Abstract:
INTRODUCTION:Progressive multifocal leukoencephalopathy is a demyelinating disease of the central nervous system caused by John Cunningham virus, mostly associated with immunodeficiency conditions, such as the human immunodeficiency virus infection. Progressive multifocal leukoencephalopathy can have multiple clinical features and usually presents a typical lesion pattern on brain magnetic resonance imaging. Its course may be rapidly progressive, although immunological responsiveness can be associated with an improved prognosis. MATERIAL AND METHODS:We performed a retrospective analysis of the clinical and radiological data from patients admitted in our institution between January 2005 and April 2014 with the diagnosis of definitive progressive multifocal leukoencephalopathy (ICD10:A81.2) in the setting of human immunodeficiency virus infection. RESULTS:Twenty-one patients were included in our study, mostly men (n = 20, 95.2%). Mean age at diagnosis was 39 years. Motor deficits were the most common clinical finding. John Cunningham virus-DNA was detected in the cerebral spinal fluid in 20 patients (95.2%). Brain imaging studies most commonly disclosed bilateral supratentorial, asymmetric lesions. Four (19%) patients developed immune reconstitution inflammatory syndrome in the follow-up. Therapeutic approach included initiation and continuation/optimization of antiretroviral therapy, with adjunctive therapy with corticosteroids in four patients. Seventeen (81%) patients died during the study period; median survival time following progressive multifocal leukoencephalopathy diagnosis was 3 months (range 1 - 13). DISCUSSION:The results of our study are in accordance with the data previously published on progressive multifocal leukoencephalopathy in human immunodeficiency virus patients. Progressive multifocal leukoencephalopathy is predominantly associated with severe immunosuppression, particularly in patients who are not under anti-retroviral therapy, and usually presents with motor and cognitive symptoms and signs. A typical bilateral asymmetric pattern in conventional magnetic resonance imaging is present in the majority of the patients. There is no specific therapy for progressive multifocal leukoencephalopathy and it is usually fatal, although outcomes can improve with highly active anti-retroviral therapy. Immune reconstitution inflammatory syndrome is also an important complication related with progressive multifocal leukoencephalopathy, usually associated with anti-retroviral therapy. Progressive multifocal leukoencephalopathy-immune reconstitution inflammatory syndrome presents with different imaging characteristics from progressive multifocal leukoencephalopathy and treatment with steroids can improve survival. CONCLUSION:The mortality rate and long-term neurological morbidity associated with progressive multifocal leukoencephalopathy are quite high. These data should increase clinician awareness to the occurrence of progressive multifocal leukoencephalopathy among human immunodeficiency virus patients and highlight the important role of magnetic resonance imaging, as early diagnosis may beassociated with better outcome. :Introdução: A leucoencefalopatia multifocal progressiva é uma patologia desmielinizante causada pelo vírus John Cunningham, geralmente associada a estados de imunodepressão, em particular a infeção pelo vírus da imunodeficiência humana. Pode apresentar múltiplas manifestações clínicas e tem habitualmente um padrão imagiológico típico. A evolução clínica é geralmente progressiva, podendo ocorrer uma melhoria do prognóstico associada à recuperação imunológica.Material e Métodos: Foi conduzida uma análise retrospetiva dos dados clínicos e imagiológicos de doentes admitidos no nosso Hospital entre janeiro de 2005 e abril de 2014 com o diagnóstico de leucoencefalopatia multifocal progressiva (ICD10:A81.2) associado a infeção por vírus da imunodeficiência humana.Resultados: Vinte e um doentes foram incluídos, sendo 20 do sexo masculino (95,2%). A idade média na altura do diagnóstico foi 39 anos. A forma de apresentação mais frequente foi défice motor. O vírus John Cunningham foi identificado no líquido cefalorraquidiano em 20 doentes (95,2%). Nos estudos de imagem verificou-se um predomínio de lesões supratentoriais, assimétricas e bilaterais. Quatro doentes (19%) desenvolveram síndrome inflamatória de resposta imunológica. A abordagem terapêutica incluiu início ou otimização de