Ressecção cirúrgica de calcinose sintomática em paciente com esclerose sistêmica / Surgical resection of symptomatic calcinosis in a patient with systemic sclerosis

Introdução: A esclerose sistêmica é uma doença rara, autoimune, com evolução progressiva, que afeta os tecidos conectivos e órgãos internos por inflamação, podendo causar calcinose de subcutâneo. Podem evoluir para quadros dolorosos e incapacitantes, podendo tornar-se infectados, principalmente quando ulceram pela pele. Objetivo: Apresentar caso de calcinose em região inguinal e sua evolução cirúrgica. Relato de Caso: Paciente feminina portadora de calcinoses em região inguinal bilateral, apresentando algia moderada/grave com falha de tratamento clínico. Realizada ressecção cirúrgica das calcinoses, que formavam cordões de fibrose com aderência na fáscia do músculo oblíquo externo. Realizado fechamento primário com nylon 2.0 pontos simples subdérmicos e ponto intradérmico continuo nylon 3.0 para fechamento estético e menor reação inflamatória. Boa evolução pós- operatório. Conclusão: O melhor tratamento da calcinoses ainda não é claro. O tratamento das complicações se torna essencial para reduzir a morbidade e aumentar a qualidade de vida do paciente.

Introduction: Systemic sclerosis is a rare, autoimmune, progressive disease that affects connective tissues and internal organs by inflammation, which can cause calcinosis cutis. It can progress to painful and disabling conditions, and can become infected, especially when skin ulceration is present. Objective: To present a case of calcinosis in the inguinal region and its surgical recovery. Case Report: A female patient with calcinosis in the bilateral inguinal region presenting with moderate/severe pain had a failed clinical treatment. We performed surgical resection of the calcinosis cutis, which had formed clusters of fibrosis with adhesion to the fascia of the external oblique muscle. We used simple nylon 2.0 sutures along the subdermal plane to perform primary closure and continuous nylon 3.0 sutures along the intradermal plane for aesthetic closure and minimal inflammatory reaction. Her postoperative recovery was positive. Conclusion: The best treatment for calcinosis cutis is still unclear. Treating complications becomes essential for reducing patients' morbidity and increasing their quality of life.

Sclerosing mediastinitis causing unilateral pulmonary edema due to left atrial and pulmonary venous compression. a case report and literature review

Abstract Sclerosing mediastinitis (SM), previously named chronic fibrosing mediastinitis, is an inflammatory process that in its end-stage results to sclerosis around the mediastinal structures. SM is quite rare and has been correlated with inflammatory and autoimmune diseases, as well as malignancy. SM may either present in a mild form, with minor symptoms and a benign course or in a more aggressive form with severe pulmonary hypertension and subsequent higher morbidity and mortality. The diagnosis of SM may be difficult and quite challenging, as symptoms depend on the mediastinal structure that is mainly involved; quite often the superior vena cava. However, practically any mediastinal structure may be involved by the fibrotic process, such as the central airways, as well as the pulmonary arteries and veins, leading to obstruction or total occlusion. The latter may be impossible to undergo proper surgical excision of the lesion, and is considered to be a real challenge to the surgeon. We herein report a case of SM that presented with arterial and venous compression. The imaging appearance was that of unilateral pulmonary edema, associated with lung collapse. The case is supplemented by a non-systematic review of the relevant literature.

Is self-report sleepiness associated with cognitive performance in temporal lobe epilepsy? / A sonolência auto-relatada está associada ao desempenho cognitivo na epilepsia do lobo temporal?

ABSTRACT Sleepiness and cognitive impairment are common symptoms observed in patients with epilepsy. We investigate whether self-reported sleepiness is associated with cognitive performance in patients with refractory mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). Seventy-one consecutive patients with MTLE-HS were evaluated with the Stanford Sleepiness Scale (SSS) before neuropsychological evaluation. Their mean SSS scores were compared with controls. Each cognitive test was compared between patients with (SSS ≥ 3) or without sleepiness (SSS < 3). Imbalances were controlled by regression analysis. Patients reported a significantly higher degree of sleepiness than controls (p < 0.0001). After multiple linear regression analysis, only one test (RAVLT total) remained associated with self-reported sleepiness. Conclusion: Self-reported sleepiness was significantly higher in MTLE-HS patients than controls, but did not affect their cognitive performance. If confirmed in other populations, our results may have implications for decision making about sleepiness screening in neuropsychological settings.

RESUMO A sonolência e o comprometimento cognitivo são queixas comuns na epilepsia. Investigamos se a sonolência relatada pelo paciente está associada ao desempenho cognitivo na epilepsia do lobo temporal mesial refratária com esclerose do hipocampo (ELTM-EH). 71 pacientes com ELTM-EH foram avaliados pela Escala de Sonolência de Stanford (ESS) antes da avaliação neuropsicológica. A média na ESS foi comparada com a de controles. Cada teste foi comparado entre os pacientes com sonolência (ESS ≥ 3) ou sem sonolência (ESS <3). Diferenças foram controladas por regressão logística múltipla. Os pacientes relataram uma sonolência maior do que os controles (p <0,0001). Após a regressão, a sonolência relatada pelos pacientes mostrou-se associada a apenas um teste (RAVLT total). Os pacientes com ELTM-EH referem mais sonolência do que os controles, mas esta não foi associada com a cognição. Se confirmado em outras populações, nossos resultados implicarão na tomada de decisão sobre o impacto da sonolência no contexto neuropsicológico.

