Caracterización de pacientes con neuralgia del trigémino en un hospital mexicano, 2009-2019 / Characterization of patients with trigeminal neuralgia in a Mexican hospital, 2009-2019

Antecedentes: La neuralgia del trigémino (NT) es un dolor estereotipado, repetitivo y paroxístico, con prevalencia anual 4-13%. Objetivo: Describir características clínicas y terapéuticas de pacientes con diagnóstico de NT, División de Cuidados Paliativos, Clínica del dolor, Hospital "Dr. Manuel Gea González". Métodos: Estudio retrospectivo descriptivo. Revisión 91 expedientes, 1 enero 2009 a 31 diciembre 2019; se eliminaron 33 pacientes que abandonaron tratamiento, obteniendo muestra a conveniencia (61). Resultados: El sexo femenino fue más afectado 62.3% (38), comparado con el masculino 37.7% (23), relación 1.6 / 1. La edad media 60.2±16.8 años. La evolución previa a la atención tuvo una media: 17.6 meses. Las ramas del trigémino afectadas fueron: combinaciones rama izquierda 36.1% (22), rama derecha 27.9% (17), maxilar derecha 13.1% (8). Las causas secundarias fueron: 55.7% (35): postraumática 11, posherpética 7, compresión vascular 8, tumores 4, y disfunción temporomandibular 5. Las comorbilidades fueron: hipertensión arterial, diabetes mellitus tipo 2. Recibieron terapia farmacológica previa con antiinflamatorio no esteroideos 31.6% (37). Al ingreso la intensidad del dolor fue severa 96.7% (59), moderada 3.3% (2). Tratamiento intrahospitalario recibido fue carbamazepina 86.8% (53) y gabapentinoides 60.6% (37). Egresaron sin dolor 24.6% (15), con dolor leve 57.4% (35), dolor moderado 13.1% (8), y dolor severo 4.9% (3). Discusión: La NT fue más frecuente en mujeres, entre 50 y 70 años, similar a lo informado. El tiempo de inicio de síntomas y evaluación por especialista en algología fue prolongado. El tratamiento farmacológico indicado: carbamazepina, gabapentinoides, opioides y antidepresivos, apegados a Guías de Manejo Dolor Neuropático...(AU)

Discinesia paroxística primaria kinesiogénica y no kinesiogénica: Reporte de 3 casos y revisión de la literatura / Paroxysmal Kinesiogenic Dyskinesia and Paroxysmal Non-Kinesiogenic Dyskinesia: Three Case Reports and a Review

Las discinesias paroxísticas son un grupo de entidades consistentes en paroxismos de diversos movimientos anormales de corta duración asociados o no a factores precipitantes. Suele afectar a sujetos jóvenes y la prevalencia es desconocida. La fisiopatología es incierta; se han identificado ciertas mutaciones que expliquen su origen. Clínicamente se pueden manifestar como ataques paroxísticos de movimientos de tipo coreoatetósico, distónicos o balísticos de corta duración y con preservación de la conciencia. Los estudios electrofisiológicos y de imagen suelen ser normales. Este grupo de trastornos del movimiento hacen parte del diagnóstico diferencial de las crisis epilépticas. El pronóstico suele ser bueno y el tratamiento es sintomático con anticonvulsivantes. Se presentarán tres casos de dos tipos de trastornos paroxísticos del movimiento y revisión de la literatura.

Paroxysmal dyskinesias are a group of entities consisting of paroxysms of diverse abnormal movements of short duration, associated or not with precipitating factors. It usually affects young subjects and its prevalence is unknown. The pathophysiology is uncertain; some mutations have been identified that explain their origin. Clinically, they can manifest as paroxysmal attacks of choreoathetosis, dystonic or ballistic movements of short duration and with preservation of consciousness. Electrophysiological and imaging studies are usually normal. This group of movement disorders are part of the differential diagnosis of epileptic seizures. Prognosis is usually good and the treatment is symptomatic with anticonvulsants. Three cases of two types of paroxysmal movement disorders and a review of the current literature are presented.

