Progressive multifocal leukoencephalopathy: a challenging diagnosis established at autopsy

Progressive multifocal leukoencephalopathy (PML) is a feared entity that occurs most frequently in conditions of extreme immunodeficiency. The diagnosis is often made long after the onset of symptoms due to the physicians' unfamiliarity, and the unavailability of diagnostic tests in some medical centers. Although the incidence of PML is decreasing among HIV patients with the advent of highly active antiretroviral therapy (HAART), in Brazil this entity is the fourth highest neurological complication among these patients. The authors present the case of a middle-aged man who tested positive for HIV concomitantly with the presentation of hyposensitivity in the face and the right side of the body, accompanied by mild weakness in the left upper limb. The clinical features worsened rapidly within a couple of weeks. The diagnostic work-up pointed to the working diagnosis of PML after brain magnetic resonance imaging; however, the detection of the John Cunningham virus (JCV) in the cerebral spinal fluid was negative. HAART was started but the patient died after 7 weeks of hospitalization. The autopsy revealed extensive multifocal patchy areas of demyelination in the white matter where the microscopy depicted demyelination, oligodendrocytes alterations, bizarre atypical astrocytes, and perivascular lymphocytic infiltration. The immunohistochemistry was positive for anti-SV40, and the polymerase chain reaction of the brain paraffin-embedded tissue was positive for JCV. The authors highlight the challenges for diagnosing PML, as well as the devastating outcome of PML among HIV patients.

Celiac Crisis: an unusual presentation of gluten-sensitive enteropathy

Celiac disease (CD)­also known as gluten-sensitive enteropathy­is a chronic, genetically predisposing and autoimmune entity with a wide range of clinical manifestations triggered by gluten ingestion, which affects 1% of the general population. Currently, up to 60% of the diagnosis of CD is in adults due to the atypical course of the disease. The severe acute onset of CD­also called celiac crisis­is very uncommon and is still not well documented in adults. We report the case of a 58-year-old man who presented a 45-day history of subtle-onset diarrhea followed by malabsorption syndrome with progressive weight loss, anasarca, and electrolyte disturbances. The diagnostic work-up included an upper digestive endoscopy, which showed scalloping of the duodenal mucosa with pathological features confirmed on biopsies. Specific antibodies were positive, and a satisfactory clinical response was obtained once a gluten-free diet was started. Celiac crisis is a rare initial presentation of CD characterized by severe diarrhea, dehydration, weight loss, hypoproteinemia, and metabolic and electrolyte disturbances. Although rare, it should be considered in patients with apparently unexplained chronic diarrhea.

Bicytopenia and leukoerythroblastosis: a rare initial presentation of signet ring cell gastric adenocarcinoma

Gastric adenocarcinoma is a common neoplasia and is responsible for up to 30% of the overall deaths due to cancer. Advanced disease is mostly characterized by peritoneum, liver, and lung involvement. The spread of the disease to the bone is rare, and bone marrow dissemination is even rarer. In this setting, leukoerythroblastosis may be the initial manifestation of the disease. The authors report the case of a 64-year-old Caucasian man who sought medical care complaining of back pain, weakness, and weight loss. The physical examination revealed pallor, and the laboratory work-up depicted severe anemia and thrombocytopenia; the peripheral blood smear was consistent with leukoerythroblastosis. The ongoing investigation through a bone marrow biopsy showed massive involvement of the bone marrow by a signet ring cell adenocarcinoma. During hospitalization, the patient presented melena, and an upper digestive endoscopy depicted an ulcerated and infiltrative lesion in the cardia, upon which the histological examination revealed a signet ring cell adenocarcinoma. This case highlights the bone marrow invasion represented by bicytopenia and leukoerythroblastosis as the initial manifestation of this histological type of gastric cancer. Although treatment attempts were made with chemotherapy and radiotherapy, the patient died early on, showing the aggressive behavior of this form of tumoral presentation.

