Neurosarcoidosis as a rapidly progressive dementia associated with normal pressure hydrocephalus.

Neurosarcoidosis (NS) is a rare subtype of sarcoidosis with a poor prognosis and diverse clinical presentations that often poses a diagnostic and therapeutic challenge. We describe the case of a 53-year-old male with an initial diagnosis of lingual sarcoidosis, who subsequently developed ataxia and rapidly progressive cognitive impairment. A lumbar puncture revealed hypoglycorrhachia, hyperproteinorrachia, lymphocytic pleocytosis, and elevated IL-6 levels (600 pg/ml). Cerebrospinal fluid flow cytometry showed an elevated CD4 lymphocyte concentration and a CD4+/CD8+ ratio of 3.91, indicative of NS. Brain MRI showed hyperintense periventricular and subcortical lesions on FLAIR/T2 resembling progressive multifocal leukoencephalopathy (PML), although negative PCR for JC virus ruled out the differential diagnosis. Following a favorable evolutionary course with corticosteroid pulses, the patient relapsed with normotensive hydrocephalus, treated with immunosuppressants and ventriculoperitoneal shunting with a good response to date. This case underscores the importance of maintaining a high index of suspicion for NS in individuals with sarcoidosis and neurologic symptoms. In these cases, cerebrospinal fluid biomarkers such as IL-6 and CD4+/CD8+ ratio are essential to guide the diagnosis. Furthermore, it highlights that hydrocephalus is a rare complication and requires a multidisciplinary approach, including medical and neurosurgical treatment.

La neurosarcoidosis es un subtipo raro de sarcoidosis con mal pronóstico y diversas presentaciones clínicas que a menudo plantea un reto diagnóstico y terapéutico. Describimos el caso de un varón de 53 años con diagnóstico inicial de sarcoidosis lingual, que posteriormente desarrolló ataxia y deterioro cognitivo de rápida evolución. Una punción lumbar reveló hipoglucorraquia, hiperproteinorraquia, pleocitosis linfocítica y niveles elevados de IL-6 (600 pg/ml). La citometría de flujo del líquido cefalorraquídeo mostró una concentración elevada de linfocitos CD4 y un cociente CD4+/CD8+ de 3.91, indicativo de neurosarcoidosis. La RM cerebral evidenció lesiones hiperintensas periventriculares y subcorticales en FLAIR/T2 que se asemejaban a una leucoencefalopatía multifocal progresiva (LMP), aunque la PCR negativa para el virus JC descartó el diagnóstico diferencial. Tras un curso evolutivo favorable con pulsos de corticoides, el paciente recayó con hidrocefalia normotensiva, tratada con inmunosupresores y derivación ventriculoperitoneal con buena respuesta hasta la fecha. Este caso subraya la importancia de mantener un alto índice de sospecha de neurosarcoidosis en individuos con sarcoidosis y síntomas neurológicos. En estos casos, los biomarcadores del líquido cefalorraquídeo tales como la IL-6 y el cociente CD4+/CD8+ son esenciales para orientar el diagnóstico. Además, destaca que la hidrocefalia es una complicación poco frecuente y requiere un abordaje multidisciplinario, que incluya tratamiento médico y neuroquirúrgico.

Pioderma gangrenoso ampollar como forma de presentación de leucemia mieloide agudo: informe de un caso / Acute myeloid leukemia presented in the form of bullous pyoderma gangrenosum: a case report

El pioderma gangrenoso ampollar es una variedad infrecuente de pioderma gangrenoso, que se asocia en el 50-70% de los casos con trastornos oncohematológicos. Se comunica el caso de una paciente de 59 años, que consultó por fiebre y ampollas purpúricas de rápida progresión, con compromiso cutáneo mucoso. Con sospecha de una enfermedad neutrofílica, ampollar, o infección por gérmenes oportunistas, se realizó biopsia de piel para estudio histopatológico, inmunofluorescencia directa y cultivo. Los cultivos y la inmunofluorescencia directa fueron negativos, y la anatomía patológica reveló un denso infiltrado inflamatorio con predominio neutrofílico en dermis. Ante el diagnóstico de pioderma gangrenoso ampollar, se realizó una punción-aspiración de médula ósea cuyo resultado fue compatible con leucemia mieloide aguda. Se instauró tratamiento con corticosteroides sistémicos, a pesar de lo cual la paciente evolucionó desfavorablemente y falleció a los 15 días de su ingreso hospitalario. Este caso ilustra la asociación de esta enfermedad cutánea con trastornos oncohematológicos y el mal pronóstico que esto implica a corto plazo. (AU)

