Department of Neurology,
University of Helsinki, Finland
A 29-year-old healthy woman suffered an
episode of transient locked-in syndrome followed
by vomiting and headache. Based on clinical data
the symptom was related to transitory ischemia
probably due to basilar artery migraine. This is
the first report of locked-in syndrome in
association with migraine
The term locked-in syndrome (LIS) was coined
by Plum and Posner to describe a usually fatal
state with tetraparesis and paralysis of the
lower cranial nerves that spares only vertical
eye movements and blinking in a fully conscious
patient. LIS results from a lesion in the
ventral pons, most commonly of vascular origin.
There are, however, some reports of LIS with
recovery. These include cases with head trauma,
brain stem encephalitis, central pontine
myelinolysis and brain stem infarct.
We report a patient with transient LIS,
probably caused by a vascular disturbance in the
vertebrobasilar arterial system associated with
a history of mtgraine.
Case report. A 29-year-old healthy
woman suddenly, without precipitating factors,
experienced right hemiparesis which progressed
rapidly to quadriplegia. The patient was fully
conscious, but could not move her limbs or talk,
and experienced horizontal diplopia and
amblyopia. However, she could freely shut and
open her eyelids and volitionally move her eyes
vertically and to some extent also horizontally.
During this episode the patient was alone but
she assumes that she could have been able to
communicate with other persons by the help of
eye movements. Alter ten minutes she gradually
recovered with a dysarthric speech and paresis
of the right arm. She was tired, kept
yawning and
felt the need to defecate. Alter an hour the
neurologic symptoms had totally disappeared but
she vomited once and suffered from diffuse
headache.
Thereafter the patient has had one attack of
throbbing occipital headache and nausea preceded
by bilateral scintillating scotomas but without
other neurologic symptoms. Earlier she had had a
few attacks of non-localized headache with
nausea and vomiling but without premonitory
symptoms. Her mother suffers from migraine.
Clinical neurologic and cardiovascular
examination were completely normal two weeks
alter the attack. CT scan did not show any
structural abnormalities in brain or brain stem.
Aortic arch angiography was completely normal.
Cerebrospinal fluid cells, protein and
lgG/Albumin-ratio were normal. An
electroencephalogram revealed some episodic
bursts of sharp waves more on the left side
superponed on a normal background. Brain stem
evoked responses showed normal conduction time
and amplitude bilaterally.
DISCUSSION
LIS usually occurs following infarction of
the ventral portion of the pons.8 In a case of
transient, post-traumatic LIS, a transitory
ischemia at a ventral pontine level due to
compression of the vertebral artery was
suspected.
The premonitory symptoms of basilar artery
migraine are known to reflect brain stem
dysfunction. Symptoms related to basilar artery
migraine include disturbancies of vision,
dysarthria, ataxia, vertigo, bilateral
peripheral dysesthesiae, impairment of
consciousness, cortical blindness, and transient
global amnesia.10-13 However, a complete LIS has
not been reported as a symptom of migraine.
The symptoms of our patient strongly suggest
a transient ventral brain stem dysfunction with
LIS including a complete tetraplegia and
anarthna. Diplopia with partially preserved
horizontal eye movements indicate, however, that
the central connections to the sixth cranial
nerve nucleus were not totally interrupted.
Our patient was a young woman in excellent
physical condition without known risk factors of
cerebrovascular disease. She had a positive
family history of migraine and had earlier
experienced a few attacks of common migraine and
11/2 years alter LIS an attack preceded by
visual symptoms. Also immediately after the
described episode of the transient LIS she
vomited and had diffuse headache. The patient
completely recovered from LIS and in the
examinations carried out, including CT scan,
electroencephalography, brain stem evoked
responses and cerebrospinal fluid, there were
nothing to indicate a permanent lesion of the
brain stem.
In conclusion, it can be stated that
transient LIS in our patient was probably due to
transitory ischemia in the vertebrobasilar
arterial system. The clinical course of the
attack parallels the description of patients
with basilar artery migraine. Our case thus
suggests that LIS should be considered as a
possible complication of migraine.
REFERENCES
1. Plum F, Posner JB: Diagnosis of stupor
and coma. Philadelphia, Davis, 1966.
2. Nordgren RE, Markesbury WR, Fukuda K,
Reeves AG: Seven cases of cerebromedullospinal
disconnection: the locked-in" syndrome.
Neurology (Minneap) 21:1140-1148, 1971.