Diagnóstico de encefalite límbica não paraneoplásica de etiologia imunomediada: estudo de caso e revisão de literatura / Diagnosis of immune-mediated non-paraneoplastic limbic encephalitis: case report and literature review

A encefalite límbica vem sendo descrita como um distúrbio neurológico raro, que afeta seletivamente as estruturas do sistema límbico. Clinicamente, é caracterizada como uma desordem neurológica debilitante, que se desenvolve como encefalopatia rapidamente progressiva, causada por inflamação encefálica. Objetivamos aqui relatar um caso de encefalite do sistema límbico de provável etiologia autoimune para melhor conhecimento da comunidade médica, bem como averiguar métodos diagnósticos deste quadro. Paciente do sexo masculino, 59 anos, admitido em nosso serviço com queixa de confusão mental. O exame clínico evidenciou desorientação, disartria, paresia e parestesia no hemicorpo esquerdo, dificuldade de marcha, desvio de rima e histórico de epilepsia há 2 anos. No estudo por ressonância magnética do crânio, foram observadas extensas lesões que acometiam a região mesial do lobo temporal direito, todo o hipocampo e giro para-hipocampal direito, estendendo-se pelo fórnix até a porção posterior do hipocampo esquerdo, substância branca do lobo frontal bilateral. Mediante os resultados da investigação complementar, o paciente foi tratado com pulsoterapia de metilpredinisolona por 5 dias, resultando na regressão parcial dos sintomas. Atualmente, o paciente se encontra em seguimento ambulatorial para acompanhamento. A encefalite límbica é uma doença rara, porém muito importante de ser investigada e diagnosticada precocemente, uma vez que a progressão da doença pode causar incapacidade e sequelas irreversíveis.

Limbic encephalitis has been described as a rare neurological disorder affecting the limbic system structures selectively. Clinically, it is characterized as a debilitating neurological syndrome that develops as a quickly progressive encephalopathy caused by brain inflammation. This paper reports a case of limbic encephalitis, probably of autoimmune etiology, aiming to improve the knowledge of the medical community, and to promote a debate on diagnosis methods for this pathology. The patient is male, 59 years old, and was admitted at our service complaining of mental confusion. The clinical examination showed disorientation, dysarthria, left hemiparesis and paresthesia, gait difficulties, light asymmetrical smile, and history of epilepsy 2 years ago. The magnetic resonance imaging of skull showed extensive lesions affecting the mesial region of the right temporal lobe, the entire hippocampus, and right parahippocampal gyrus, extending through the fornix to the posterior portion of the left hippocampus, white matter of bilateral frontal lobe. Based on the complementary investigation results, the patient was treated with intravenous methylprednisolone for five days. Currently, he is being followed in the outpatient's department. Although being rare, limbic encephalitis shall be investigated and diagnosed early because its progression can lead to disability and irreversible sequelae

Epilepsia en niños con hemiparesia congénita secundaria a infartos cerebrales vasculares perinatales / Epilepsy in children with congenital hemiparesis secondary to perinatal ictus

El objetivo fue describir la frecuencia, modo de presentación y características de la epilepsia en niños con hemiparesia congénita (HC). Estudio retrospectivo, descriptivo y multicéntrico, basado en la recolección de datos de las historias clínicas de pacientes de 0 a 19 años con HC secundaria a infarto perinatal en diferentes centros de la comunidad de Cataluña. Se incluyeron 310 niños (55% varones y 45% mujeres) de un total de 13 centros de Cataluña. Edad media del debut de las crisis fue de 2 ± 1 año. Presentaron epilepsia el 29.5% (n = 76), el subtipo vascular más frecuente fue el infarto presumiblemente perinatal (51.3%), seguido del accidente isquémico arterial neonatal (18.4%), infarto hemorrágico venoso periventricular (15.8%), infarto hemorrágico neonatal (10.5%) y trombosis venosa neonatal (3.9%). La semiología de las crisis más frecuente fue la focal motora en un 82%, seguida de las focales motoras con bilateralización secundaria en el 23%, focales discognitivas en 13.5%, generalizadas 2% y espasmos 6.5%. El 67.3% se controló con monoterapia y los fármacos empleados fueron el valproato, levetiracetam o carbamacepina. Se identificó el antecedente de estatus eléctrico durante el sueño en 3 pacientes, todos asociados a lesiones extensas que incluían al tálamo. Del total con epilepsia, el 35% debutaron con convulsiones neonatales en los primeros 3 días de vida. El 30% con accidente cerebrovascular perinatal y HC presentan riesgo de padecer epilepsia durante la infancia. Aquellos con infartos isquémicos tienen el riesgo más alto, por lo que requerirán un seguimiento dirigido a detectar precozmente la epilepsia e iniciar tratamiento.