Bullous pyoderma gangrenosum is an infrequent type of pyoderma gangrenosum, associated with onco hematological diseases in 50-70% of cases. We present the case of a 59-year-old patient with fever and mucocutaneous hemorrhagic bullous of rapid progression. A biopsy for histopathology, direct immunofluorescence (DIF) and skin culture was made, considering the possibility of neutrophilic dermatoses, bullous dermatosis or an opportunistic infection. The results of both the culture and the DIF were negative. The histopathological examination of the specimen revealed a dense dermal polymorphic infiltrate composed primarily of neutrophils. Considering bullous pyoderma gangrenosum as a potential diagnosis, a bone-marrow biopsy was performed. This study revealed an acute myeloid leukemia. Although systemic corticosteroid therapy was begun, the patient presented an unfavorable evolution that led to her death 15 days after her admission at the hospital. This case shows the association between bullous pyoderma gangrenosum and onco hematological diseases. In addition, it highlights the poor prognosis related to these diseases in the short term. (AU)

[Mortality and recurrence of venous thromboembolic disease in adult patients: prospective cohort]. / Mortalidad y recurrencia de la enfermedad tromboembólica venosa en pacientes adultos: cohorte prospectiva.

INTRODUCTION: Venous thromboembolic disease (VTE) is a pathology that increases with age. RESULTS: 446 patients were included, 63% (292) were older than 65 years. Survival was lower in the elderly compared to the young (p 0.007), at 3 months 87% vs. 95% and at one year 73% vs. 87%, respectively. The elderly had a HRc1.71 and HR at 1.68. The overall recurrence was 5% (95% CI 3-8) at one month, 6% (95% CI 4-9) at 3 months, 8% (95% CI). 6-11) at one year and 13% (95% CI 9-18) at two years. No association was found between age and recurrence sub hazard 0.8 (CI 0.34-1.86). Bleeding occurred in 9% (39) of the patients. OBJECTIVE: to compare the survival of the elderly and the young with a first episode of acute and symptomatic VTE. MATERIALS AND METHODS: Prospective cohort of incident VTE cases included in the Institutional Registry of Venous Thromboembolic Disease (NCT01372514) of the Italian Hospital of Buenos Aires between 2012-2014, divided into young groups (17-64 years old) and elderly (65 years old). All the patients were followed annually to assess the time to recurrence (progression or new symptomatic event of VTE) as competitive events in the context of death and major bleeding. Raw (c) and adjusted (a) risks were presented. CONCLUSIONS: The overall mortality in patients with confirmed VTE is higher in the elderly population. There were no differences in relation to the recurrence of VTE, or bleeding, and neither with age.

INTRODUCCIÓN: La enfermedad tromboembólica venosa (ETV) es una patología que aumenta con la edad. CONCLUSIONES: La mortalidad global en pacientes con ETV confirmada es mayor en la población anciana. No hubo diferencias en relación a la recurrencia de ETV, ni   el sangrado y tampoco con la edad. OBJETIVO: Comparar la sobrevida de los ancianos y los jóvenes con un primer episodio de ETV aguda y sintomática. MATERIALES Y MÉTODOS: Cohorte prospectiva de casos incidentes de ETV incluidos en el Registro Institucional de Enfermedad Tromboembólica venosa (NCT01372514) del Hospital Italiano de Buenos Aires entre 2012-2014, dividido en grupos jóvenes (17-64 años) y ancianos (≥ 65 años). Todos los pacientes fueron seguidos anualmente para evaluar el tiempo a la recurrencia (progresión o nuevo evento sintomático de ETV) como eventos competitivos en contexto de muerte y sangrado mayor. Se presentaron los riesgos crudos (c) y ajustados. (a). RESULTADOS: Se incluyeron 446 pacientes, el 63% (292) fueron mayores de 65 años. La sobrevida fue menor en los ancianos comparados con los jóvenes (p 0.007), a los 3 meses 87% vs 95% y al año 73% vs 87%, respectivamente. Los ancianos presentaron un HRc1,71 y HR a 1.68. La recurrencia global fue 5% (IC 95% 3-8) al mes, 6% (IC 95% 4-9) a los 3 meses, 8% (IC 95% 6-11) al año y 13% (IC 95% 9-18) a los dos años. No se encontró asociación entre la edad y la recurrencia sub hazard 0.8(IC 0,34-1,86). El sangrado ocurrió en un 9% (39) de los pacientes.

