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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