The objective was to describe the frequency, mode of presentation and characteristics of epilepsy in children with congenital hemiparesis (CH). It is a etrospective, descriptive and multicenter study, based on the collection of data from the clinical records of patients from 0 to 19 years with CH secondary to perinatal infarction in different centers of the community of Catalonia. A total of 310 children were included (55% males and 45% females), from a total of 13 centers in Catalonia. Average age of onset of the crises was 2 ± 1 year. Epilepsy was present in 29.5% (n = 76), among which the most frequent vascular subtype was arterial presumed perinatal ischemic stroke (51.3%), followed by neonatal arterial ischemic stroke (18.4%), periventricular venous infarction (15.8%), neonatal hemorrhagic stroke (10.5%) and neonatal cerebral sinovenous thrombosis (3.9%). Semiology of the most frequent seizures was motor focal in 82%, followed by focal motor with secondary bilateralization in 23%, focal discognitive in 13.5%, generalized by 2% and spasms in 6.5%. The 67.3% were controlled with monotherapy and the drugs used were valproate, levetiracetam or carbamazepine. The antecedent of electrical status during sleep was identified in 3 patients, all associated with extensive lesions that included the thalamus. Of the total number of children with epilepsy, 35% began with neonatal seizu res in the first 3 days of life. The 30% of children with perinatal stroke and CH present a risk of epilepsy during childhood. Children with ischemic strock have the highest risk, so they will require a follow-up aimed at detecting prematurely the epilepsy and start a treatment.

Compromiso neuropático y autonómico en Enfermedad de Fabry: presentación de casos clínicos / Neuropathy and Fabry's disease: report of five cases

Fabry's disease is an X-linked multisistemic lisosomal storage disorder caused by deficiency or absence in α-Galatosidase A. Symptoms develop early in childhood with small fiber neuropathy, autonomic disorders and skin lesions (angiokeratomas). More severe in males, patients develop over years heart disease (hypertrophic cardiomyopathy, bradycardia), proteinuria, renal failure, transient ischemic attacks and stroke, associated with decreased life expectancy. We report five patients with Fabry's disease aged between 21 to 56 years and with family history. Neuropathic symptoms are described and neurophysiological testing findings of nerve conduction studies, quantitative sensory testing, autonomic testing and sympathetic skin response are presented.

Caso clínico: hipofosfatasia de la niñez. Seguimiento clínico / Clinical case: childhood hypophosphatasia. Clinical follow-up

La hipofosfatasia (HP) es una enfermedad congénita, causada por mutaciones con pérdida de función en el gen ALPL que codifica la isoenzima no específica de tejido de la fosfatasa alcalina (TNSALP). Su expresión clínica es muy variable, desde casos de muerte intraútero por alteración grave de la mineralización ósea, hasta casos solo con caída prematura de la dentición. Se presenta el caso clínico de un varón al que se le diagnosticó odontohipofosfatasia a los 30 meses por pérdida temprana de piezas dentarias y niveles anormalmente bajos de fosfatasa alcalina, sin signos de raquitismo ni deformidades óseas. Durante su seguimiento, hasta los 13 años, presentó síntomas compatibles con HP infantil leve, como cansancio al caminar, incoordinación en la marcha y dolor en miembros inferiores que aumentaban con la actividad física. Ante la aparición de edema bimaleolar y poca respuesta al tratamiento con calcitonina y antiinflamatorios, se descartaron patologías infecciosas o reumáticas o ambas y se diagnosticó, por biopsia de tibia y peroné, periostitis sin detección de cristales de pirofosfato. Los controles radiológicos durante su evolución mostraron ensanchamiento metafisario en muñeca, falta de remodelado de metacarpianos, hojaldrado perióstico en tibia y peroné e hipomineralización en metáfisis tibiales, con "lenguas radiolúcidas" características de HP. Como conclusión, la hipofosfatasia debe considerarse como una entidad clínica para descartar en niños que presentan pérdida temprana de dientes. La presencia de este cuadro clínico es en general suficiente para realizar el diagnóstico de HP de la niñez. (AU)