[Granulocytic Sarcoma: Two cases with Lymph Node Presentation]. / Sarcoma Granulocítico : Dos Casos con compromiso Ganglionar.

Two cases of granulocytic sarcoma (GS) with lymph node presentation without marrow involvement are presented because it is a rare presentation of acute myeloid leukemia (AML) that can coexist with or precede it and as initial symptom (case 1) or disease relapse (case 2). The most common differential diagnoses are lymphoma or solid tumor. Biopsy is essential for definitive diagnosis. Imaging studies such as positron emission tomography (PET) are very useful for staging and monitoring. The prognosis and treatment according to the literature is not different from AML. The two cases presented were diagnosed with lymph node biopsy with a bone marrow that did not meet criteria for AML.

Huge Pulmonary Arteriovenous Malformation, Venous Thromboembolism and Anticoagulation Treatment in a Patient with Hereditary Hemorrhagic Telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT) usually presents in association with pulmonary arteriovenous malformations (PAVMs). In addition, the incidence of venous thromboembolism tends to be increased in these patients. A 74-year-old female with HHT presented with cyanosis and hypoxemia. Contrast-enhanced multislice computed tomography (MSCT) revealed two left PAVMs and one in the right upper lobe. Both left PAVMs were treated with embolotherapy. Follow-up MSCT revealed an incidental pulmonary embolism in the right pulmonary branches. Deep venous thrombosis was confirmed and anticoagulation was initiated. Follow-up MSCT revealed the resolution of thromboembolism. Finally, embolotherapy was performed. This case illustrates the chronic adaptation to hypoxemia and adds further evidence to the relative safety of anticoagulation treatment in these patients.

[POEMS syndrome. A report of four cases]. / Síndrome POEMS. Comunicación de cuatro casos.

Four cases of a rare paraneoplastic syndrome associated to a plasmatic cell disorder with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesions (POEMS) are here reported. The purpose of the communication is to warn of different forms of presentation of POEMS syndrome to decrease the time of diagnosis, because early treatment reduces sequels and improves quality of life in the long term, also to remark the importance of classifying the hematological disease for specific treatment.

Síndrome POEMS: Comunicación de cuatro casos / POEMS syndrome. A report of four cases

Se presentan cuatro casos de un raro síndrome paraneoplásico de una discrasia de células plamáticas compuesto por polineuropatía, organomegalia, endocrinopatía, banda monoclonal y lesiones cutáneas (POEMS). El objetivo de la comunicación de esta serie es alertar sobre diferentes formas de presentación del síndrome de POEMS para disminuir el tiempo de diagnóstico, ya que el tratamiento temprano reduce las secuelas y mejora la calidad de vida a largo plazo; también, señalar la importancia de la clasificación de la enfermedad hematológica para realizar el tratamiento específico.

Four cases of a rare paraneoplastic syndrome associated to a plasmatic cell disorder with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin lesions (POEMS) are here reported. The purpose of the communication is to warn of different forms of presentation of POEMS syndrome to decrease the time of diagnosis, because early treatment reduces sequels and improves quality of life in the long term, also to remark the importance of classifying the hematological disease for specific treatment.

[Delirium in elderly inpatients. An 18 month follow-up]. / Delirium en ancianos hospitalizados. Seguimiento de 18 meses.

