Table 1 Review of reported cases of CAA-related inflammation
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 (+) |