Rapidly progressive dementia due to neurosarcoidosis / Demência rapidamente progressiva causada por neurosarcoidose

Rapidly progressive dementia (RPD) is typically defined as a cognitive decline progressing to severe impairment in less than 1-2 years, typically within weeks or months. Accurate and prompt diagnosis is important because many conditions causing RPD are treatable. Neurosarcoidosis is often cited as an unusual reversible cause of RPD. METHODS: We report two cases of neurosarcoidosis presenting as RPD. RESULTS: Case 1: A 61-year-old woman developed a RPD associated with visual loss. In seven months she was dependent for self-care. Magnetic resonance imaging (MRI) revealed temporal and suprasellar brain lesions. Treatment with high-dose intravenous prednisolone was associated with partial improvement. Case 2: A 43-year-old woman who was being treated for diabetes insipidus developed a severe episodic amnesia one year after onset of cognitive symptoms. Previous MRI had shown a hypothalamic lesion and she had been treated with oral prednisone and cyclophosphamide. There was reduction of the MRI findings but no improvement in the cognitive deficits. Brain biopsy disclosed noncaseous granulomas and granulomatous angiitis; treatment was changed to high-dose intravenous methylprednisolone, with poor symptomatic response. CONCLUSION: The diagnosis of RPD due to neurosarcoidosis can be challenging when the disease is restricted to the nervous system. In these cases, clinical presentation of RPD associated with neuroendocrine and visual dysfunction, imaging findings showing hypothalamic lesions and, in some cases, brain biopsy, are the key to a correct diagnosis. It is possible that earlier diagnoses and treatment could have led to a better outcome in these patients.

Demência rapidamente progressiva (DRP) é tipicamente definida como um declínio cognitivo que progride para prejuízo funcional severo em menos de 1-2 anos, geralmente em semanas a meses. O diagnóstico rápido e acurado é fundamental, já que muitas condições que levam a DRP são reversíveis. MÉTODOS: Relatamos dois casos de neurosarcoidose que se apresentaram como DRP. RESULTADOS: Caso 1: Uma mulher de 61 anos desenvolveu uma DRP associada a perda de acuidade visual. Em sete meses evoluiu com dependência para auto-cuidado. A ressonância magnética (RM) revelou lesões encefálicas temporais e supraselares. Evoluiu com melhora parcial após tratamento com metilprednisolona intravenosa em altas doses. Caso 2: Uma mulher de 43 anos que estava em tratamento para diabetes insipidus desenvolveu uma amnésia episódica severa um ano após o início dos sintomas cognitivos. A RM anterior mostrava uma lesão hipotalâmica, e ela recebeu tratamento oral com prednisona e ciclofosfamida. Houve redução dos achados da RM, porem sem melhora dos déficits cognitivos. A biópsia cerebral mostrou granulomas não caseosos e angeíte granulomatosa; o tratamento foi modificado para metilprednisolona intravenosa em altas doses, com resposta sintomática pobre. CONCLUSÃO: O diagnóstico de DRP por neurosarcoidose pode ser desafiador quando a doença está restrita ao sistema nervoso central. Nestes casos, a apresentação clínica da DRP associada a disfunção neuroendócrina e visual, exames de imagem com lesões hipotalâmicas e, em alguns casos, a biópsia cerebral são fundamentais para um diagnóstico correto. é possível que o diagnóstico e tratamento precoces poderiam ter trazido melhores resultados nesses pacientes.

Rapidly progressive dementia due to neurosarcoidosis.

Rapidly progressive dementia (RPD) is typically defined as a cognitive decline progressing to severe impairment in less than 1-2 years, typically within weeks or months. Accurate and prompt diagnosis is important because many conditions causing RPD are treatable. Neurosarcoidosis is often cited as an unusual reversible cause of RPD. METHODS: We report two cases of neurosarcoidosis presenting as RPD. RESULTS: Case 1: A 61-year-old woman developed a RPD associated with visual loss. In seven months she was dependent for self-care. Magnetic resonance imaging (MRI) revealed temporal and suprasellar brain lesions. Treatment with high-dose intravenous prednisolone was associated with partial improvement. Case 2: A 43-year-old woman who was being treated for diabetes insipidus developed a severe episodic amnesia one year after onset of cognitive symptoms. Previous MRI had shown a hypothalamic lesion and she had been treated with oral prednisone and cyclophosphamide. There was reduction of the MRI findings but no improvement in the cognitive deficits. Brain biopsy disclosed noncaseous granulomas and granulomatous angiitis; treatment was changed to high-dose intravenous methylprednisolone, with poor symptomatic response. CONCLUSION: The diagnosis of RPD due to neurosarcoidosis can be challenging when the disease is restricted to the nervous system. In these cases, clinical presentation of RPD associated with neuroendocrine and visual dysfunction, imaging findings showing hypothalamic lesions and, in some cases, brain biopsy, are the key to a correct diagnosis. It is possible that earlier diagnoses and treatment could have led to a better outcome in these patients.