Delirium usually hardens care during hospitalization and increases morbidity during hospital stay and after discharge. The objective of this study was to describe the prevalence of delirium in elderly inpatients in a Buenos Aires hospital, its morbidity and mortality during hospital stay and the next 18 month follow-up. Patients aged 70 or older admitted to internal medicine unit between September 2005 and May 2006 were enrolled. Delirium was assessed with the Spanish version of Confusion Assessment Method. Demographic data, cause of admition and length of stay, destination after discharge and mortality were registered. A new evaluation was made 18 months after discharge. We evaluated 194 patients and 74 were excluded. Of the 120 included, 52 (43.3%) presented delirium. We found significant differences between patients with and without delirium in previous placement in nursing home (17.3% vs. 1.5%; p < 0.002), dementia (40.4% vs. 8.8%; p < 0.001), median activity of daily living (5 vs. 6; p < 0.001), length-of-stay (7 vs. 5; p = 0.04) and mortality rate (21.2% vs. 1.5%; p < 0.001). Evaluation 18 months later showed differences between patients with and without delirium in median of activity of daily living (1/6 vs. 5/6), patients living in nursing homes (27.5% vs. 7.9%), estimated survival 35.3% (CI 95%: 24-49%) at day 569 and 49% (CI 95%: 32.9-65.4%) at day 644. The difference between survival curves was statistically significant (p = 0.027). Delirium increases morbidity and mortality during hospital stay. Elderly with delirium are at risk of worsening disability and of becoming dependent after discharge and it is a risk factor for higher mortality during the following months after discharge.

Delirium en ancianos hospitalizados: Seguimiento de 18 meses / Delirium in elderly inpatients: An 18 month follow-up

El objetivo de este trabajo fue describir la prevalencia de síndrome confusional agudo en ancianos hospitalizados, la evolución durante la internación y a los 18 meses. Se evaluó en forma prospectiva a pacientes de 70 años de edad o mayores, internados en el Servicio de Clínica Médica de nuestro hospital, entre septiembre de 2005 y mayo de 2006. Se utilizó una versión validada en español del Confussion Assessment Method para diagnosticar delirium. A los 18 meses se evaluó el estado vital, lugar de residencia, actividades de la vida diaria, dependencia de cuidadores y reinternaciones. Se evaluaron 194 pacientes y 74 fueron excluidos. De los 120 casos incluidos, 52 (43.3%) presentaron delirium. La edad media fue de 82.6 años (DS: 7.4) en el grupo de pacientes con síndrome confusional agudo y de 80.4 años (DS: 5.6) en el grupo de pacientes sin delirium. Al comparar estos dos grupos encontramos diferencias significativas en las características, siendo más frecuentes en el grupo con delirium la residencia previa en un centro de tercer nivel (17.3% vs. 1.5%; p < 0.002), la demencia (40.4% vs. 8.8%; p < 0.001), menor puntaje en la evaluación de las actividades de la vida diaria (5 vs. 6; p < 0.001), más días de internación (7 vs. 5; p = 0.04) y mayor mortalidad intrahospitalaria (21.2% vs. 1.5%; p < 0.001). A los 18 meses de seguimiento después del alta, el desarrollo de delirium durante la internación se asoció a mayor grado de dependencia evidenciado por el peor puntaje en las actividades de la vida diaria (mediana 1/6 vs. 5/6) y a menor sobrevida actuarial 35.3% (CI 95%: 24-49%) a los 569 días y 49% (CI 95%: 32.9-65.4%) a los 644 días (p=0.027).

Delirium usually hardens care during hospitalization and increases morbidity during hospital stay and after discharge. The objective of this study was to describe the prevalence of delirium in elderly inpatients in a Buenos Aires hospital, its morbidity and mortality during hospital stay and the next 18 month follow-up. Patients aged 70 or older admitted to internal medicine unit between September 2005 and May 2006 were enrolled. Delirium was assessed with the Spanish version of Confusion Assessment Method. Demographic data, cause of admition and length of stay, destination after discharge and mortality were registered. A new evaluation was made 18 months after discharge. We evaluated 194 patients and 74 were excluded. Of the 120 included, 52 (43.3%) presented delirium. We found significant differences between patients with and without delirium in previous placement in nursing home (17.3% vs. 1.5%; p < 0.002), dementia (40.4% vs. 8.8%; p < 0.001), median activity of daily living (5 vs. 6; p < 0.001), length-of-stay (7 vs. 5; p = 0.04) and mortality rate (21.2% vs. 1.5%; p < 0.001). Evaluation 18 months later showed differences between patients with and without delirium in median of activity of daily living (1/6 vs. 5/6), patients living in nursing homes (27.5% vs. 7.9%), estimated survival 35.3% (CI 95%: 24-49%) at day 569 and 49% (CI 95%: 32.9-65.4%) at day 644. The difference between survival curves was statistically significant (p = 0.027). Delirium increases morbidity and mortality during hospital stay. Elderly with delirium are at risk of worsening disability and of becoming dependent after discharge and it is a risk factor for higher mortality during the following months after discharge.