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P1‐192: Variable clinical and neuroimaging features in patients with cerebral amyloid angiopathy–related inflammation
Author(s) -
Tokutake Takayoshi,
Akaiwa Yasuhisa,
Ishiguro Takanobu,
Kasuga Kensaku,
Nishizawa Masatoyo,
Ikeuchi Takeshi
Publication year - 2015
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1016/j.jalz.2015.06.391
Subject(s) - cerebral amyloid angiopathy , medicine , hyperintensity , neuroimaging , fluid attenuated inversion recovery , cognitive decline , pathology , magnetic resonance imaging , white matter , susceptibility weighted imaging , pittsburgh compound b , amyloid (mycology) , inflammation , pathophysiology , cognitive impairment , radiology , dementia , disease , psychiatry
Background: Cerebral amyloid angiopathy-related inflammation (CAA-ri) is clinically characterized by variable combinations of subacute confusion, cognitive decline, headache, seizure and focal neurological deficits. MRI scans show cortical/subcortical multiple microbleeds which are demonstrated by T2*-gradient echo (GRE) or susceptibility-weighted imaging (SWI) as well as asymmetric white matter hyperintensities detected by T2-weighted or FLAIR image. CAA-ri is thought to be caused by inflammatory response to b amyloid (Ab) in the walls of blood vessels. We here characterized clinical and neuroimaging features in patients with CAA-ri. In addition, biomarkers including Ab42, tau, and p-tau in CSF and anti-Ab antibodies in serum were also investigated to explore the pathophysiology of CAA-ri. Methods: We recruited 8 patients with CAA-ri (male/female 1⁄4 6/2). Diagnosis of probable CAA-ri was made on the basis of the proposed diagnostic criteria (Chung et al. 2011). MRI (1.5 Tesla) scans were conducted. To detect microbleeds, MRI scans using T2*-GRE or SWI were performed. Three patients had [11C]PIB amyloid PET imaging and one patient had [11C](R)-PK11195 PET scan used as a marker of in vivomicroglial activation. Levels of Ab42, tau, p-tau in CSF were determined using xMAP technology. Anti-Ab antibodies in the serum were measured by immunoprecipitation assay. Results:The mean age at onset was 72 6 10 years (range:52-86 years). Two of eight e patients were positive for the APOE ε4/ε4 genotype. Five patients presented with relatively mild cognitive decline and three patients presented with severe cognitive decline. Four of eight patients were treated with corticosteroids. Three of four patients had clinical and neuroimaging improvement by immunosuppressive treatment. There was a decrease of PiB uptake in the affected white matter in two patients. One patient showed increased uptake of [11C](R)PK11195 in the correponding region. The levels of anti-Ab antibodies in serum of CAA-ri patients were significantly elevated compared to controls. Conclusions: Clinical and neuroimaging findings in patients with CAA-ri were highly variable. Decreased Ab accumulation and increased activated microglia in the affected white matter suggest that inflammation occurring in CAA-ri may enhance the clearance of parenchymal Ab deposit.