Clinical Diagnosis and Successful Treatment of Inflammatory Cerebral Amyloid Angiopathy
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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.
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.
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.
KeywordsCerebral amyloid angiopathy Amyloid-related inflammation Seizures Microhemorrhages Lobar hemorrhage Leukoencephalopathy