Subacute diencephalic angioencephalopathy: biopsy diagnosis and radiological features of a rare entity.
Clin Neurol Neurosurg
; 103(3): 160-7, 2001 Oct.
Article
em En
| MEDLINE
| ID: mdl-11532556
ABSTRACT
Subacute diencephalic angioencephalopathy (SDAE) is a rare and fatal disease of unknown etiology that involves the thalami bilaterally. To date, there have been four cases reported, in which the diagnosis was established only after post mortem examination of the brain. We report two male patients, ages 69 and 41 years, who presented with progressive dementia and somnolence. Radiological evaluation revealed enhancing lesions involving both thalami. The differential diagnosis included a number of neoplastic, inflammatory and vascular processes. In both cases, pathological evaluation of biopsy specimens suggested the diagnosis of SDAE. Despite supportive care, the disease progressed rapidly and both patients died within weeks after initial presentation. The diagnosis was confirmed at autopsy in both cases. SDAE is a rare cause of bithalamic disease that can be mistaken for a neoplasm as well as a number of conditions that necessitate different treatment choices. The histopathological findings can establish the diagnosis when combined with radiological and clinical information. This report emphasizes the utility of stereotactic biopsy in early diagnosis of SDAE.
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Base de dados:
MEDLINE
Assunto principal:
Doenças Talâmicas
/
Encefalopatias
/
Diencéfalo
Tipo de estudo:
Diagnostic_studies
/
Screening_studies
Limite:
Adult
/
Aged
/
Humans
/
Male
Idioma:
En
Ano de publicação:
2001
Tipo de documento:
Article