Hypophosphatasia (HP) is a congenital disease, caused by mutations with loss of function in the gene ALPL that encodes the non-specific tissue isoenzyme of alkaline phosphatase (TNSALP). Its clinical expression displays considerable variability, from cases of intrauterine death due to severe alteration of bone mineralization, to cases with only early loss of teeth. We report the case of a male, diagnosed as odontohypophosphatasia at 30 months of age due to early loss of teeth and abnormally low levels of alkaline phosphatase, without signs of rickets or bone deformities. During follow-up, up to 13 years of age, he presented symptoms consistent with mild infantile HP such as tiredness when walking, lack of gait coordination, and pain in lower limbs, especially after physical activity. Due to the appearance of bimalleolar edema and poor response to treatment with calcitonin and anti-inflammatory drugs, infectious and / or rheumatic pathologies were ruled out. Periostitis without pyrophosphate crystal detection was diagnosed by tibial and fibular biopsy. Radiological controls during follow up showed metaphyseal wrist enlargement, lack of remodeling of metacarpals, periosteal flaking in the tibia and fibula and hypomineralization in the tibial metaphysis, with "radiolucent tongues"; characteristic of HP. In conclusion, hypophosphatasia should be considered as a clinical entity in children who present early loss of teeth. The presentation of this clinical case is generally sufficient to make the diagnosis of childhood HP. (AU)

Paroxismia vestibular: reporte de un caso / Vestibular paroxysmia: case report

Presentamos el caso de un paciente joven quien presenta 4 a 5 crisis diarias de vértigo espontáneo de segundos de duración, todos o casi todos los días desde hace 9 meses. Estas crisis no tienen gatillo posicional, y hay completa ausencia de sintomatologia entre crisis. Como discutimos en el artículo, este cuadro coíncide con los recientemente publicados criterios para una paroxismia vestibular, entidad supuestamente secundaria a la compresión neurovascular del nervio vestibular. El paciente respondió de forma inmediata y completa a carbamazepina a dosis bajas, el tratamiento de elección en la paroxismia vestibular.

We present the case of a young patient, with a 9-month long history of 4 to 5 daily spells of spontaneous vertigo, each lasting only seconds. There is no positional trigger, and there is a complete lack of symptoms between attacks. As is discussed in the article, this matches the recently published criteria for Vestibular Paroxysmia, an entity allegedly secondary to neurovascular compression of the vestibular nerve. The patient responded immediately and completely to carbamazepine at low dosage, the preferred treatment for vestibular paroxysmia.

Levetiracetam em monoterapia para epilepsia focal em pacientes com falha no tratamento com carbamazepina / Levetiracetam monotherapy for focal epilepsy in patients failing treatment with carbamazepine

Contexto: A epilepsia é uma doença cerebral crônica causada por diversas etiologias e caracterizada pela recorrência de crises epilépticas não provocadas. O tratamento disponível no Sistema Único de Saúde (SUS) atualmente inclui as drogas antiepiléticas fenobarbital, fenitoína, primidona, topiramato, lamotrigina, carbamazepina e valproato de sódio, indicadas no Protocolo Clínico do Ministério da Saúde (MS). Pergunta: O uso do levetiracetam em monoterapia é tão eficaz e seguro quanto as demais drogas antiepilépticas (lamotrigina) e topiramato) disponíveis no SUS, por meio do Componente Especializado da Assistência Farmacêutica - CEAF, para o tratamento de pacientes com epilepsia focal após a falha no tratamento com carbamazepina? Evidências científicas: Não há evidências clínicas para o uso do levetiracetam em monoterapia em crises epiléticas focais para a pergunta de pesquisa estabelecida na presente solicitação de avaliação. Avaliação econômica: Foi apresentada uma análise de custo-minimização seguindo a premissa de que o levetiracetam não possui superioridade clínica sobre os medicamentos oferecidos pelo SUS. Porém o custo de tratamento do levetiracetam é maior do que os tratamentos disponíveis no sistema público. Deliberação final: A análise do conteúdo de todas a contribuições da consulta pública não trouxe elementos que pudessem alterar a recomendação de não incorporação. Assim, os membros da CONITEC recomendaram por unanimidade a não incorporação no SUS do levetiracetam em monoterapia para epilepsia focal em pacientes com falha no tratamento com carbamazepina. Decisão: Não incorporar o levetiracetam em monoterapia para epilepsia focal em pacientes com falha no tratamento com carbamazepina, no âmbito do Sistema Único de Saúde - SUS. A decisão foi dada pela Portaria SCTIE-MS nº 9 publicada no Diário Oficial da União (DOU) nº 38, de 22 de fevereiro de 2017.

