Vascular disease affects many different arterial beds throughout the body. Yet the brain is susceptible to several vascular disorders that either are not found in other parts of the body or when found are much less likely to cause clinical syndromes in other organs. This specific vulnerability of the brain may be explained by structural and functional differences between the vessels of the brain and those of vessels in other parts of the body. In this review, we focus on how cerebrovascular anatomy and physiology may make the brain and its vessels more susceptible to unique vascular pathologies. To highlight these differences, we use our knowledge of five diseases and syndromes that most commonly manifest in the intracranial vasculature. For each, we identify characteristics of the intracranial arteries that make them susceptible to these diseases, while noting areas of uncertainty requiring further research.

1.
Schaeffer
S
,
Iadecola
C
.
Revisiting the neurovascular unit
.
Nat Neurosci
.
2021
;
24
(
9
):
1198
209
.
2.
Bell
AH
,
Miller
SL
,
Castillo-Melendez
M
,
Malhotra
A
.
The neurovascular unit: effects of brain insults during the perinatal period
.
Front Neurosci
.
2020
;
13
:
1452
.
3.
Roth
W
,
Morgello
S
,
Goldman
J
,
Mohr
JP
,
Elkind
MS
,
Marshall
RS
,
.
Histopathological differences between the anterior and posterior brain arteries as a function of aging
.
Stroke
.
2017
;
48
(
3
):
638
44
.
4.
Ratinov
G
.
Extradural intracranial portion of carotid artery; a clinicopathologic study
.
Arch Neurol
.
1964
;
10
:
66
73
.
5.
Gutierrez
J
,
Rosoklija
G
,
Murray
J
,
Chon
C
,
Elkind
MS
,
Goldman
J
,
.
A quantitative perspective to the study of brain arterial remodeling of donors with and without HIV in the Brain Arterial Remodeling Study (BARS)
.
Front Physiol
.
2014
;
5
:
56
.
6.
Ritz
K
,
Denswil
NP
,
Stam
OC
,
van Lieshout
JJ
,
Daemen
MJ
.
Cause and mechanisms of intracranial atherosclerosis
.
Circulation
.
2014
;
130
(
16
):
1407
14
.
7.
Etminan
N
,
Rinkel
GJ
.
Unruptured intracranial aneurysms: development, rupture and preventive management
.
Nat Rev Neurol
.
2016
;
12
(
12
):
699
713
.
8.
Humphrey
JD
,
Taylor
CA
.
Intracranial and abdominal aortic aneurysms: similarities, differences, and need for a new class of computational models
.
Annu Rev Biomed Eng
.
2008
;
10
:
221
46
.
9.
Aydin
F
.
Do human intracranial arteries lack vasa vasorum? A comparative immunohistochemical study of intracranial and systemic arteries
.
Acta Neuropathol
.
1998
;
96
(
1
):
22
8
.
10.
Hamner
JW
,
Tan
CO
.
Relative contributions of sympathetic, cholinergic, and myogenic mechanisms to cerebral autoregulation
.
Stroke
.
2014
;
45
(
6
):
1771
7
.
11.
Takebayashi
S
,
Kaneko
M
.
Electron microscopic studies of ruptured arteries in hypertensive intracerebral hemorrhage
.
Stroke
.
1983
;
14
(
1
):
28
36
.
12.
Feske
SK
.
Posterior reversible encephalopathy syndrome: a review
.
Semin Neurol
.
2011
;
31
(
2
):
202
15
.
13.
Fugate
JE
,
Rabinstein
AA
.
Posterior reversible encephalopathy syndrome: clinical and radiological manifestations, pathophysiology, and outstanding questions
.
Lancet Neurol
.
2015
;
14
(
9
):
914
25
.
14.
Fugate
JE
,
Claassen
DO
,
Cloft
HJ
,
Kallmes
DF
,
Kozak
OS
,
Rabinstein
AA
.
Posterior reversible encephalopathy syndrome: associated clinical and radiologic findings
.
Mayo Clin Proc
.
2010
;
85
(
5
):
427
32
.
15.
Edvinsson
L
,
Owman
C
,
Siesjö
B
.