Comprometimento do sistema nervoso no lupus eritematoso sistemico / Involvement of the nervous system in systemic lupus erythematosus

O comprometimento do sistema nervoso (SN) no lupus eritematoso sistemico (LES) envolve multiplas manifestacoes de graus variaveis, entre elas fadiga, perda de capacidade de concentracao, convulsoes e coma. O diagnostico precoce do envolvimento do SN e de fundamental importancia para a adocao de medidas terapeuticas mais objetivas. No presente trabalho os autores discorrem sobre aspectos clinicos, laboratoriais e terapeuticos do comprometimento do SN no LES.

Miosite tropical / Tropical myositis

Os autores descrevem seis casos de miosite tropical ou piomiosite que ocorreram na Divisao de Clinica Medica do Hospital Universitario da USP durante o periodo de 1985 a 1992. O Staphylococcus aureus foi o agente responsavel por este quadro infeccioso sendo isolado a partir de abscessos musculares em quatro casos e de hemoculturas em dois casos. A antibioticoterapia adequada associada a drenagem cirurgica determinou evolucao satisfatoria, sem mortalidade e sequelas osteomusculares.

Indice de estadiamento radiologico na osteoartrose / Radiological staging index in osteoarthrosis

A associacao de dor articular e alteracoes radiologicas (como osteofitos, reducao do espaco articular e em alguns casos cistos e erosoes osseas) e o criterio mais comumente adotado na pratica diaria para o diagnostico de osteoartrose (OA). No entanto, o achado de tais alteracoes radiologicas nao permite quantificar de maneira objetiva a intensidade do comprometimento articular. Com o objetivo de mensurar de forma quantitativa as lesoes radiologicas que ocorrem na OA, os autores elaboram um INDICE DE ESTADIAMENTO RADIOLOGICO (IER), que leva em consideracao os seguintes parametros: I. gravidade das alteracoes radiologicas; II. area da articulacao comprometida e III. numero de articulacoes afetadas (uma ou mais de acordo com o interesse do investigador). A aplicacao deste indice na pratica medica, permite obter de forma simples a quantificacao das lesoes radiologicas da osteoartrose, possibilitando deste modo uma melhor monitorizacao clinica.

Mini-pulsos de ciclofosfamida no tratamento da dermatopolimiosite com envolvimento pulmonar / Mini-pulses of cyclophosphamide in the treatment of dermatopolymyositis with pulmonary envolvement

Os autores apresentam um novo esquema terapeutico para o tratamento da Dermatopolimiosite (DM/PM) com fibrose pulmonar refrataria a corticoterapia. Com o objetivo de atingir um controle clinico adequado e minimizar a ocorrencia de efeitos adversos, os autores propoem a utilizacao de ciclofosfamida endovenosa em esquema de pequenas doses mensais (mini-pulsos) e descrevem dois pacientes portadores de DM/PM com comprometimento pulmonar que nao respondem a prednisona. Foram submetidos ao esquema de mini-pulsos por aproximadamente dois anos com normalizacao do quadro clinico muscular e pulmonar que se mantiveram ate a ultima avaliacao ambulatorial.

Lupus eritematoso sistemico no idoso: caracteristicas clinicas e laboratoriais / Systemic lupus erythematosus in the elderly: clinical and laboratorial characteristics

As caracteristicas clinicas e laboratoriais de 199 pacientes com lupus eritematoso sistemico (LES) foram estudadas. Os pacientes que apresentaram inicio apos 50 anos foram comparados aqueles com inicio mais precoce. O acometimento clinico definiu diferencas importantes entre as duas populacoes. Os pacientes idosos apresentaram maior frequencia de manifestacoes musculares (p<0,05) e menor frequencia de alteracoes cutaneas (p<0,001) e de alopecia (p<0,02). Alem disso a apresentacao clinica mais frequente foi fraqueza muscular, poliartrite e emagrecimento (>10Kg), condicoes estas que podem sugerir o diagnostico de polimialgia reumatica ou doenca neoplasica. A presenca dos diversos auto-anticorpos foi semelhante nos dois grupos. Em contraste com estudos anteriores nao encontramos maior frequencia de anti-La/SSB no LES de inicio tardio. A apresentacao clinica do LES no idoso e menos exuberante e muitas vezes pouco caracteristica, exigindo consideracao especial para este diagnostico no sentido de se evitar retardo na sua terapeutica.