|
| Table 3: |
| Benign Angiopathy of the Central Nervous System: Patient Characteristics (n=16) |
| Demographic and Clinical Symptoms |
Diagnostic Studies |
| Mean
age, years (range) |
40
(10-66)
|
Severe
CSF abnormalities*, n (%) |
1
(7)
|
| Female/male,
n |
13/3
|
Abnormal
MRI, n (%) |
10
(77)
|
| Mean
prodrome, days (range) |
25
(0-155)
|
Positive
brain biopsy, n (%) |
0
(0)
|
| Mean
follow-up, months (range) |
35
(0-128)
|
Positive
cerebral angiography, n (%) |
16
(100)
|
| Headaches,
n (%) |
14
(88)
|
|
| Focal
symptoms, n (%) |
10
(63)
|
| Diffuse
symptoms, n (%) |
7
(44)
|
| Constitutional
symptoms, n (%) |
1
(6)
|
*
Protein >70 mg/dl or white blood cell count >10/mm3
CSF = cerebrospinal fluid; MRI = magnetic resonance imaging
Adapted from reference 8
with permission. |
|
| Table
4: |
|
Comparison
of Clinical and Diagnostic Characteristics of Benign Angiopathy of the
Central Nervous System and Granulomatous Angiitis of the Central Nervous
System
|
| |
BACNS
|
GACNS
|
| Patients |
Female-predominant
|
Male-predominant
|
| Disease
onset |
Acute
|
Chronic
|
| Clinical
course |
Monophasic
|
Recurrent
|
| CSF
findings |
Normal
|
Abnormal
|
| Abnormal
angiography |
100%
|
15%
|
| Diagnostic
modality |
Angiography
|
CNS
biopsy
|
| Treatment |
GC
and Ca-Blockers
|
GC
and CYC
|
| Outcomes |
Excellent*
|
Good†
|
CSF
= cerebrospinal fluid; CNS = central nervous system; GC = glucocorticoids;
CYC = cyclophosphamide; Ca-Blockers = calcium channel blockers
* 100% of BACNS patients had mild to no disability on the Barthel Index15
at follow-up
† 86% of GACNS patients showed mild to no disability and 14% moderate
to severe disability on the Barthel Index15
at follow-up |
|
 |
| Cerebral
angiography of a patient with BACNS at diagnosis (left) and after 1 month
of therapy (right). Note the multiple areas of stenosis (white arrow) and
dilation (black arrow) in multiple vessels of the M2 branch of the middle
cerebral artery, and their resolution after treatment. Adapted from reference
8 with permission. |
| Figure 2 |
|
 |
Clinical
algorithm: The approach to primary angiitis of the CNS.
Adapted from Calabrese LH, Advances in Immunotherapy, with permission. |
| Figure 3A |
|
 |
Clinical
algorithm: The approach to primary angiitis of the CNS.
Adapted from Calabrese LH, Advances in Immunotherapy, with permission. |
| Figure 3B |
|
 |
Typical
angiographic findings in a patient with CNS vasculitis. Arrows point to
areas alternating stenosis and ectasia.
Adapted from Hajj-Ali, et al. Crit Care Clin. 2002;18:897-914. |
| Figure 4 |
|
|
