Leukoencephalopathy-related cerebral amyloid angiopathy with cystatin C deposition.
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.


METHODS
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.
Shimode
K
K
Third Internal Medicine Department, Shimane Medical University, Japan.
Kobayashi
S
S
Imaoka
K
K
Umegae
N
N
Nagai
A
A
D000368:Aged; D000682:Amyloid / Q000032:analysis; D016657:Cerebral Amyloid Angiopathy / Q000150:complications*; D002536:Cerebral Arteries / Q000737:chemistry; D002540:Cerebral Cortex / Q000098:blood supply / Q000473:pathology; D055316:Cystatin C; D015891:Cystatins / Q000134:cerebrospinal fluid*; D015853:Cysteine Proteinase Inhibitors / Q000134:cerebrospinal fluid; D017809:Fatal Outcome; D006801:Humans; D008297:Male
D000682:Amyloid; C525030:CST3 protein, human; D055316:Cystatin C; D015891:Cystatins; D015853:Cysteine Proteinase Inhibitors