Rapidly progressive dementia due to neurosarcoidosis

Demência rapidamente progressiva causada por neurosarcoidose

ABSTRACT

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.

Key words:
neurosarcoidosis; rapidly progressive dementia; diencephalic amnesia; primary CNS vasculitis.

RESUMO

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.

Palavras-chave:
neurosarcoidose; demência rapidamente progressiva; amnésia diencefálica; vasculite primária do SNC.

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Publication Dates

• Publication in this collection
  Dec 2013

History

• Received
  15 Aug 2013
• Accepted
  30 Sept 2013