IgG4-related disease (IgG4-RD) is a recently described systemic entity of unknown origin. It predominantly affects older men and has distinctive histopathologic features as storiform fibrosis, obliterative phlebitis, dense lymphoplasmacytic infiltrate with immunostaining for IgG4, and it may be associated with elevated serum levels of IgG4. Although any organ can be affected, pituitary gland is rarely involved. We describe the case of a 36-year-old man who presented with headaches, impaired vision, panhypopituitarism with diabetes insipidus and an infiltrative lesion mainly of infundibulum and pituitary. We arrived at diagnosis of IgG4-RD by pituitary biopsy. A successful response to treatment with immunosuppressive doses of corticosteroids was achieved.

La enfermedad relacionada a IgG4 (IgG4-RD) constituye una entidad sistémica recientemente descrita, de causa desconocida. Afecta predominantemente a hombres mayores y presenta características histopatológicas distintivas, como fibrosis estoriforme, flebitis obliterante y denso infiltrado linfoplasmocitario con inmunomarcación para IgG4, pudiendo estar asociada a elevación sérica de dicha inmunoglobulina. Si bien cualquier órgano puede estar afectado, el compromiso de la hipófisis es infrecuente. Describimos el caso de un hombre de 36 años que se presentó con cefaleas, alteración del campo visual, panhipopituitarismo, diabetes insípida y una imagen que mostraba una lesión infiltrativa infundíbulo-panhipofisaria extendida. Arribamos al diagnóstico de IgG4-RD a través de biopsia hipofisaria. La respuesta al tratamiento con dosis inmunosupresoras de corticoides fue exitosa.

Neurocysticercosis is a central nervous system (CNS) infection caused by the larval stage of Taenia solium. This major parasitic infection is the most common cause of adult-onset epilepsy. The most common clinical manifestations of the parenchymal form of this disease are headache and seizures, whereas extraparenchymal forms typically present with hydrocephalus. In this context, it is important to emphasize that the clinical manifestations of this disease are the result of the death of the tapeworm larvae and of the perilesional inflammatory reaction that occurs in the CNS. The diagnosis of neurocysticercosis is based on epidemiology, clinical manifestations, neuroimaging findings and serology. Treatment of neurocysticercosis includes the use of antiepileptic drugs, corticosteroids and antiparasitic therapy with albendazole or praziquantel. We report two cases of parenchymal neurocysticercosis with single lesions presenting with headache and seizures.

La neurocisticercosis (NCC) es una infección del sistema nervioso central (SNC) originada por el estadio larvario de Taenia solium. Esta importante parasitosis es la causa más frecuente de epilepsia adquirida del adulto. Las manifestaciones clínicas más comunes de las formas parenquimatosas son la cefalea y las convulsiones, mientras que las formas extraparenquimatosas suelen presentarse con hidrocefalia. En este aspecto, es importante destacar que las manifestaciones clínicas de la enfermedad son el resultado de la muerte de la larva del cestodo y de la reacción inflamatoria perilesional que se produce en el SNC. El diagnóstico de NCC se basa en la epidemiología, las manifestaciones clínicas, los hallazgos de las neuroimágenes y la serología, y su tratamiento incluye el uso de fármacos antiepilépticos, corticoesteroides y drogas antiparasitarias, como el albendazol o el praziquantel. En este trabajo se describen dos casos de neurocisticercosis parenquimatosa con lesiones únicas que se manifestaron con cefalea y convulsiones.

A meningoencefalomielite pelo vírus varicela-zoster (VVZ) é uma complicação neurológica rara mas grave da reativação do VVZ em pacientes imunocomprometidos. Nós relatamos o caso de um indivíduo infectado por HIV que desenvolveu uma meningoencefalomielite aguda e grave acompanhada por uma erupção cutânea por causa do VVZ. A presença do DNA do VVZ no líquor foi confirmada pela técnica de reação em cadeia da polimerase (PCR). O paciente iniciou uma terapia intravenosa com aciclovir com uma leve recuperação das manifestações neurológicas. O vírus varicela-zoster deve ser incluído como uma causa de meningoencefalomielite nos pacientes com AIDS. O diagnóstico precoce seguido por terapia específica pode modificar o curso rápido e fulminante deste tipo de pacientes.

Varicella-zoster virus (VZV) meningoencephalomyelitis is a rare but severe neurological complication of VZV reactivation in immunocompromised patients. We report the case of an HIV-infected individual who developed an acute and severe meningoencephalomyelitis accompanied by a disseminated cutaneous eruption due to VZV. The presence of VZV DNA in cerebrospinal fluid was confirmed by polymerase chain reaction (PCR) technique. The patient started undergoing an intravenous acyclovir therapy with a mild recovery of neurological manifestations. Varicella-zoster virus should be included as a cause of acute meningoencephalomyelitis in patients with AIDS. Early diagnosis followed by specific therapy should modify the rapid and fulminant course for this kind of patients.

