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(Stroke. 1996;27:1417-1419.)
© 1996 American Heart Association, Inc.

Articles

Leukoencephalopathy-Related Cerebral Amyloid Angiopathy With Cystatin C Deposition

Koichi Shimode, MD, PhD; Shotai Kobayashi, MD, PhD; Kaoru Imaoka, MD; Nobuyuki Umegae, MD Atsushi Nagai, MD

the Third Internal Medicine Department, Shimane Medical University (Japan).

Correspondence to Koichi Shimode, MD, PhD, Third Internal Medicine Department, Shimane Medical University, 89-1, Enya, Izumo, Shimane 693, Japan.

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 ß-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.

Key Words: amyloid • cerebrospinal fluid • cystatins • leukoencephalopathy

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