Skip to main content

Table 1 Studies concerning immunosuppresive treatment of CAA encephalopathy

From: Steroid responsive encephalopathy in cerebral amyloid angiopathy: a case report and review of evidence for immunosuppressive treatment

Author n Age Pathology Radiology Therapy Clinical improvement Radiological improvement Follow-up Clinical features Comments
Ginsberg 1988 [10] 1 73 TGA Confluent Dx, Pn Yes Yes >1 year Gait disturbance  
Mandybur 1992 [11] 1 62 TGA Mass CP, Pn Yes Yes Death 8 months Encephalopathy Focal neurology Hallucinations Remarkable pathological improvement lesions post-mortem compared to initial biopsy
Osumi 1995 [4] 1 59 ? Mass CS No ? Death 5 months Focal neurology
Headaches
Seizures
 
Silbert 1995 [12] 1 74 ? Confluent Dx No No Death 6 weeks Headache
Seizures
 
Fountain 1996 [13] 1 66 TGA Confluent CP, Dx, Pn, No Partial 20 months Encephalopathy
Headaches
Seizures
 
Fountain 1996 [13] 1 69 TGA Confluent CP, Dx, Pn, Partial Partial Death 6 months Encephalopathy
Headaches
Seizures
Relapse
Ortiz 1996 [14] 1 68 PVI Mass Dx, Pn Yes Yes ? Encephalopathy
Gait disturbance Headaches
 
Masson 1998 [15] 1 64 PVI Confluent CP, Pn Yes No 15 months Encephalopathy
Headaches
 
Fountain 1999 [16] 1 71 PVI, TGA Confluent CP Yes Yes 22 months Encephalopathy
Gait disturbance
Headaches
Relapse after stop CP
Streichenberger 1999 [17] 1 67 TGA Mass/Confluent CS Yes Yes Death 1 month Headaches
Encephalopathy
 
Hoshi 2000 [18] 1 65 - (after treatment) Recurrent
ICH
Pn Yes NA 6 months Focal neurology  
Schwab 2003 [19] 1 74 PVI/TGA Mass Dx 1m, Pn Yes ? 12 months Encephalopathy
Headaches
Seizures
 
Schwab 2003 [19] 1 70 PVI//TGA Mass Pn, CP Partial Yes 18 months Encephalopathy
Headaches
Seizures
 
Oh 2004 [20] 1 80 PVI Confluent Dx, Pn Yes Yes 8 months Encephalopathy
Focal Neurology
Seizures
1 patient with no therapy excluded
Oh 2004 [20] 1 77 TGA Confluent Dx, Pn Yes Yes 6 weeks Encephalopathy
Focal Neurology
Seizures
1 patient with no therapy excluded
Safriel 2004 [5] 1 49 TGA Mass Dx, tap 6 weeks Yes Partial 9 months Seizures  
Scolding 2005 [21] 7 69* TGA Confluent Pn, Dx, CP 43% ? 58 months* Encephalopathy
Focal Neurology
Headaches
Seizures
2 patients excluded because of mass resection as therapy
Kinnecom 2007 [6] 12 63.2 ± 10 PVI Confluent CS, CP 83% 83% 47 months* Encephalopathy Headache
Seizures
25% relapse, 33% died
McHugh 2007 [9] 1 80 PVI, TGA Confluent Pn Yes Yes 24 months Encephalopathy
Focal Neurology
Seizures
 
Machida 2008 [22] 1 69 PVI Confluent Dx, Pn Yes Yes 12 months Encephalopathy
Focal neurology
Relapsing/remitting
Salvarini 2008 [23] 8 63 TGA Confluent Pn, CP 75% 100% 24 months* Encephalopathy
Focal Neurology
Headaches
25% relapse, both after discontinuation of treatment
  1. CS = corticosteroid (not otherwise specified), CP = Cyclophospamid, Dx = dexamethasone, Pn = prednisone, PVI = perivascular inflammation, TGA = transmural (non)-granulomatous angiitis
  2. Encephalopathy is characterised as diffuse cognitive disturbances, somnolence and apathy, focal neurology is characterised as hemiparesis, hemihyesthesia, aphasia or hemianopsy.
  3. * calculated mean