Physiological role of cerebrovascular sympathetic nerves in the autoregulation of cerebral blood flow
.
Brain Res
.
1976
;
117
(
3
):
519
23
.
16.
ter Laan
M
,
van Dijk
JM
,
Elting
JW
,
Staal
MJ
,
Absalom
AR
.
Sympathetic regulation of cerebral blood flow in humans: a review
.
Br J Anaesth
.
2013
;
111
(
3
):
361
7
.
17.
Beausang-Linder
M
,
Bill
A
.
Cerebral circulation in acute arterial hypertension: protective effects of sympathetic nervous activity
.
Acta physiol Scand
.
1981
;
111
(
2
):
193
9
.
18.
Singhal
AB
,
Topcuoglu
MA
,
Fok
JW
,
Kursun
O
,
Nogueira
RG
,
Frosch
MP
,
.
Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison
.
Ann Neurol
.
2016
;
79
(
6
):
882
94
.
19.
Ducros
A
.
Reversible cerebral vasoconstriction syndrome
.
Lancet Neurol
.
2012
;
11
(
10
):
906
17
.
20.
Calado
S
,
Vale-Santos
J
,
Lima
C
,
Viana-Baptista
M
.
Postpartum cerebral angiopathy: vasospasm, vasculitis or both
.
Cerebrovasc Dis
.
2004
;
18
(
4
):
340
1
.
21.
Chen
SP
,
Yang
AC
,
Fuh
JL
,
Wang
SJ
.
Autonomic dysfunction in reversible cerebral vasoconstriction syndromes
.
J Headache Pain
.
2013
;
14
:
94
.
22.
Schwedt
TJ
,
Matharu
MS
,
Dodick
DW
.
Thunderclap headache
.
Lancet Neurol
.
2006
;
5
(
7
):
621
31
.
23.
Chen
SP
,
Fuh
JL
,
Wang
SJ
,
Tsai
SJ
,
Hong
CJ
,
Yang
AC
.
Brain-derived neurotrophic factor gene Val66Met polymorphism modulates reversible cerebral vasoconstriction syndromes
.
PLoS One
.
2011
;
6
(
3
):
e18024
.
24.
Chen
SP
,
Wang
YF
,
Huang
PH
,
Chi
CW
,
Fuh
JL
,
Wang
SJ
.
Reduced circulating endothelial progenitor cells in reversible cerebral vasoconstriction syndrome
.
J Headache Pain
.
2014
;
15
:
82
.
25.
Mawet
J
,
Boukobza
M
,
Franc
J
,
Sarov
M
,
Arnold
M
,
Bousser
MG
,
.
Reversible cerebral vasoconstriction syndrome and cervical artery dissection in 20 patients
.
Neurology
.
2013
;
81
(
9
):
821
4
.
26.
John
S
,
Hajj-Ali
RA
,
Min
D
,
Calabrese
LH
,
Cerejo
R
,
Uchino
K
.
Reversible cerebral vasoconstriction syndrome: is it more than just cerebral vasoconstriction
.
Cephalalgia
.
2015
;
35
(
7
):
631
4
.
27.
Mukerji
SS
,
Buchbinder
BR
,
Singhal
AB
.
Reversible cerebral vasoconstriction syndrome with reversible renal artery stenosis
.
Neurology
.
2015
;
85
(
2
):
201
2
.
28.
Chabriat
H
,
Joutel
A
,
Dichgans
M
,
Tournier-Lasserve
E
,
Bousser
MG
.
Cadasil
.
Lancet Neurol
.
2009
;
8
(
7
):
643
53
.
29.
Dong
Y
,
Hassan
A
,
Zhang
Z
,
Huber
D
,
Dalageorgou
C
,
Markus
HS
.
Yield of screening for CADASIL mutations in lacunar stroke and leukoaraiosis
.
Stroke
.
2003
;
34
(
1
):
203
5
.
30.
Coupland
K
,
Lendahl
U
,
Karlström
H
.
Role of NOTCH3 mutations in the cerebral small vessel disease cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
.
Stroke
.
2018
;
49
(
11
):
2793
800
.
31.
