Subacute brainstem angioencephalopathy: a case report and review of the literature

A previously healthy 69-year-old man developed a progressive neurological illness with bulbar signs and ataxic paraparesis. Repeated MRI examinations revealed a large space occupying lesion in the lower brain stem with patchy contrast enhancement. MRI angiography was unremarkable and CSF had normal...

Full description

Saved in:
Bibliographic Details
Published in:Journal of the neurological sciences 2003-04, Vol.208 (1), p.101-104
Main Authors: Rauschka, Helmut, Retzl, Johann, Baumhackl, Ulf, Bankl, Hans Christian, Salomonowitz, Erich, Schmidbauer, Manfred
Format: Article
Language:English
Subjects:
Aged
Angioencephalopathy
Biological and medical sciences
Brain Neoplasms
Brain Stem - pathology
Brainstem
Cerebral Veins - pathology
Cerebrovascular Disorders
Edema
Fibromuscular Dysplasia
Humans
Magnetic Resonance Imaging
Medical sciences
Necrosis
Neurology
Pathology
Vascular diseases and vascular malformations of the nervous system
Vasculitis
Veins
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:A previously healthy 69-year-old man developed a progressive neurological illness with bulbar signs and ataxic paraparesis. Repeated MRI examinations revealed a large space occupying lesion in the lower brain stem with patchy contrast enhancement. MRI angiography was unremarkable and CSF had normal cell count but raised protein content. A brainstem tumor was suspected and a course of intravenous glucocorticosteroids was started. No improvement occurred and the patient died of pneumonia 11 weeks after onset. Neuropathology revealed confluent areas of complete or incomplete necrosis with marked edema in the lower brainstem. Predominantly venous meningeal vessels of the brainstem showed extensive fibromuscular thickening of all layers with luminal narrowing. In addition, intramural mononuclear infiltration was found. With the exception of localisation, this case exhibits all pathologic features of subacute diencephalic angioencephalopathy (SDAE), a rare fatal disease of unknown aetiology. In addition, the clinical features of typical age, male sex, disease duration and raised CSF proteins are shared. A common disease entity is suggested and the pathogenetic relevance of inflammation and venous outflow obstruction is discussed.
ISSN:0022-510X
1878-5883
DOI:10.1016/S0022-510X(02)00395-7