La migraine
Accidents vasculaires cérébraux

mise à jour du
5 février 2006
1983; 23 ;238-239
Locked-in syndrome with rapid recovery:
a manifestation of basilar artery migraine?
Sulkava R, Kovanen J
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.
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.
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