Paracoccidioidomycosis is an endemic systemic disease in subtropical areas of Central and South America caused by a dimorphic fungus Paracoccidioides brasiliensis. Central nervous system involvement is a rare complication of the chronic disseminated disease that can affect the brain, cerebellum, brainstem and the spinal cord. The most frequent clinical form of neuroparacoccidiodomycosis is the cerebral abscess; with less frequency, the disease presents as a diffuse chronic meningoencephalitis. Here we present a patient with diagnosis of disseminated paracoccidioidomycosis and multiple cerebral lesions compatible with abscesses. Stereotactic biopsy followed by the microbiological and histopathological examination of the smears showed the characteristic yeast cells that confirmed the diagnosis of neuroparacoccidioidomycosis.

La paracoccidioidomicosis es una micosis sistémica, endémica de áreas tropicales y subtropicales de América Central y del Sur, causada por un hongo dimorfo denominado Paracoccidioides brasiliensis. El compromiso del sistema nervioso central es una rara complicación de la forma diseminada crónica de la enfermedad y puede comprometer el cerebro, el cerebelo, el tronco cerebral y la médula espinal. La forma clínica más común de la neuroparacoccidioidomicosis es el granuloma o absceso cerebral y, con menos frecuencia, la meningoencefalitis crónica. Se presenta un paciente con diagnóstico de paracoccidioidomicosis diseminada crónica con múltiples lesiones cerebrales compatibles con abscesos. La biopsia estereotáxica seguida del estudio histopatológico y microbiológico del material obtenido de las lesiones permitió observar las levaduras redondeadas con los brotes característicos de Paracoccidioides brasiliensis.

Focal brain lesions are frequent complications among HIV/AIDS patients. Between January 1999 and May 2007, 83 procedures of stereotactic brain biopsies in HIV/AIDS patients with focal cerebral lesions were carried out. The inclusion criteria were lack of response to current diagnostic and therapeutic guidelines for brain lesions. All the samples underwent microscopic evaluation during surgery to assert valid material and delayed histopathological and microbiological examination. Forty one patient images demonstrated multiple brain lesions. Sixty two cases had supratentorial localization, 4 lesions were located beneath the tentorium and 17 showed both settings. Fifty one lesions presented peripheral enhancement after contrast computed tomography (CT) or magnetic resonance imaging (MRI). A 100% of useful samples recovery was achieved. Progressive multifocal leucoencephalopathy (PML) was the most frequent diagnosis (29%), followed by primary central nervous system lymphoma (PCNSL) (23%), and toxoplasmosis (15.7%). Statistically significant association was observed between histopathological diagnosis and lesion location and between those and peripheral ring enhancement images. The positive diagnostic rate of the invasive procedure was 90.3%. The morbidity/mortality rate was 2.4% in this series. In conclusion, the stereotactic brain biopsy ordered early during the patient’s evolution showed a good performance in order to achieve a prompt and accurate diagnosis and to guide the therapeutic scheme in these AIDS patients with focal brain lesions.

Las lesiones cerebrales focales constituyen una complicación frecuente en los pacientes con infección por el virus de la inmunodeficiencia humana (HIV) y síndrome de inmunodeficiencia adquirida (sida). Durante el período comprendido entre enero de 1999 y mayo de 2007 se realizaron un total de 83 biopsias en pacientes con sida y lesiones cerebrales. Se incluyeron aquellos pacientes que no hubiesen respondido al algoritmo habitual de enfoque diagnóstico-terapéutico de estas lesiones. Todas las muestras obtenidas fueron sometidas a evaluación intraoperatoria para asegurar la obtención de material patológico y posterior análisis histopatológico y exámenes microbiológicos. De los 41 pacientes con lesiones cerebrales múltiples, 62 tenían localización supratentorial, en 4 eran infratentoriales y 17 mostraron ambas localizaciones. Cincuenta y un lesiones seleccionadas como blanco estereotáctico tuvieron refuerzo periférico del contraste. Se obtuvo material histopatológico en el 100% de los procedimientos. El diagnóstico más frecuente fue el de leucoencefalopatía multifocal progresiva (LEMP) con 24 casos (29%), seguido del linfoma primario del sistema nervioso central (LPSNC) con 19 diagnósticos (23%) y de toxoplasmosis en 13 pacientes (15.7%). Se comprobó una relación significativa entre los diagnósticos histopatológicos y la localización de las lesiones y entre los diagnósticos histopatológicos y el comportamiento de las imágenes luego de la administración de la sustancia de contraste. El rédito diagnóstico alcanzó el 90.3% (75 biopsias). La morbiletalidad en esta serie fue de 2.4%. La biopsia cerebral estereotáctica permitió alcanzar el diagnóstico etiológico y adecuar el enfoque terapéutico en la mayoría de los pacientes de esta serie.

