A 47-year-old man presented to the
emergency department with a
drooping right eye. He also complained
of a constant right-sided
headache of 1 week's duration; the
pain involved the temporal region.
Another physician had diagnosed
new-onset migraine and prescribed
sumatriptan, which failed to alleviate
the pain. The patient had no weakness,
vomiting, or double vision.
Both his father and his son had
The patient's vital signs were
normal. Mild right-sided ptosis and
miosis--accentuated by dim light--
were noted. Cranial nerves were
otherwise intact, and no other neurologic
abnormalities were found.
The neck was supple without bruits.
Breath sounds were normal. There
was no digital clubbing. A chest radiograph
and noncontrast CT scan
of the brain were normal.
Carotid dissection and subarachnoid
hemorrhage were considered
in the differential. Analysis of
cerebrospinal fluid from a lumbar
puncture showed no white blood
cells, 3 to 6 red blood cells per microliter,
a glucose level of 55 mg/dL,
and a protein level of 53 mg/dL.
These findings ruled out subarachnoid
During the patient's hospital
stay, a CT scan of the chest was negative
for Pancoast tumor of the lung.
An MRI scan/magnetic resonance
angiogram of the brain showed caliber
irregularity and slight course
derangement of the high cervical,
petrous portion of the right internal
carotid artery. Long-segment atherosclerosis
was considered unlikely
because there was no other site of
atherosclerotic change. Fibromuscular
dysplasia was also considered.
Carotid dissection was strongly
suspected. An angiogram of the
brain performed a month later
showed a 2.5-cm-long segment of
mild irregularity and focal ulceration
in the middle right internal carotid
artery, with no definite evidence of a
focal arterial dissection in this area.
Horner syndrome--a functional
sympathectomy of the ipsilateral
eye--is caused by injury or disruption
of the neural plexus that runs
from the sympathetic chain, past the
apex of the lung, and up the carotid
artery to the eye. Ptosis may be subtle.
Miosis is more marked in dim
light; it may be difficult to notice in
bright light. Potential causes of
Horner syndrome include carotid or
vertebral artery dissection, aortic
dissection, traumatic carotid injury,
deep neck infections, cerebrovascular
accident, cerebellar bleed, cluster
headache, and Pancoast tumor
of the lung.
Initial symptoms of carotid dissection
usually involve pain that affects
one side of the neck, face, or
head; the pain may start abruptly
but usually the onset is gradual. Pulsatile
tinnitus or a bruit is present in
about 25% of affected patients. Early
neurologic findings may involve the
sympathetic plexus (Horner syndrome
is present in about 50% of patients),
cranial nerve XII, or cerebellar
function. Eventually, transient
ischemic attack or thrombotic stroke
may occur. The mean time between onset of pain and onset of neurologic
symptoms is 4 days. Carotid dissection
is an important cause of
stroke in young adults and accounts
for up to 25% of cases.1
If carotid dissection is suspected,
magnetic resonance angiography
(MRA) with fat suppression is
the "gold standard" test. If MRA is
not available, alternative tests include
carotid duplex ultrasonography
and CT angiography (which is
almost as sensitive as MRA).
Treatment of suspected carotid
dissection involves consultation with
a neurologist and a neurosurgeon.
In contrast to aortic dissection,
complications, and treatment with
standard-dose heparin is started in
suspicious cases, following a negative
head CT scan. Anticoagulation
is usually maintained for 3 months.
Surgery is rarely required.
This patient almost certainly
had a carotid dissection related to
subclinical Marfan syndrome. Although
his imaging studies were
nondiagnostic, they were highly
suggestive of these disorders. He
probably should have received anticoagulant
therapy for 3 months.
Nevertheless, he did well and did
not have a stroke. His Horner syndrome
resolved after 3 months, although
he complained of occasional
mild right temporal headaches.
(Case and photograph courtesy of D. Brady
Pregerson, MD. Dr Pregerson is the author of 2
medical reference pocket books, Quick Essentials
and Side-Kick, available at www.EDinsight.com.)
1. Chaves C, Estol C, Esnaola MM, et al. Spontaneous
intracranial internal carotid artery dissection:
report of 10 patients. Arch Neurol. 2002;59:977-981.