Subacute diencephalic angioencephalopathy: Biopsy diagnosis and radiological features of a rare entity

Tarik Tihan, Peter C. Burger, Martin Pomper, Oscar Sanchez, Muhammad Ramzan, Charles G. Eberhart, Calvin Hansen, Thomas W. Smith

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
Pages (from-to)160-167
Number of pages8
JournalClinical Neurology and Neurosurgery
Volume103
Issue number3
DOIs
StatePublished - Jan 1 2001

Keywords

  • Angioencephalopathy
  • Dementia
  • Thalamic degeneration

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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