Comparative role of pregabalin and carbamazepine regarding efficacy in painful diabetic neuropathy

Neuropathic pain is the most severe and resistant type of pain which has impact on quality of life ana behaviour; it most commonly occurs at night causing disturbed; sleep. Diabetes mellitus is a common cause of painful neuropathy. In this study, we are comparing the effectiveness of old treatment Carbamazepine with Pregabalin in painful diabetic neuropathy. The study was an open-label trial conducted in Diabetic Clinic of Medical Unit-Ill, Jinnah Postgraduate Medical Center, Karachi. The duration of the study was 90 days, from December 2010 to March 2011. The study has been approved from ethical committee of JPMC, Karachi with the reference NO.F.2-81/2010-GENL/195/JPMC. 60 established patients of painful diabetic peripheral neuropathy from Diabetic Clinic of Medical Unit-Ill OPD were included in the 90-day study, irrespective of gender, with duration of diabetes more than 10 years. All subjects are placed into two groups. In group A, comprising of 30 patients [n=30], Pregabalin was administered and in group B, also comprising of 30 patients [n=30], Carbamazepine. The intensity of pain was compared on visual analog scale of McGill pain questionnaire. In group A [Pregabalin], the mean pain score fell from 6.17+/-0.14 to 3.50+/-0.15 from day 0 to day 90 [p-value=0.001] and the percentage of change also in visual analog scale of McGill pain questionnaire was -43.31%. In group B [Carbamazepine], the changes in pain score from initially 6.07+/-0.14 falling to 4.23+/-0.13 from day 0 to day 90 [p-value=0.001] and the percentage of change was -30.31%. Pregabalin was observed to be more potent. Both drugs were well tolerated by all participants that also completed the entire duration of the trial

Correlation between hyperhomocysteinemia and common carotid artery intima media thickness in carbamazepine treated epileptic patients using ultrasonography

Objective: The objective was to assess the role of homocysteine in the development of atherosclerosis in common carotid artery in the carbamazepine treated epileptic patients

Methods: This study was conducted in the Department of Biochemistry, Basic Medical Sciences Institute [BMSI], Jinnah Postgraduate Medical Center [JPMC], Karachi. Three hundred individuals, aged 34 +/- 9.5 years were selected and divided into three groups. Each group comprised of 100 subjects labeled as Group-A [control group had healthy individuals], Group-B [newly diagnosed epileptic patients without antiepileptic therapy], Group-C [epileptic patients on Carbamazepine therapy, which was further subdivided into C-l having epileptic patients on Carbamazepine therapy less than 1 year n=33, C-ll had epileptic patients on Carbamazepine therapy 1-2 years n = 33 and C-lll comprised of epileptic patients on Carbamazepine therapy more than 2 years n = 34]. Blood concentration of homocysteine was measured and ultrasound of Common Carotid Artery for intima-media thickness was performed

Results: Significantly elevated level of homocysteine was observed in epileptic patients on CBZ therapy. Common Carotid Artery Intima-media thickness [CCAIMT] was observed significantly high throughout group Cbut it was more profound in Group-C-lll. Homocysteine was found positively correlated with right CCA IMT, left CCA IMT and mean CCA IMT

Conclusion: Hyperhomocysteinemia was linked with increased risk of atherosclerosis in CBZ treated epileptic patients

Carbamazepine for prevention of chemotherapy-induced nausea and vomiting: a pilot study / Carbamazepina para prevenção de náusea e vômito induzidos por quimioterapia: um estudo piloto