Peters
N
,
Opherk
C
,
Bergmann
T
,
Castro
M
,
Herzog
J
,
Dichgans
M
.
Spectrum of mutations in biopsy-proven CADASIL: implications for diagnostic strategies
.
Arch Neurol
.
2005
;
62
(
7
):
1091
4
.
32.
Joutel
A
,
Andreux
F
,
Gaulis
S
,
Domenga
V
,
Cecillon
M
,
Battail
N
,
.
The ectodomain of the Notch3 receptor accumulates within the cerebrovasculature of CADASIL patients
.
J Clin Invest
.
2000
;
105
(
5
):
597
605
.
33.
Joutel
A
,
Favrole
P
,
Labauge
P
,
Chabriat
H
,
Lescoat
C
,
Andreux
F
,
.
Skin biopsy immunostaining with a Notch3 monoclonal antibody for CADASIL diagnosis
.
Lancet
.
2001
;
358
(
9298
):
2049
51
.
34.
Kalimo
H
,
Ruchoux
MM
,
Viitanen
M
,
Kalaria
RN
.
CADASIL: a common form of hereditary arteriopathy causing brain infarcts and dementia
.
Brain Pathol
.
2002
;
12
(
3
):
371
84
.
35.
Ruchoux
MM
,
Maurage
CA
.
CADASIL: cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
.
J Neuropathol Exp Neurol
.
1997
;
56
(
9
):
947
64
.
36.
Miao
Q
,
Paloneva
T
,
Tuominen
S
,
Poyhonen
M
,
Tuisku
S
,
Viitanen
M
,
.
Fibrosis and stenosis of the long penetrating cerebral arteries: the cause of the white matter pathology in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
.
Brain Pathol
.
2004
;
14
(
4
):
358
64
.
37.
Ghosh
M
,
Balbi
M
,
Hellal
F
,
Dichgans
M
,
Lindauer
U
,
Plesnila
N
.
Pericytes are involved in the pathogenesis of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
.
Ann Neurol
.
2015
;
78
(
6
):
887
900
.
38.
Ruchoux
MM
,
Guerouaou
D
,
Vandenhaute
B
,
Pruvo
JP
,
Vermersch
P
,
Leys
D
.
Systemic vascular smooth muscle cell impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
.
Acta Neuropathol
.
1995
;
89
(
6
):
500
12
.
39.
Kamada
F
,
Aoki
Y
,
Narisawa
A
,
Abe
Y
,
Komatsuzaki
S
,
Kikuchi
A
,
.
A genome-wide association study identifies RNF213 as the first moyamoya disease gene
.
J Hum Genet
.
2011
;
56
(
1
):
34
40
.
40.
Scott
RM
,
Smith
ER
.
Moyamoya disease and moyamoya syndrome
.
N Engl J Med
.
2009
;
360
(
12
):
1226
37
.
41.
Hosoda
Y
,
Ikeda
E
,
Hirose
S
.
Histopathological studies on spontaneous occlusion of the circle of Willis (cerebrovascular moyamoya disease)
.
Clin Neurol Neurosurg
.
1997
;
99
(
Suppl 2
):
S203
8
.
42.
Yamashita
M
,
Oka
K
,
Tanaka
K
.
Histopathology of the brain vascular network in moyamoya disease
.
Stroke
.
1983
;
14
(
1
):
50
8
.
43.
Takagi
Y
,
Kikuta
K
,
Nozaki
K
,
Hashimoto
N
.
Histological features of middle cerebral arteries from patients treated for moyamoya disease
.
Neurol Med Chir
.
2007
;
47
(
1
):
1
4
.
44.
Kono
S
,
Oka
K
,
Sueishi
K
.
Histopathologic and morphometric studies of leptomeningeal vessels in moyamoya disease
.
Stroke
.
1990
;
21
(
7
):
1044
50
.
45.
Ikeda
E
.
Systemic vascular changes in spontaneous occlusion of the circle of Willis
.
Stroke
.
1991
;
22
(
11
):
1358
62
.
46.
Togao
O
,
Mihara
F
,
Yoshiura
T
,
Tanaka
A
,
Kuwabara
Y
,
Morioka
T
,
.
