Leukoencephalopathy-related cerebral amyloid angiopathy with cystatin C deposition.

Abstract

BACKGROUND:

We have described sporadic cases of cerebral amyloid angiopathy with cerebral hemorrhage showing a low cystatin C level in the cerebrospinal fluid detected by enzyme-linked immunosorbent assay. Recently, several cases of leukoencephalopathy in patients with cerebral amyloid angiopathy have been reported. We describe a sporadic case of leukoencephalopathy with cystatin C-type cerebral amyloid angiopathy diagnosed during life by enzyme-linked immunosorbent assay.

CASE DESCRIPTION:

A 74-year-old man who had suffered from progressive dementia for 3 years was admitted with right hemiparesis, dysarthria, and ataxia. MRI revealed pontine infarction and multiple lacunar state with leukoaraiosis. We suspected cystatin C-type cerebral amyloid angiopathy because of the low level of cystatin C in the cerebrospinal fluid. The patient died of sepsis 3 months later, and the presence of leukoencephalopathy with cerebral amyloid angiopathy was confirmed by autopsy. Immunohistological examination disclosed cystatin C and beta-protein deposition in amyloid structures of the cortical cerebral arteries.

CONCLUSIONS:

Measurement of cystatin C in the cerebrospinal fluid by enzyme-linked immunosorbent assay is a useful method of diagnosing leukoencephalopathy related to sporadic cystatin C-type cerebral amyloid angiopathy.

• Authors: K Shimode, S Kobayashi, K Imaoka, N Umegae, A Nagai
• Journal: Stroke (Stroke) Vol. 27 Issue 8 Pg. 1417-9 (Aug 1996) ISSN: 0039-2499 [Print] United States
• PMID: 8711812 (Publication Type: Case Reports, Journal Article)

Chemical References

• Amyloid
• CST3 protein, human
• Cystatin C
• Cystatins
• Cysteine Proteinase Inhibitors

Topics

• Aged
• Amyloid (analysis)
• Cerebral Amyloid Angiopathy (complications)
• Cerebral Arteries (chemistry)
• Cerebral Cortex (blood supply, pathology)
• Cystatin C
• Cystatins (cerebrospinal fluid)
• Cysteine Proteinase Inhibitors (cerebrospinal fluid)
• Fatal Outcome
• Humans
• Male