CONTEXT AND OBJECTIVE: Nausea and vomiting are major inconveniences for patients undergoing chemotherapy. Despite standard preventive treatment, chemotherapy-induced nausea and vomiting (CINV) still occurs in approximately 50% of these patients. In an attempt to optimize this treatment, we evaluated the possible effects of carbamazepine for prevention of CINV. DESIGN AND LOCATION: Prospective nonrandomized open-label phase II study carried out at a Brazilian public oncology service. METHODS: Patients allocated for their first cycle of highly emetogenic chemotherapy were continuously recruited. In addition to standard antiemetic protocol that was made available, they received carbamazepine orally, with staggered doses, from the third day before until the fifth day after chemotherapy. Considering the sparseness of evidence about the efficacy of anticonvulsants for CINV prevention, we used Simon's two-stage design, in which 43 patients should be included unless overall complete prevention was not achieved in 9 out of the first 15 entries. The Functional Living Index-Emesis questionnaire was used to measure the impact on quality of life. RESULTS: None of the ten patients (0%) presented overall complete prevention. In three cases, carbamazepine therapy was withdrawn because of somnolence and vomiting before chemotherapy. Seven were able to take the medication for the entire period and none were responsive, so the study was closed. There was no impact on the patients' quality of life. CONCLUSION: Carbamazepine was not effective for prevention of CINV and also had a deleterious side-effect profile in this population. .

CONTEXTO E OBJETIVO: Náusea e vômito são inconvenientes importantes para pacientes submetidos a quimioterapia. A despeito do tratamento preventivo padrão, náuseas e vômitos induzidos por quimioterapia (NVIQ) ocorrem em aproximadamente 50% dos pacientes. Na tentativa de otimizar este tratamento, avaliamos os possíveis efeitos da carbamazepina na prevenção de náuseas e vômitos induzidos por quimioterapia. TIPO DE ESTUDO E LOCAL: Estudo fase II, prospectivo, não randomizado, aberto, realizado em um serviço público brasileiro de oncologia. MÉTODOS: Recrutaram-se continuamente pacientes alocados para o primeiro ciclo de quimioterapia altamente emetogênica. Além do protocolo anti-emético padrão disponibilizado, os pacientes receberam carbamazepina, por via oral, em doses escalonadas, a partir do terceiro dia anterior até o quinto dia após a quimioterapia. Dada a escassa evidência de eficácia dos anticonvulsivantes na prevenção de NVIQ, adotamos o desenho de Simon em duas fases, que deveria incluir 43 pacientes a não ser que prevenção completa global não fosse alcançada em 9 dos primeiros 15 participantes. O questionário "Functional Living Index-Emesis" foi usado para avaliar o impacto na qualidade da vida. RESULTADOS: Nenhum dos 10 pacientes (0%) apresentou prevenção completa global. Três tiveram a carbamazepina suspensa por sonolência e vômito antes da quimioterapia. Sete foram capazes de tomar a medicação por todo o período proposto e nenhum obteve resposta, sendo então interrompido o estudo. Não houve impacto na qualidade da vida. CONCLUSÃO: Carbamazepina não foi efetiva para prevenção de NVIQ e apresentou perfil deletério de efeitos adversos nesta população. .

Tonic spasms are a common clinical manifestation in patients with neuromyelitis optica / Espasmos tônicos são manifestações clínicas frequentes em pacientes com neuromielite óptica

Tonic spasms have been most commonly associated with multiple sclerosis. To date, few reports of series of patients with neuromyelitis optica and tonic spasms have been published. Methods: We analyzed the characteristics and frequency of tonic spasms in 19 subjects with neuromyelitis optica. Data was collected using a semi-structured questionnaire for tonic spasms, by both retrospectively reviewing medical records and performing clinical assessment. Results: All patients except one developed this symptom. The main triggering factors were sudden movements and emotional factors. Spasms were commonly associated to sensory disturbances and worsened during the acute phases of the disease. Carbamazepine was most commonly used to treat the symptom and patients showed good response to the drug. Conclusions: Tonic spasms are a common clinical manifestation in patients with neuromyelitis optica. .

Espasmos tônicos têm sido mais frequentemente associados com esclerose múltipla. Foram publicados até agora poucos relatos de série de pacientes com neuromielite óptica e espasmos tônicos. Métodos: Foram analisadas as características e a frequência de espasmos tônicos em 19 indivíduos com neuromielite óptica. Os dados foram coletados por meio de um questionário semiestruturado para espasmos tônicos, mediante a avaliação retrospectiva dos prontuários e a análise dos dados clínicos Resultados: Todos os pacientes com neuromielite óptica exceto um apresentaram espasmos tônicos. Os principais fatores desencadeantes foram movimentos bruscos e fatores emocionais. Espasmos foram frequentemente associados a perturbações sensoriais e se agravaram durante a fase aguda da doença. A carbamazepina foi utilizada frequentemente para tratar os sintomas, com boa resposta. Conclusões: Os espasmos tônicos são manifestações clínicas frequentes em pacientes com neuromielite óptica. .

