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Table 1 Review of reported cases of CAA-related inflammation

From: Cerebral amyloid angiopathy-related inflammation presenting with steroid-responsive higher brain dysfunction: case report and review of the literature

Reference n Age Sex Clinical presentation MRI lesion Micro bleeds in T2*-weighted images MRI enhanced lesion Pathology treatment Outcome
Greenberg et al. 1993 [10] 1 72 F dementia headache left frontal NA (-) vasculitis NA NA
Ortiz et al. 1996 [11] 1 68 F headache right temporal/parietal NA (-) vasculitis steroid NA
Fountain et al. 1996 [12] 2 66 M fluent aphasia right hemianopia bilateral temporal/parietal NA (-) vasculitis perivasculitis steroid cyclophosphamide alive relapse (+)
   69 F headache confusion focal neurology seizure bilateral confluent multifocal NA NA vasculitis steroid cyclophosphamide died relapse (+)
Anders et al. 1997 [13] 2 70 M mental status change right frontal NA NA vasculitis NA NA
   69 M headache lethargy behavior change bilateral white matter NA (+) vasculitis NA NA
Fountain et al. 1999 [14] 1 71 M headache confusion gait difficulty left hand apraxia right temporal/parietal NA NA vasculitis cyclophosphamide alive relapse (+)
Scully et al. 2000 [15] 1 63 M behavior change ataxia bilateral white matter NA (+) perivasculitis cyclophosphamide alive
Oide et al. 2002 [16] 1 69 M dizziness dementia seizure bilateral symmetrical periventricular NA NA vasculitis (-) NA
Schwab et al. 2003 [8] 2 74 M seizure dementia headache bilateral multifocal NA (+) perivasculitis steroid alive relapse (+)
   70 F dementia headache right temporal NA (+) perivasculitis steroid alive relapse (+)
Tamargo et al. 2003 [17] 1 80 F dementia left-side hemineglect word finding difficulty bilateral left frontal right parietal NA (+) vasculitis steroid alive
Oh et al. 2004 [1] 2 80 F Headache aphasia bilateral right parietal/occipital left frontal NA (-) perivasculitis steroid alive
   77 M aphasia left temporal NA (-) vasculitis steroid alive
Safriel et al. 2004 [18] 1 49 M seizure right occipital/temporal NA (-) vasculitis steroid alive
Hashizume et al. 2004 [19] 1 65 M headache left hemianopsia left-side hemineglect right temporal/occipital NA (+) vasculitis steroid cyclophosphamide died
Harkness et al. 2004 [20] 1 72 F dementia bilateral frontal NA (-) vasculitis no specific therapy alive
Jacobs et al. 2004 [21] 1 81 F confusion Balint's syndrome agraphia right-left confusion finger anomia left-side neglect bilateral parietal/occipital NA (+) vasculitis steroid alive
Scolding et al. 2005 [3 6 69.3* M 3 F 3 encephalopathy 6 focal neurology 2 seizure 1 headache 2 NA mutifocal 1 frontal 1 diffuse white matter 1 right occipital 1 left frontal 1 bilateral confluent 1 NA (+) 1 (-) 5 vasculitis steroid 3 steroid cyclophosphamide 2 tumor resection steroid 1 alive 4 (relapse NA) died 2
Mikolaenko et al. 2006 [22] 1 50 M seizure right frontal NA (+) vasculitis surgery alive
Wong et al. 2006 [23] 1 79 F higher brain dysfunction fatigue right frontal/temporal/parietal NA NA vasculitis steroid alive relapse (+)
Kinnecom et al. 2007 [4] 1 62.3* M 9 F 3 encephalopathy 9 headache 5 seizure 7 aphasia 1 presyncope 1 NA NA NA (the presence of microbleeds are mentioned but the proportion is not mentioned) NA perivasculitis steroid 9 steroid cyclophosphamide 3 alive 11 (relapse (+) 3) died 1
Greenberg et al. 2007 [24] 1 63 M headache behavioral change cognitive change bilateral multiple NA (+) vasculitis cyclophosphamide alive relapse (+)
Marotti et al. 2007 [25] 1 57 F headache seizure bilateral frontal/temporal/insular right thalamus (+) (+) vasculitis seizure control died