terapêutica anti-retrovirica, associada a corticoterapia em quatro casos. Dezassete (81%) doentes morreram no período do estudo, sendo a sobrevida mediana após diagnóstico de três meses (intervalo 1 a13).Discussão: Os resultados do nosso estudo são concordantes com os dados previamente publicados relativamente à leucoencefalopatia multifocal progressiva, evidenciando a sua associação à infecção pelo vírus da imunodeficiência humana, particularmente nos doentes com imunossupressão grave, o predomínio de sinais e sintomas motores e cognitivos, e a existência de um atingimento bilateral e assimétrico evidente nas sequências convencionais dos estudos de ressonância magnética. Não existe terapêutica específica e esta patologia é normalmente fatal, apesar de se verificar um aumento da sobrevida associado à terapêutica anti-retrovírica. A síndrome inflamatória de reconstituição imunológica é uma complicação associada a leucoencefalopatia multifocal progressiva, habitualmente ocorrendo após início de terapêutica anti-retrovírica, apresentando caraterísticas imagiológicas diferentes da leucoencefalopatia multifocal progressiva. A instituição de corticoterapia pode melhorar o prognóstico dos doentes que desenvolvem síndrome inflamatória de reconstituição imunológica associada a leucoencefalopatia multifocal progressiva.Conclusão: A mortalidade e morbilidade associadas à leucoencefalopatia multifocal progressiva são elevadas, com sequelas neurológicas importantes a longo-prazo. Os dados apresentados deverão alertar os clínicos para a ocorrência desta patologia nos doentes com infecção vírus da imunodeficiência humana, evidenciando o papel cada vez mais determinante da Ressonância Magnética no diagnóstico precoce da leucoencefalopatia multifocal progressiva e consequente melhoria do prognóstico.
journal_name
Acta Med Portjournal_title
Acta medica portuguesaauthors
Augusto L,Neves N,Reis C,Abreu C,Sarmento Adoi
10.20344/amp.5950subject
Has Abstractpub_date
2015-05-01 00:00:00pages
286-96issue
3eissn
0870-399Xissn
1646-0758journal_volume
28pub_type
杂志文章,评审abstract:PURPOSE:To evaluate importance of homocysteinemia as risk factor of thrombotic cerebrovascular disease, in terms of age and homocysteinemia levels. METHODS:A group of patients under 55 years old (n = 35, 21 males) that had suffered a stroke 3 months to 1 year before the study, defined by clinical criteria and the pres...
journal_title:Acta medica portuguesa
pub_type: 临床试验,杂志文章,评审
doi:
更新日期:1996-01-01 00:00:00
abstract:INTRODUCTION:Visceral leishmaniasis is an endemic disseminated infection, considered to be the third most frequent opportunistic parasitic infection in Europe. It is especially prevalent in patients co-infected with human immunodeficiency virus, in whom it poses a great therapeutic challenge due to increased risk of re...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:10.20344/amp.8291
更新日期:2017-06-30 00:00:00
abstract::The occurrence of metastases is documented in a varied number of hepato-cellular carcinomas (HCC). The indicative metastasis is very rarely the form of clinical presentation of the disease. Two cases of HCC which were clinically presented in a secondary location, in the bone and skeletal muscle respectively, are descr...
journal_title:Acta medica portuguesa
pub_type: 杂志文章,评审
doi:
更新日期:1995-04-01 00:00:00
abstract::We reviewed all the cases of Squamous Cell Carcinoma in people under 40 years of age, diagnosed histologically at the Dermatopathology Laboratory of the Santa Maria Hospital, from January 1985 to December 1993. The 19 cases detected were analysed for age, sex, occupation, anatomical site of the tumor, evolution, smoke...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:1997-11-01 00:00:00
abstract::In Portugal, piroxicam is one of the most prescribed non-steroidal anti-inflammatory agents (NSAIDs) and is frequently associated to photosensitivity skin reactions, well known by all dermatologists. The authors review the physiopathology and clinical patterns of this adverse drug reaction. Importance is given to the ...
journal_title:Acta medica portuguesa
pub_type: 杂志文章,评审
doi:
更新日期:1998-11-01 00:00:00
abstract:INTRODUCTION:The growing concern surrounding health safety issues makes it essential that everyone, in particular the elderly due to their commonly prescribed multiple drugs, has a complete and up to date list of prescriptions. We planned to assess the quality of the electronic records of prolonged medication. MATERIA...
