General Jordi

Case

Ruscalleda,

MD

of the #{149} Pilar

Day1

Coscojuela,

MD

HISTORY

U

ischemic

man attacks

attacks

were

had for

experienced

tion

past

the

past

10 years.

characterized

2 years,

in intellectual Bluish

The

by paresthesia.s

of

and lanworsened

accompanied of the

skin

Computed

magnetic resonance bral angiography

on

the

trunk

were

tomography

(MR) imaging, performed.

and

From

the

September

arteries,

1991;

1 1:929-93

Department

Avenida San sion requested RSNA.

Carotid

Antonio April

of Diagnostic

diseases,

17. 183,

17.259

diffuse

MD

severe

images

showed

areas

corresponding

with

of increased

lesions;

marked

slow

flow

in-

and

demy-

atrophy

were also evident of medium-sized

and

angiomatous

right

and

(CT),

teries

were

seen

with

cere-

(Fig

left

clusters

middle

carotid

(Fig 2). arteries

cerebral

ar-

angiography

3).

of low attenuation obtained

cerebral

ventric1). MR

signal

to ischemia

distal

#{149} Cerebral

and

tensity,

in the

Figure 1 . (a) Axial CT scan shows ischemic areas and both thalami (small arrows). (b) Axial CT scan and arachnoid spaces.

RadioGraphics

Dejuan,

with dilatation of the arachnoid spaces (Fig

and lacunar lesions Multiple occlusions

extremities was seen at physical Results of laboratory tests were

unremarkable.

terms:

#{149} Manuel

cortical atrophy, ular system and

elinating

by deteriora-

capabilities.

mottling

and the lower examination.

Index

MD

FINDINGS CT scans demonstrated

transient

the left hand and lapses of memory guage. The clinical symptoms had in the

Guardia,

U

A 40-year-old

(

#{149} Esteve

in the right

at a higher

angiography,

level

17. 124

shows

frontal lobe dilatation

blood

#{149} Cerebral

vessels,

(large arrow) of ventricles

diseases.

17. 183.

17.2S9

1 Radiology.

Maria Claret 167, 08025 10 and received May 20;

Section Barcelona. accepted

of Neuroradiology.

Hospital

Spain. From the 1990 May 2 1 . Address reprint

de

Ia Santa

Creu

i Sant

RSNA scientific assembly. requests toJ.R.

Pau.

Universidad

Received

February

Autonoma. 2.

1991;

revi.

1991

1991

Ruscalleda

et al

U

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a Figure 2. (a) Axial T2 proton-density MR image shows increased signal intensity corresponding to ischemia and demyelinating lesions in the right frontal lobe (arrow) and thalamus (arrowhead). (b) Axial T2 proton-density MR image obtained at a higher level shows paraventricular white matter lesions

(arrowheads).

image

shows

(c)

lacunar

T2-weighted

Coronal

lesions

DIAGNOSIS:

Cerebromeningeal

tosis

syndrome).

U

(Sneddon

MR

(arrowheads).

angioma-

DISCUSSION

Angiodysplasias

are

vascular

anomalies

that

are linked to an embryologic developmental disorder ofvascular sheaths, which can affect the whole organism. Cerebromeningeal angiomatoses comprise several entities that are accompanied by cutaneous lesions or characteristic neurologic symptoms that help determine the diagnosis. These entities may be classified

fuse

according

systemic

to whether

involvement

Weber syndrome, Bean syndrome,

Louis-Bar

regional angiomatosis, syndrome,

Klippel-Trenaunay

syndrome)

or or

factors,

leads

nervous

system

The main Sneddon

(1).

of medium-sized

The

RadioGraphics

vessels.

influence

arteriopThe

ofhormonal

U

Ruscafleda

cause

and

et al

involve-

that supply as the skin

with

pathologic

syndrome

derivatives and the cen-

its covering

is

im-

tion

nervous in the

skin

alterations occur

reticularis, which physical examination part in the global

noninflammatory

or nicotine

layers

(2,3).

gressive,

occlusive,

hypertension,

to a noninflammatory

ment of small arteries of the ectoderm, such tral

involvement (eg, Bonnet-DeCobb syndrome,

syndrome)

abuse

central

athy

U

munologic

naevus Baily

Sneddon syndrome can be included in this second category. It is a rare disease with pro-

obscure.

930

is dif-

Rendu-Osler-

blue rubber-bleb Maffucci syndrome,

gliomangiomatosis,

predominantly Sturge-Weber Chaume-Blanc

there

(eg,

system. is the

caused

in the

A clinical

skin

by

and

the

manifesta-

aforementioned

livedo

is an important finding at and plays an important diagnosis. For this reason,

Volume

11

Number

5

a.

b. Figure

3.

(a, b) Bilateral

angiograms

of carotid

artery

show arterial-phase multiple occlusions and angiomatous clusters in the distal part of mediumsized arteries (arrows). (c) Angiogram from late capillary and venous phase shows evidence of slow

flow.

U

C.

1. 2.

several

authors

include

graphic

studies

of the

articles.

However,

patient’s

visit

involvement, gressive

alterations extremities

the

main

is the

central

manifests

signs

with

areas

only

The

with

arterial geal

occlusions

This

entity

of cutaneous

logic

emic

lesions.

formed signs

September

or

to help (5-7).

1991

cerebromenin(3,4).

6.

remembered

in cases

and

long-term

neuro-

either

cutaneous

Angiography recognize

visible

multiple

without

show

and

in which

demyelinizing should the

5.

lacunar are

a rich

arteriosclerosis

MR imaging

small

shows

be

lesions

vasculopathy

or

atrophy

which with

4.

nonspecific,

alterations

revascularization

involvement

CT and

totally

hypoxia

should

as pro-

to severe

cortical

vascular

angiography,

collateral

system

leading

are

3. the

clinically

of accentuated

of chronic

infarctions.

for

nervous

strokes,

cerebral dysfunction. CT and MR findings showing

in their reason

which cerebral

in angio-

be

characteristic

ischper-

7.

REFERENCES AndreJM, Picard L. Les angiodisplasies systematis#{233}s. J Neuroradiol 1974; 1:3-45. Bruyn RPM. . Sneddon’s syndrome. In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook ofclinical neurology. Vol II. Vascular diseases, part 3. New York: Elsevier, 1989. Rebollo M, Va1JF, Garijo F, Quintana F, BercianoJ. Livedo reticularis and cerebrovasculax lesions (Sneddon’s syndrome). Brain 1983; 106:965-979. Blom RJ. Sneddon syndrome: CT, arteriography, and MR imaging. J Comput Assist Tomogr 1989; 13:119-122. VonsattelJG, Hedley-Whyte T. Diffuse meningocerebral angiomatosis and leucoencephalopathy. In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of clinical neurology. Vol 11. Vascular diseases, part 3. New York: Elsevier, 1989. Marsch WC, Muckelmann R. Generalized racemose livedo with cerebrovascular lesions (Sneddon syndrome) : an occlusive arteriopathy due to proliferation and migration of medial smooth muscle cells. BrJ Dermatol 1985; 112:703-708. Levine SR, Welch KM. The spectrum of neurologic disease associated with antiphospholipid antibodies. Arch Neurol 1987; 44:876883.

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et al

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