A regeneração do colo uterino após tratamentos excisionais das neoplasias intraepiteliais cervicais: repercussões futuras / Uterine cervix regeneration after excisional treatmentfor cervical intraepithelial neoplasia: future implications

Os tratamentos excisionais para as neoplasias intraepiteliais cervicais podem ter como consequência o trabalho de parto prematuro e a colposcopia inadequada em decorrência da estenose do canal cervical, além da possibilidade de comprometimento da resposta imune aferente contra novas lesões. Algumas escolhas feitas pelo cirurgião podem otimizar o processo cicatricial e minimizar esses efeitos. São elas: evitar o tratamento desnecessário das lesões, as cauterizações profundas e as suturas em demasia, bem como orientar a paciente sobre o período de tempo que deverá ser aguardado antes de uma possível gestação.(AU)

Excisional treatments for cervical intraepithelial neoplasia may result in preterm labor and inadequate colposcopy, as a consequence of cervical canal stenosis, in addition to the possibility of reducing the afferent immunologic response against new lesions. Some choices made by the surgeon can optimize the healing process and minimize these effects, such as: avoiding unnecessary treatment of the lesions, deep cauterizations and excessive sutures, as well as advising the patient about the proper time to wait before a possible pregnancy.(AU)

IgG4-Related Sclerosing Disease Involving the Superior Vena Cava and the Atrial Septum of the Heart

A 55-year-old woman presented with frequent episodes of syncope due to sinus pauses. During ambulatory Holter monitoring, atrial fibrillation and first-degree atrioventricular nodal block were observed. Magnetic resonance imaging and CT scans showed a tumor-like mass from the superior vena cava to the right atrial septum. Open chest cardiac biopsy was performed. The tumor was composed of proliferating IgG4-positive plasma cells and lymphocytes with surrounding sclerosis. The patient was diagnosed with IgG4-related sclerosing disease. Because of frequent sinus pauses and syncope, a permanent pacemaker was implanted. The cardiac mass was inoperable, but it did not progress during the one-year follow-up.

Hippocampal sclerosis and status epilepticus: cause or consequence? A MRI study

BACKGROUND: Transient imaging abnormalities, including changes on diffusion-weighted imaging (DWI), may be seen in status epilepticus. These abnormalities can be followed by hippocampal sclerosis. CASE REPORT: We report a 15-year-old lady with focal non convulsive status epilepticus (NCSE) and focal slowing on EEG. DWI exhibited abnormal hyperintense signals in bilateral temporal and insular cortices. After 3 weeks, MRI performed a localizated hippocampal atrophy. CONCLUSION: The MRI findings indicated vasogenic and cytotoxic edema during seizure activity and subsequent loss of brain parenchyma.

INTRODUÇÃO: Anormalidades transitórias de imagem, incluindo imagens de ressonância magnética por difusão (DWI), podem ser vistas no status epilepticus. Essas anormalidades podem ser seguidas de esclerose hipocampal. RELATO DE CASO: Nós relatamos uma jovem de 15 anos com status focal não convulsivo e lentificação focal no EEG. DWI mostrava sinal hiperintenso em regiões temporais bilaterais e córtex insular. Após 3 semanas, RM de encéfalo mostrava atrofia localizada do hipocampo. CONCLUSÃO: Os achados de RM indicam edema vasogênico e citotóxico durante as crises epilépticas com subseqüente atrofia de parênquima cerebral.

Mediastinite esclerosante no diagnóstico diferencial de tumores mediastinais / Sclerosing mediastinitis in the differential diagnosis of mediastinal tumors

A mediastinite esclerosante é uma doença rara, caracterizada por tecido fibrótico mediastinal extenso, que mimetiza uma neoplasia devido à compressão ou invasão das estruturas mediastinais. Apresentamos três casos de síndrome de veia cava superior em que se comprovou a mediastinite esclerosante. A fisiopatologia relaciona-se a adenomegalias mediastinais, proliferação de fibroblastos e deposição de colágeno. As principais causas são histoplasmose e tuberculose, doenças prevalentes em nosso meio. O diagnóstico histopatológico geralmente é difícil por métodos pouco invasivos. Necessita-se de exploração cirúrgica para o diagnóstico definitivo e resolução da obstrução das vias aerodigestiva e vascular, uma vez que não há tratamento clínico efetivo para esta afecção.

