title: Encephalopathy with amyloid angiopathy and numerous amyloid plaques with low levels of CSF Aβ1-40/Aβ1-42.
abstract: A middle-aged male suffering from encephalopathy with cerebral amyloid angiopathy (CAA) with amyloid beta (Aβ) presented with initial symptoms of transient consciousness disturbance and left visual field photophobia. Lesions with aberrantly high signal on T2-weighted magnetic resonance imaging (MRI) of the brain appeared in the right temporal lobe posterior to the occipital lobe and spread to other areas. Brain biopsy revealed Aβ deposits in vascular walls and numerous diffuse plaques in parenchymal areas. Based on MRI findings, Initial corticosteroid therapy with beta methasone effectively improved the neurological symptoms of consciousness disturbance and motor deficits. After corticosteroid therapy was stopped at 4 weeks, recurrence occurred. Additional corticosteroids did not improve clinical symptoms and the patient progressed to a bed-ridden state with a severe consciousness disturbance. Notably, CSF Aβ1-42 and CSF Aβ1-40 decreased while the recurrent encephalopathy worsened. After intense deterioration, the patient became stable. CSF Aβ1-42 increased but remained at a very low level. This case of CAA encephalopathy with apolipoprotein E ϵ4/ϵ4 homozygosity showed Aβ deposits in vascular walls and numerous diffuse plaques in parenchymal areas. The clinical course suggests that reduction of CSF Aβ1-42 and Aβ1-40 might be related to clinical deterioration in cases of encephalopathy.
authors.affiliation: Department of Neurology, Gunma University Graduate School of Medicine, Maebashi, Japan. mikeda@med.gunma-u.ac.jp
authors.forename: Masaki
authors.initials: M
authors.lastname: Ikeda
authors.forename: Kimitoshi
authors.initials: K
authors.lastname: Hirayanagi
authors.forename: Motonobu
authors.initials: M
authors.lastname: Arai
authors.forename: Satoko
authors.initials: S
authors.lastname: Kakuda
authors.forename: Kouki
authors.initials: K
authors.lastname: Makioka
authors.forename: Natsumi
authors.initials: N
authors.lastname: Furuta
authors.forename: Eriko
authors.initials: E
authors.lastname: Takai
authors.forename: Hiroo
authors.initials: H
authors.lastname: Kasahara
authors.forename: Setsuki
authors.initials: S
authors.lastname: Tsukagoshi
authors.forename: Yukio
authors.initials: Y
authors.lastname: Fujita
authors.forename: Masakuni
authors.initials: M
authors.lastname: Amari
authors.forename: Masamitsu
authors.initials: M
authors.lastname: Takatama
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Nakazato
authors.forename: Koichi
authors.initials: K
authors.lastname: Okamoto
mesh_terms: D016229:Amyloid beta-Peptides / Q000134:cerebrospinal fluid*; D001057:Apolipoproteins E / Q000235:genetics; D001706:Biopsy; D001928:Brain Diseases, Metabolic / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009778:Occipital Lobe / Q000378:metabolism / Q000473:pathology; D010446:Peptide Fragments / Q000134:cerebrospinal fluid*; D058225:Plaque, Amyloid / Q000134:cerebrospinal fluid / Q000473:pathology*; D013702:Temporal Lobe / Q000378:metabolism / Q000473:pathology
chemical_list: D016229:Amyloid beta-Peptides; D001057:Apolipoproteins E; D010446:Peptide Fragments; C078592:amyloid beta-protein (1-40); C075222:amyloid beta-protein (1-42)