Clinical diagnosis and successful treatment of inflammatory cerebral amyloid angiopathy.
BACKGROUND
Cerebral amyloid angiopathy is a major cause of lobar hemorrhage in older adults, and of microvascular ischemic disease. The rarest form of this disease is an inflammatory form causing seizures. It is important to recognize because the patients usually respond to a brief course of immunosuppression.


METHODS
Case report.


RESULTS
A 66-year-old man developed gradual cognitive decline, insidiously increasing headaches, and then had a likely seizure. MRI showed diffuse white matter edema, and innumerable superficial microhemorrhages characteristic of amyloid angiopathy. He was empirically treated with oral prednisone and an anticonvulsant. His symptoms improved and the white matter edema resolved over several months. ApoE genotype was 4/4, which is commonly found in inflammatory amyloid angiopathy.


CONCLUSIONS
Inflammatory cerebral amyloid angiopathy can be clinically diagnosed and treated without brain biopsy. Clinical diagnosis is important because of the good response to a brief course of steroids in most cases.
Department of Neurology and Neurological Sciences, Feinberg School of Medicine of Northwestern University, 710 North Lake Shore Drive, Abbott Hall 11th Floor, Chicago, IL 60611, USA. r-bernstein@northwestern.edu
Richard A
RA
Bernstein
Michael
M
Gibbs
H
H
Hunt Batjer
D000368:Aged; D000927:Anticonvulsants / Q000627:therapeutic use; D053327:Apolipoprotein E4 / Q000235:genetics; D001929:Brain Edema / Q000175:diagnosis / Q000188:drug therapy; D028243:Cerebral Amyloid Angiopathy, Familial / Q000175:diagnosis* / Q000188:drug therapy* / Q000235:genetics; D038524:Diffusion Magnetic Resonance Imaging; D004359:Drug Therapy, Combination; D005838:Genotype; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D000077287:Levetiracetam; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D009778:Occipital Lobe / Q000187:drug effects / Q000473:pathology; D010889:Piracetam / Q000031:analogs & derivatives / Q000627:therapeutic use; D011241:Prednisone / Q000627:therapeutic use*; D013702:Temporal Lobe / Q000187:drug effects / Q000473:pathology
D000927:Anticonvulsants; D053327:Apolipoprotein E4; D007166:Immunosuppressive Agents; D000077287:Levetiracetam; D011241:Prednisone; D010889:Piracetam
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