Demência rapidamente progressiva (DRP) é tipicamente definida como um declínio cognitivo que progride para prejuízo funcional severo em menos de 1-2 anos, geralmente em semanas a meses. O diagnóstico rápido e acurado é fundamental, já que muitas condições que levam a DRP são reversíveis. MÉTODOS: Relatamos dois casos de neurosarcoidose que se apresentaram como DRP. RESULTADOS: Caso 1: Uma mulher de 61 anos desenvolveu uma DRP associada a perda de acuidade visual. Em sete meses evoluiu com dependência para auto-cuidado. A ressonância magnética (RM) revelou lesões encefálicas temporais e supraselares. Evoluiu com melhora parcial após tratamento com metilprednisolona intravenosa em altas doses. Caso 2: Uma mulher de 43 anos que estava em tratamento para diabetes insipidus desenvolveu uma amnésia episódica severa um ano após o início dos sintomas cognitivos. A RM anterior mostrava uma lesão hipotalâmica, e ela recebeu tratamento oral com prednisona e ciclofosfamida. Houve redução dos achados da RM, porem sem melhora dos déficits cognitivos. A biópsia cerebral mostrou granulomas não caseosos e angeíte granulomatosa; o tratamento foi modificado para metilprednisolona intravenosa em altas doses, com resposta sintomática pobre. CONCLUSÃO: O diagnóstico de DRP por neurosarcoidose pode ser desafiador quando a doença está restrita ao sistema nervoso central. Nestes casos, a apresentação clínica da DRP associada a disfunção neuroendócrina e visual, exames de imagem com lesões hipotalâmicas e, em alguns casos, a biópsia cerebral são fundamentais para um diagnóstico correto. é possível que o diagnóstico e tratamento precoces poderiam ter trazido melhores resultados nesses pacientes.

[Causes of hospitalization of patients with ongoing varicella in a French children hospital: evolution between 1990 and 2001]. / Motifs d'hospitalisation des patients atteints de varicelle dans un établissement pédiatrique parisien: évolution entre 1990 et 2001.

AIMS OF STUDY: To describe the characteristics of paediatric varicella in patients admitted through the emergency department (ED) of the Armand-Trousseau Children hospital in Paris and the evolution of the causes of admission during the study period. STUDY DESIGN: Retrospective longitudinal cohort study from March 1990 to February 2001. RESULTS: One hundred and eight children were hospitalized for varicella during these 11 years. Sixty per cent were under 2 years of age and all but one were immunocompetent. The first cause of admission was the presence of at least 1 complication (83,3%) classified as: cutaneous (34%), digestive (19,8,%), respiratory (17,6%), neurological (15,3%) and ENT complications (8,8%). Over 11 years, the rate of overall complications remained stable but there was an important increase in varicella cases admitted through the ED mainly due to increase in cutaneous superinfections. CONCLUSION: These data confirm the potential severity of varicella and underline the emerging problem of increasing cutaneous complications. Additional studies are necessary to address this phenomenon and to determine optimal prophylactic measures.

[Management of acute pyelonephritis in patients older than 3 months: survey conducted in 39 paediatric emergency departments of the Ile de France Region in 2004]. / Prise en charge des pyélonéphrites aiguës du nourrisson de plus de 3 mois et de l'enfant: enquête effectuée parmi les services d'urgences pédiatriques d'Ile de France en 2004.

OBJECTIVES: To describe the different modalities of ambulatory management of acute pyelonephritis in patients older than 3 months of age in paediatric emergency units of the Ile de France region in 2004. METHODS: Between October 2003 and April 2004, referents of 39 paediatric emergency units of the Ile de France region were questioned through a written questionnaire concerning the management of acute pyelonephritis: in or outpatient modalities, antibiotic regimen (molecule and route of administration), investigations and follow-up. RESULTS: Thirty-one questionnaires (79.5%) were returned and analysed. A written protocol was available in 60% of the units. Outpatient management was performed in 24/31 centres. Young age, poor clinical tolerance, urological abnormalities and social difficulties were the major contra-indications for such management. Ultrasonic echography at diagnosis (within 24 h) was performed in 50% of the units. Antibiotics were started using IV route in 18/24 units (75%) and ceftriaxone and aminoside were respectively prescribed in 100% and 29.4% of the units for a duration of 1 to 5 days before switching to the oral route. Antibiotherapy was started orally in 6 units and cefixime was chosen by 5 of them. Follow-up consultations were scheduled in 100% of the units but with various delay after initiation of the treatment. The total duration of treatment was mostly 10 days and oral prophylactic antibiotherapy was prescribed by 10/24 centres after completion of the treatment. Cystoureterography was systematically realized by 83.3% of the units. CONCLUSIONS: Despite important differences in the management of acute pyelonephritis in Ile-de-France, a majority of the units follows similar therapeutic modalities. In the absence of consensus, new recommendations are necessary concerning the management of pyelonephritis in infants and children in France.