal y con tomograf ías cerebrales que reporten zonas hipodensas o quiste con un anillo hiper -denso. El tratamiento con antiparasitarios es controversial en pediatr ía, se sugiere administrarse en la fase quí stica no calcifi cada y en casos de epilepsia asociada. Pocos son los casos que ameritan tratami ento quir úrgico por hidrocefalia o por cisticerco intraventricular, que es muy raro. El presente caso tiene como finalidad conocer una forma de presentaci ón de la neurocisticercosis en pediatr ía , as í como el adverti r cl ínico que debe tener en cuenta esta entidad dentro del diagnó stico diferen cial del " S índrome Convulsivo " haciendo uso razonable de la tecnolog ía en im ágenes y del laboratorio , perimitiendo as í un diag- nóstico y tratamiento oportuno . Palabras claves: Neurocisticercosis, Taenia solium , TAC cerebral, RMN cerebral...(AU)

Crisis epilépticas en la población infantil / Seizures in children

Las crisis epilépticas son un problema neurológico que afecta entre un 4-10 por ciento de los niños hasta los 16 años. Las crisis deben ser diferenciadas de otros desórdenes neurológicos de los niños y clasificadas según su tipo. El tratamiento de emergencia está focalizado en la estabilización del paciente, terminar con la actividad convulsiva y determinar la causa de ésta. Diferentes modalidades de tratamiento existen a largo plazo, pero el adecuado tratamiento va a depender de un diagnóstico correcto y el conocimiento de los beneficios y efectos adversos de la terapia indicada.

Seizures are a neurological problem that affects between 4 to 10 per cent of children up to age 16. The crisis must be differentiated from other neurological disorders of children and classified by type. Emergency treatment is focused on stabilizing the patient, ending the seizure activity and determine the cause of this. There are different treatment modalities in the long term, but proper treatment will depend on a correct diagnosis and awareness of the benefits and adverse effects of prescribed therapy.