Paroxysmal dystonia and neuromyelitis optica / Distonia paroxística e neuromielite óptica

Paroxysmal dyskinesias (PD) are thought to be rare movement disorders. The overwhelming majority of reported cases are primary. Secondary PD has seen reported to occur in some conditions, mainly in multiple sclerosis and head trauma. The anatomic origin of the lesion is also rarely seen at the spinal cord. Our objective was to describe four patients with paroxysmal dystonia secondary to spinal lesions during the recovering phase of a neuromyelitis optica (NMO) bout. In the reviewed literature, we do not find any report of PD related to NMO.

Discinesias paroxísticas (DP) são distúrbios do movimento raros. A maioria dos casos relatados é de origem primária. DP secundárias têm sido relatadas em algumas condições, principalmente na esclerose múltipla e no trauma craniano. A origem anatômica da lesão também é raramente observada na medula. O objetivo deste trabalho foi descrever quatro pacientes com distonia paroxística secundária a lesões medulares, ocorrida durante a fase de recuperação do surto de neuromielite óptica (NMO). Na literatura consultada, não encontramos qualquer relato de DP secundárias à NMO.

Síndrome DRESS por ingestión de carbamazepina: reporte de un caso pediátrico / The DRESS syndrome by carbamazepine ingestion: report of a pediatric case

Se presentó el caso de una niña de 10 años de edad con antecedentes de ingestión de carbamazepina por diagnóstico presuntivo de epilepsia, quien ingresó en el Hospital Provincial Pediátrico Docente Eliseo Noel Caamaño, en Matanzas, en mayo del 2010, por cuadro de fiebre, rash cutáneo y vómitos al inicio, con evolución severa y la aparición de ictericia, hepato-esplenomegalia. Se realizó diagnóstico de síndrome DRESS, con evolución satisfactoria sin el uso de la terapia esteroidea. Se revisó la literatura sobre síndrome de hipersensibilidad por anticonvulsivantes, medicamentos de uso cada vez más frecuente.

We presented the case of a 10-years-old girl with antecedents of carbamazepine ingestion for epilepsy presumptive diagnosis, who entered the Teaching Pediatric Provincial Hospital Eliseo Noel Caamaño, in Matanzas, in May 2010, with fever, skin rash and vomiting at the beginning, followed by severe evolution, with jaundice and hepato-splenomegaly. We diagnosed the DRESS syndrome, with satisfactory evolution without using the steroidal therapy. We reviewed the literature about the hypersensibility syndrome for anticonvulsants, drugs of more and more frequent use.

Nevralgia do trigêmeo associada à hanseníase / Hanseniase associated Trigeminal Nevralgia

A hanseníase é uma doença infecto-contagiosa, que além de alterações dermatológicas, possui manifestações neurológicas. Relatamos o caso de um homem de 66 anos, que apresentou nevralgia do trigêmeo após ter apresentado quadro de hanseníase.

Hansen's disease is an infectious disease that presents with skin changes, as well as neurological features. We report a case of a 66 years man, who presented with trigeminal neuralgia after developing Hansen's disease.

Case report of a thermal burns patient with diabetes insipidus

We report a rare case of diabetes insipidus following fire burn injury. Meticulous fluid balance and the use of carbamazepine resulted in her survival.

Carbamazepine does not alter the intrinsic cardiac function in rats with epilepsy / Carbamazepina não altera o funcionamento cardíaco intrínseco em ratos com epilepsia