McHugh et al. 2007 [26] 1 80 F confusion incontinent urine global aphasia seizure right hemianopia right hemiparesis bilateral frontal (+) (-) vasculitis perivasculitis steroid alive relapse (+)
Takada et al. 2007 [27] 1 69 F headache cognitive decline bilateral right frontal/parietal bilateral parietal/occipital (+) (-) vasculitis steroid died
Machida et al. 2008 [28] 1 69 F cognitive decline bilateral multifocal (-) (+) perivasculitis steroid alive relapse (+)
Salvarani et al. 2008 [29] 8 63* M6 F2 encephalopathy 6 focal neurology 2 headache 3 only aphasia with alexia 1 bilateral 8 multifocal NA (+) 5 (-) 3 vasculitis steroid 3 steroid cyclophosphamide 5 improved 6 died 1 worsened 1
Amick et al. 2008 [30] 1 79 F transient right sided weakness left occipital/parietal NA (-) vasculitis (-) died
Alcalay et al. 2009 [31] 1 92 F mental status change bilateral multifocal (+) (+) (-) steroid alive
Daniëls et al. 2009 [32] 1 80 F mental status change right sided hemiparesis dysphasia seizure bilateral left hemisphere right parietal/occipital (+) (-) (-) steroid alive relapse (+)
Greenberg et al. 2010 [9] 1 87 F seizure cognitive impairment bilateral multifocal (+) NA perivasculitis steroid died
Kloppenborg et al. 2010 [7] 1 74 M increased sleepiness loss of initiative seizure bilateral frontal (+) (+) perivasculitis steroid alive
Morishige et al. 2010 [33] 1 78 F motor aphasia dementia left frontal NA (+) vasculitis steroid alive
Savoiardo et al. 2010 [34] 1 76 M fatigue confusion bilateral temporal/occcipital/frontal (+) (-) (-) steroid alive
Cano et al. 2010 [35] 1 76 M transient motor aphasia transient headache bilateral temporal (+) NA (-) (-) alive
DiFrancesco et al. 2011 [36] 1 68 M memory loss mood disorder bilateral multifocal (+) (-) NA steroid alive
Chung et al. 2011 [37] 3 83 F seizure bilateral multifocal NA NA vasculitis steroid died
   forties F headache mild hemiparesis sensory loss right parietal/occipital (+) NA vasculitis steroid alive haemorrhage (+)
   72 M seizure left-side neglect left hemianopia bilateral multifocal NA NA vasculitis perivasculitis steroid cyclophosphamide died
our case 1 56 M Seizure sensory disturbance higher brain dysfunction right parietal (-) (+) perivasculitis steroid alive relapse (+)
  1. From the literature, we extracted the cases of CAA-related inflammation in which an MRI was evaluated. If autopsy or biopsy was examined, the cases without inflammation were excluded. All cases satisfy the diagnostic criteria of definite or probable CAA-related inflammation proposed by Chung et al. [37]. In 64 cases, 10 presented with higher brain dysfunction without encephalopathy or dementia (15.3%). The most frequent symptom was aphasia (6 cases: 9.3%), followed by hemineglect (2 cases: 3.1%). One case besides the current presented with various higher brain dysfunction without mental change or dementia [23]. In these 10 cases with higher brain dysfunction, MRI lesions and the presence of leptomeningeal enhancement were inconsistent. Thirteen cases were examined with MRI with an echo gradient sequence, and microhemorrhages were not seen in 2 cases, including our case (13.3%).
  2. The leptomeningeal enhancement status of 42 patients was mentioned, and the clinical courses of 39 patients were described. Only one patient among 19 patients with leptomeningeal enhancement died (5.3%); however, 7 of 20 patients without enhancement died (35%), suggesting that leptomeningeal enhancement might be a factor in good prognosis. *: calculated mean
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