Intracranial and intraspinal involvement is a rare complication of Hodgkin's disease. We report a case of a patient with diagnosis of AIDS and Hodgkin's lymphoma who developed brain and spinal involvement at the time of the relapse of the neoplasm disease. Mixed cellularity histology was the subtype of Hodgkin's disease in our patient; we identified the Epstein-Barr virus genome in the Reed-Sternberg cells by immunohistochemistry and in situ hybridization.

Spinal epidural abscess (SEA) is a rare infectious disorder that often has delayed diagnosis and is associated with significant morbidity and mortality rates. We present a case of an AIDS patient with a SEA due to Mycobacterium tuberculosis. This type of SEA in AIDS patients is characterized by localized spinal pain and prolonged fever. Magnetic resonance imaging is the method of choice in the diagnostic process. Early diagnosis, followed by specific therapy (surgical decompression combined with antituberculous drugs), is necessary to improve the prognosis of these kinds of patients.

Relatamos um caso de meningoencefalite devida ao Trypanosoma cruzi em um paciente com síndrome de imunodeficiência adquirida. O paciente apresentou convulsões e sinais neurológicos focais. O diagnóstico definitivo de meningoencefalite chagásica foi feito pela demonstração de formas tripomastigotas livres no líquor. Foi iniciado benznidazol com melhora clínica e neurológica. As drogas antiretrovirais melhoraram a imunidade celular e três anos mais tarde o paciente tinha uma boa condição clínica com reconstituição imunológica e carga viral indetectável. A meningoencefalite chagásica tem um prognóstico ruim quando o tratamento específico não é iniciado ou quando há demora para substituí-lo. É necessário um alto índice de suspeita para o diagnóstico e tratamento precoces, especialmente em áreas endêmicas para a infecção pelo Trypanosoma cruzi.

We report a case of cerebral meningoencephalitis due to Trypanosoma cruzi in a patient with acquired immunodeficiency syndrome. The patient presented with seizures and focal neurological signs. Definitive diagnosis of chagasic meningoencephalitis was made by demonstration of free trypomastigote forms in the cerebrospinal fluid. Benznidazol was prescribed with clinical and neurological improvement. Antiretroviral drugs improved cellular immunity and three years later the patient presents a good clinical condition with immune reconstitution and undetectable viral load. Chagasic meningoencephalitis has a poor prognosis when specific treatment is not initiated or is delayed. A high index of diagnosis is necessary for early diagnosis and treatment, especially in endemic areas for Trypanosoma cruzi infection.

In the last years, new techniques of neuroimages and histopathological methods have been added to the management of cerebral mass lesions in patients with AIDS. Stereotactic biopsy is necessary when after 14 days of empirical treatment for Toxoplasma gondii encephalitis there is no clinical or neuroradiologic improvement. We report a woman with AIDS who developed a single focal brain lesion on the right frontal lobe. She presented a long history of headache and seizures. After two weeks of empirical treatment for toxoplasma encephalitis without response, a magnetic resonance image with spectroscopy was performed and showed a tumoral pattern with a choline peak, diminished of N-acetyl-aspartate and presence of lactate. A stereotactic biopsy was performed. Histopathological diagnosis was a diffuse oligodendroglioma type A. A microsurgical resection of the tumor was carried out and antiretroviral treatment was started. To date she is in good clinical condition, with undetectable plasma viral load and CD4 T cell count > 200 cell/uL.

En los últimos años, las nuevas técnicas de neuroimágenes y diversos métodos de diagnóstico histopatológico se han agregado al manejo clínico de las lesiones de masa cerebral ocupante en los pacientes con sida. La biopsia estereotáxica es necesaria cuando, luego de dos semanas de tratamiento empírico para toxoplasmosis cerebral, no se comprueba mejoría clínica ni neurorradiológica. Presentamos una paciente con sida que desarrolló una lesión cerebral a nivel del lóbulo frontal derecho. Como antecedente refirió una larga historia de cefalea y convulsiones. La resonancia nuclear magnética con espectroscopia de voxel único ubicado a nivel de la lesión mostró un patrón de lesión tumoral con pico de colina, déficit de N-acetil-aspartato y presencia de ácido láctico. La biopsia estereotáxica y el estudio histopatológico permitieron arribar al diagnóstico de oligodendroglioma difuso de tipo A. Se le efectuó resección por microcirugía y tratamiento antirretroviral de alta eficacia. Actualmente la paciente se encuentra en buen estado clínico, con carga viral indetectable y recuento de linfocitos T CD4 + > de 200 cél/uL.