Among the causes for sudden unexpected death (SUDEP) in epilepsy, the effects of antiepileptic drugs on the heart have been poorly explored. Based on this, the aim of our study was to evaluate the heart rate (in vivo and isolated ex vivo) and ventricular pressure (isolated ex vivo) of rats with and without epilepsy treated with carbamazepine. Four groups of adult, male Wistar rats (200-250 g) were studied: [A] control rats (n=8), received neither pilocarpine nor carbamazepine [B] carbamazepine-treated rats (n=8), received a daily dose of 120 mg/Kg, i.p. of carbamazepine for two weeks; [C] rats with epilepsy that received just saline solution (n=8); [D] rats with epilepsy that received a daily dose of 120 mg/Kg, i.p. of carbamazepine for two weeks (n=8). Our results showed significant increase in heart rate in animals with epilepsy (with and without the use of carbamazepine) when compared to the control groups in vivo. In contrast, we did not find differences during isolated ex vivo experiments comparing animals with and without epilepsy and despite the use of carbamazepine. Our results suggest that, in isolation, carbamazepine may not be a potential risk factor for sudden unexpected death in epilepsy.

Entre as causas de morte súbita em epilepsia (SUDEPE), os efeitos das drogas antiepilépticas no coração têm sido pobremente explorados. Desta forma, o objetivo deste estudo foi avaliar a frequência cardíaca (in vivo e de forma isolada ex vivo) e a pressão ventricular (de forma isolada ex vivo) de ratos com e sem epilepsia tratados com carbamazepina. Quatro grupos de ratos Wistar machos adultos (peso 200 a 250 g) foram estudados: [A] ratos controle (n=8), não receberam pilocarpina ou carbamazepina; [B] ratos tratados com carbamazepina (n=8), receberam dose diária de carbamazepina de 120 mg/kg intraperitoneal, durante duas semanas (n=8); [C] ratos com epilepsia que receberam solução salina; [D] ratos com epilepsia que receberam dose diária de carbamazepina de 120 mg/kg intraperitoneal durante duas semanas. Nossos resultados evidenciaram uma diferença estatisticamente significativa na média da freqüência cardíaca in vivo entre os animais com epilepsia (com e sem o uso de carbamazepina) quando comparados aos grupos controles in vivo. Em contraste, não observamos diferenças estatísticas nos experimentos ex vivo quando comparados os animais com ou sem epilepsia, a despeito do uso da carbamazepina. Nossos resultados sugerem que, de forma isolada, a carbamazepina pode não ser um fator de risco potencial para a ocorrência de morte súbita em epilepsia.

Modification in body weight associated with antiepileptic drugs / Alteração de peso corpóreo associado às drogas antiepilépticas

Antiepileptic drugs (AED) may cause body weight changes. OBJECTIVE: To evaluate the dietary habits and body weight associated with AED in epileptic patients. METHOD: Sixty-six patients were subjected to two interviews, and had their weight and body mass index calculated and compared at both times, interval between six to eight months. RESULTS: It was observed that 59.1 percent showed weight gain. The patients who had no weight gain had a greater proportion of individuals who engaged in some form of physical activity. However, of the 45 patients who maintained their initial dietary and medication pattern, 75.6 percent recorded a weight gain. Weight gain was seen in 66.7 percent of patients on carbamazepine (n=18), 60 percent on valproate (n=5), 50 percent on carbamazepine+clobazam treatment (n=14), and 58.3 percent of patients on other(s) polytherapy (n=12). CONCLUSION: The patient should be alerted to possible weight gain, and should be advised about dieting and participating in regular physical activity.

Drogas antiepilépticas (DAE) podem causar alteração do peso corpóreo. OBJETIVO: Avaliar o hábito alimentar e do peso corpóreo associado às DAE em pacientes epilépticos. MÉTODO: Sessenta e seis pacientes foram submetidos a duas entrevistas, e tiveram peso e índice de massa corpórea (IMC) calculados e comparados nos dois momentos, com intervalo de 6 a 8 meses. RESULTADOS: Apresentaram aumento de peso 59,1 por cento dos pacientes. Porém, os pacientes que não tiveram ganho de peso apresentaram maior proporção de indivíduos desenvolvendo alguma atividade física. Enquanto que dentre os 45 que mantiveram o padrão alimentar e medicação inicial 75,6 por cento registraram ganho de peso. Observou-se ganho de peso em 66,7 por cento dos pacientes com carbamazepina (n=18); 60 por cento com valproato (n=5); 50 por cento com carbamazepina e clobazam (n=14); 58,3 por cento dos pacientes com politerapia (n=12). CONCLUSÃO: Deve-se alertar o paciente sobre o ganho de peso, orientar quanto à dieta alimentar e, principalmente, incentivar atividade física regular.