journal_title:Acta medica portuguesa
pub_type: 杂志文章,多中心研究
doi:
更新日期:2014-09-01 00:00:00
abstract::Free radicals are highly reactive molecules, and therefore transient, which have an odd number of electrons and are generated in vivo as byproducts of normal metabolism. In this review we survey basic concepts on the chemistry of oxygen free radicals, their cellular sources and the reactions they can undergo. We also ...
journal_title:Acta medica portuguesa
pub_type: 杂志文章,评审
doi:
更新日期:1989-05-01 00:00:00
abstract::Fever of unknown origin is a diagnostic challenge. When the diagnosis remains elusive despite extensive non-invasive diagnostic studies, it can be necessary to conduct more invasive procedures, such as liver biopsy. We report a male with fever of unknown origin who had tender enlarged liver and raised liver enzymes wh...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2010-07-01 00:00:00
abstract::Many studies have shown that social support is related with psychological disorders and both are high consumers of health service resources. A study was carried out by the author to identify psychological disorder relationships with social support and how they change in presence or absence of life events. An inquiry b...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:1997-12-01 00:00:00
abstract::The interaction between HPV E6 and p53 protein is known as the most important event in HPV-associated carcinogenesis. Some in vitro studies suggested that p53 genetic variants are targeted for ubiquitin-proteasome degradation induced by E6 with different abilities. A common p53 variant at position 72 (R72P) has leaded...
journal_title:Acta medica portuguesa
pub_type: 杂志文章,评审
doi:
更新日期:2011-01-01 00:00:00
abstract:INTRODUCTION:Disciplinary actions may have a significant impact in medical doctors' and patients' lives. The objective of this study was the assessment of the disciplinary actions in Obstetrics and Gynecology that occurred in the north of Portugal in years 2008 to 2012. MATERIAL AND METHODS:Retrospective descriptive s...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2015-03-01 00:00:00
abstract::This paper describes the process followed to create and validate the Portuguese versión of a quality of life measurement instrument for patients in palliative care. After a literature review about the measurement of the quality of life in this particular and very specific kind of patients, we opt by the Irene Higginso...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2008-03-01 00:00:00
abstract::Over the years a number of high quality studies has established the effectiveness of external measures (tape, brace) and neuromuscular training for the prevention of ankle sprains. The general conclusion derived from these studies is that recurrent ankle sprains can be effectively prevented using any of these measures...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2013-03-01 00:00:00
abstract::The case of a 31 man with acute disautonomia envolving the parasympathetic and sympathetic systems but sparing sympathetic cholinergic division is presented. A Hodgkin lymphoma was diagnosed allowing the diagnosis of a paraneoplastic syndrome. ...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2002-03-01 00:00:00
abstract::The accurate treatment of a dyslipidaemia is based on correct diagnosis. Together with the laboratory values. A good clinical interview, focusing on the family history, is the bases for a good diagnosis strategy. The author schematze the rules of etiological diagnosis of inherited defects of lipid metabolism. ...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:1995-02-01 00:00:00
abstract::Treatment of breast cancer generally involves quadrantectomy and mastectomy followed by radiotherapy and chemotherapy depending on indication. The object of the present study was to verify the extent of movement of the shoulder after breast cancer treatment and the influence of the type of surgery (quadrantectomy and ...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2009-09-01 00:00:00
abstract:INTRODUCTION:Metals are a common cause of allergic contact dermatitis. After the introduction of the EU Nickel Directive (1994/27/CE; 2004/96/EC) and, more recently, the Cement Directive (2003/53/EC) there has been a significant decrease in sensitization to metals mainly in the Nordic countries. The applicability of th...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2014-05-01 00:00:00
abstract::Schistosomiasis is a major cause of liver damage and portal hypertension, especially in tropical countries, affecting about 200 million people in the world. We report the cases of two patients presenting persistent alterations of serum liver enzymes. We enhance the importance of considering the history of past travels...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:1998-10-01 00:00:00