Tratamento cirúrgico da epilepsia do lobo temporal: análise de 43 casos consecutivos / Surgical treatment of temporal lobe epilepsy: a series of forty-three cases analysis

Quarenta e três pacientes com epilepsia refratária ao tratamento medicamentoso foram submetidos à cirurgia de epilepsia do lobo temporal no Instituto de Neurologia de Curitiba, entre os anos de 1998 a 2003. Trinta e nove (90,6 por cento) pacientes apresentavam esclerose mesial temporal, e quatro (9,4 por cento), tumores cerebrais. Dos trinta e sete pacientes que possuíam avaliação pós-operatória completa, 83,7 por cento apresentaram classificação I, segundo Engel (livres de crises incapacitantes). Complicações pós-operatórias ocorreram em 18,6 por cento: uma infecção da ferida operatória, um caso de hidrocefalia, um de fístula liquórica, dois casos de paralisia transitória do IV nervo craniano e um de hemiparesia transitória. Não houve nenhum óbito relacionado à cirurgia de epilepsia no presente estudo.

Febrile convulsion--an overview.

Febrile convulsion is the most frequently occurring epilepsy syndrome, experienced in infants/children between 6 months and 5 years of age associated with fever >38 degrees C. Children having first or second degree relative with history of febrile convulsion, neonatal nursery stay of more than 30 days, developmental delay or attendance at day care centre are at increased risk of developing febrile convulsion. Single febrile convulsion does not increase the risk of epilepsy and there is no causal relationship between febrile convulsion and subsequent epilepsy. It has been recognised that there is significant genetic component for susceptibility to febrile seizures. To make the diagnosis of febrile convulsion, meningitis, encephalitis, serious electrolyte imbalance and other acute neurologic illnesses are to be excluded. While managing acute attack the steps to be taken are--airway management, a semi-prone position to avoid aspiration, monitoring vital signs and other supportive care. Diazepam or lorazepam is the drug to be used. There is no reason to expect phenobarbitone administered at the time of fever to be effective in prevention of febrile convulsion. The parents should be counselled about the benign nature of the convulsion. Although the febrile convulsion a frightening event, still it is a benign condition.

Porfiria cutánea tarda esclerodermiforme: presentación de cuatro casos y revisión de la literatura / Sclerodermic porphyria cutanea tarda: report of 4 casis and a review of the literature

La porfiria cutánea tarda esclerodermiforme (PCTE) es una forma poco frecuente de la presentación de la porfiria cutánea tarda (PCT), representando el 18-33 por ciento de los casos. Se caracteriza por manifestarse con placas esclerodermiformes de aparición insidiosa y tardía en el curso de la enfermedad. El déficit de la actividad de la enzima uroporfirinógeno decarboxilasa (UPDC), lleva a la acumulación de metabolitos intermedios responsables de las manifestaciones clínicas y bioquímicas. Se presentan cuatro pacientes con PCTE estudiados en nuestro Servicio, en un período de cuatro años (1992-1996)

Unilateral mesial temporal atrophy after a systemic insult as a possible etiology of refractory temporal lobe epilepsy: case report

Mesial temporal sclerosis is the main pathological substrate present in refractory temporal lobe epilepsy and its presence is often related to the occurrence of febrile seizures in infancy. These is an on-going discussion on the nature of mesial temporal sclerosis as it related to epilepsy: cause or consequence. A previously normal child developed hyperosmolar coma after abdominal surgery at the age of 6. Three months afterwards he developed simple and complex partial seizures with an increasing frequency and refractory to multiple mono- and polytherapic drug regimens. He was evaluated for surgery at the age of 13. Ictal and interictal recordings showed left temporal lobe abnormalities. Early CT scaning suggested left temporal atrophy. MRI showed mesial temporal sclerosis. Neuropsichological testing showed verbal memory deficits and he passed a left carotid artery amytal injection. He was submitted to a cortico-amygdalo-hippocampectomy and has been seizure-free since then. The clinical data obtained from this patient suggest that at least in this case mesial temporal sclerosis would be related to the cause of epilepsy and not resultant from repeated seizure activity.

Neuralgia del trigémino / Trigeminal neuralgia

La neuralgia del trigémino es un dolor facial unilateral e intermitente, que se presenta en forma de accesos paroxísticos, parecidos a descargas eléctricas, circunscrito al territorio inervado por todo el nervio (20 por ciento) o por algunas de sus 3 ramas

Primary biliary cirrhosis in a patient with progressive systemic sclerosis, a case report

A 63-year-old woman suffering from progressive systemic sclerosis for about 20 years disclosed symptoms of liver disease within the last three years. Diagnosis of biliary cirrhosis was established on the basis of clinical picture, pathological examination of the hepatic tissue sample, immunological tests, and x-ray studies. Association of systemic sclerosis with primary biliary cirrhosis is briefly reviewed

Esclerosis endoscópica de várices esofágicas / Endoscopic sclerosis of esophageal varices

Se presenta una síntesis de las indicaciones, contraindicaciones, resultados y complicaciones, así como las distintas técnicas empleadas y una sucinta historia de la esclerosis en todo el mundo. También se consignan los resultados de los 72 pacientes tratados por nuestro grupo entre agosto de 1978 y marzo de 1983, es decir a través de 56 meses. La sobrevida global es de 75 por ciento, obteniéndose supresión de la hemorragia en el 83,8 por ciento. Es de destacar que las complicaciones fueron mínimas, y ninguna muerte fué imputable a la técnica.