Prevalence of stenoocclusive lesions in the renal and abdominal arteries in moyamoya disease
.
AJR Am J Roentgenol
.
2004
;
183
(
1
):
119
22
.
47.
Yamada
I
,
Himeno
Y
,
Matsushima
Y
,
Shibuya
H
.
Renal artery lesions in patients with moyamoya disease: angiographic findings
.
Stroke
.
2000
;
31
(
3
):
733
7
.
48.
Nagel
MA
,
Gilden
D
.
Developments in varicella zoster virus vasculopathy
.
Curr Neurol Neurosci Rep
.
2016
;
16
(
2
):
12
.
49.
Askalan
R
,
Laughlin
S
,
Mayank
S
,
Chan
A
,
MacGregor
D
,
Andrew
M
,
.
Chickenpox and stroke in childhood: a study of frequency and causation
.
Stroke
.
2001
;
32
(
6
):
1257
62
.
50.
Braun
KP
,
Bulder
MM
,
Chabrier
S
,
Kirkham
FJ
,
Uiterwaal
CS
,
Tardieu
M
,
.
The course and outcome of unilateral intracranial arteriopathy in 79 children with ischaemic stroke
.
Brain
.
2009
;
132
(
Pt 2
):
544
57
.
51.
Heininger
U
,
Seward
JF
.
Varicella
.
Lancet
.
2006
;
368
(
9544
):
1365
76
.
52.
Nagel
MA
,
Gilden
D
.
Update on varicella zoster virus vasculopathy
.
Curr Infect Dis Rep
.
2014
;
16
(
6
):
407
.
53.
Nagel
MA
,
Cohrs
RJ
,
Mahalingam
R
,
Wellish
MC
,
Forghani
B
,
Schiller
A
,
.
The varicella zoster virus vasculopathies: clinical, CSF, imaging, and virologic features
.
Neurology
.
2008
;
70
(
11
):
853
60
.
54.
Reshef
E
,
Greenberg
SB
,
Jankovic
J
.
Herpes zoster ophthalmicus followed by contralateral hemiparesis: report of two cases and review of literature
.
J Neurol Neurosurg Psychiatry
.
1985
;
48
(
2
):
122
7
.
55.
Mayberg
M
,
Langer
RS
,
Zervas
NT
,
Moskowitz
MA
.
Perivascular meningeal projections from cat trigeminal ganglia: possible pathway for vascular headaches in man
.
Science
.
1981
;
213
(
4504
):
228
30
.
56.
Saito
K
,
Moskowitz
MA
.
Contributions from the upper cervical dorsal roots and trigeminal ganglia to the feline circle of Willis
.
Stroke
.
1989
;
20
(
4
):
524
6
.
57.
Nagel
MA
,
Traktinskiy
I
,
Azarkh
Y
,
Kleinschmidt-DeMasters
B
,
Hedley-Whyte
T
,
Russman
A
,
.
Varicella zoster virus vasculopathy: analysis of virus-infected arteries
.
Neurology
.
2011
;
77
(
4
):
364
70
.
58.
Amlie-Lefond
C
,
Kleinschmidt-DeMasters
BK
,
Mahalingam
R
,
Davis
LE
,
Gilden
DH
.
The vasculopathy of varicella-zoster virus encephalitis
.
Ann Neurol
.
1995
;
37
(
6
):
784
90
.
59.
Hall
S
,
Carlin
L
,
Roach
ES
,
McLean
WT
Jr.
.
Herpes zoster and central retinal artery occlusion
.
Ann Neurol
.
1983
;
13
(
2
):
217
8
.
60.
Tezcan
ME
,
Teksut
TK
,
Onal
AB
,
Oztürk
MA
.
Reactivated varicella zoster virus may cause peripheral arterial thrombosis
.
J Rheumatol
.
2010
;
37
(
8
):
1785
6
.
61.
Massano
J
,
Ferreira
D
,
Toledo
T
,
Mansilha
A
,
Azevedo
E
,
Carvalho
M
.
Stroke and multiple peripheral thrombotic events in an adult with varicella
.
Eur J Neurol
.
2008
;
15
(
10
):
e90
1
.
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