abstract::Two types of platelet alloantigens are considered: type I, which refers to platelet alloantigens shared by platelets and other cells; and type II, which comprises the platelet-specific alloantigens. The Human Platelet Alloantigens (HPA) System consists of five diallelic alloantigens and eleven low frequency antigens. ...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2000-09-01 00:00:00
abstract::The aim of this study was to evaluate the association between maternal nutritional factors and intrauterine growth retardation (IUGR). We studied 1233 mothers who delivered single term newborns: 144 IUGR-birth weight less than one 10th percentile for sex and gestational age and 1119 controls-birth weight between the 1...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:1994-12-01 00:00:00
abstract::In this work, 139 intra-cranial aneurysms diagnosed at the Egas Moniz Hospital were studied, from 1980 to the first quarter of 1992. The nature of this type of lesions was discussed, and aneurysms were defined as an abnormal and segmental dilatation of an intracranial artery wall. A statistical study was performed, in...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:1992-11-01 00:00:00
abstract::The development of a kidney abscess is extremely rare despite the fact that urinary tract infections are quite common during pregnancy. The pathogenesis of renal abscess has changed over the years. Nowadays, more than 75% of the renal abscess arises from a previous urinary tract infection. The authors report the case ...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2006-09-01 00:00:00
abstract::Nowadays, the digestive system, must be considered as an actual endocrinous gland. The authors, after pointing out the pharmacological and immunomodulating actions of endogenous somatostatine, briefly describe a new synthetic octapeptid, somatostatine-analogue, with similar pharmacological effects (octreotide). Finall...
journal_title:Acta medica portuguesa
pub_type: 杂志文章,评审
doi:
更新日期:1993-01-01 00:00:00
abstract::The authors present a case of a 60-year-old male patient, previously diagnosed with B-cell chronic lymphocytic leukemia, who was admitted to the Emergency Room with dyspnea. The initial evaluation revealed severe anemia (Hgb = 5.0 g/dL) with hyperleukocytosis (800.000/µL), nearly all of the cells being mature lymphocy...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2016-05-01 00:00:00
abstract:INTRODUCTION:Deep brain stimulation (DBS) is effective in advanced Parkinson's disease (PD), improving motor symptoms, fluctuations and quality of life. However, adverse psychiatric outcomes have been reported, albeit variably and in an unstandardized fashion. We aimed to summarize the published evidence on the outcome...
journal_title:Acta medica portuguesa
pub_type: 杂志文章,meta分析,评审
doi:10.20344/amp.4928
更新日期:2014-05-01 00:00:00
abstract::The effect of different concentrations of aluminum sulphate on the nonenzymatic oxidation of dopamine was studied in order to evaluate the action of this metal on neuromelanin synthesis. Data shows that under the studied conditions, aluminum partially inhibits dopamine self-oxidation, decreasing the formation of some ...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:1994-11-01 00:00:00
abstract::After a brief introduction about the clinical, histological and therapeutic characteristics of myxopapillar spinal ependymoma of cone medullar and filum terminale, three cases with medullar and intracranial seeding of myxopapillar spinal ependymoma are retrospectively reviewed. Surgical resection was total in the chil...
journal_title:Acta medica portuguesa
pub_type: 杂志文章,评审
doi:
更新日期:2001-01-01 00:00:00
abstract:INTRODUCTION:The concept of resilience refers to the possibility of individuals to develop positively when exposed to situations of adversity or stress. This is a complex process involving the interaction of vulnerability and protection factors. Researching resilience only makes sense when applied to populations consid...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:2011-12-01 00:00:00
abstract:OBJECTIVE:To evaluate, in an elderly Portuguese population, the diagnostic capacities of the most popular treadmill stress test protocols. DESIGN:Retrospective study of an elderly Portuguese population submitted to a stress test. PATIENTS AND METHODS:A population of 45 patients (35 male), aged 65 or more years, conse...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:1997-04-01 00:00:00
abstract::The authors present the experience of 63 clinical cases of microsurgical reconstruction of the head and neck. These reconstructions were accomplished by different microvascular free flaps which were selected according to the type, size and location of the defects. Fasciocutaneous, osteofasciocutaneous, adipofascial, m...
journal_title:Acta medica portuguesa
pub_type: 杂志文章
doi:
更新日期:1998-10-01 00:00:00