Locked-in syndrome may be classified into 3 categories: the classic form, the incomplete form (patients have other voluntary movements), and the total form with a completely motionless patient including absence of vertical eye movements.1 Clinical experience has shown that classic locked-in syndrome from pontine infarction may improve, but rarely, to functional independence.2 In a series of 14 patients with classic or total locked-in syndrome seen 3 to 6 months following neurorehabilitation, motor recovery was found in 21%; return of swallowing in 42%; verbal communication in 28%; bladder and bowel control in 35%; and weaning of the ventilator in half of the patients.3 Most dramatic is early recovery from locked-in syndrome usually reported after recanalization of the basilar artery4 but improvement may be the result of major fluctuation of deficit in the early defining stage.5 We present an improvement of a patient in a nearly complete locked-in syndrome.
A 22-year-old healthy man who exercised 3 to 4 hours daily presented with an acute basilar artery occlusion. Pontine and cerebellar infarctions were found despite aggressive treatment with intravenous thrombolysis and endovascular thrombectomy (Figure, A). Follow-up vascular imaging confirmed complete recanalization. He remained awake and attentive, with a gaze preference to the left and minimal horizontal eye movements to the right. He had a complete flaccid quadriparesis. Vertical eye movements and blinking were preserved. He had a trace of jaw and oropharyngeal movement (Video). His stroke was found to be due to relative dehydration in the setting of a heterozygous mutation for factor V Leiden. At 3 months, he was weaned off the ventilator and tracheostomy was removed. There was a severe dysarthria and some cephalic, but no trunk control. At 6 months, he was able to sit with support of his hands. At 10 months, he was able to ambulate with a walker. At 17 months, he was able to climb stairs. His trajectory of recovery was very gradual but eventually led to a substantial recovery (Video). His follow-up magnetic resonance imaging scan showed a large punched-out infarct in the ventral pons (Figure, B).
A, Magnetic resonance imaging performed 1 day after the onset of symptoms depicting diffusion restriction in the bilateral posterior cerebral artery territories and pons. B, Follow-up magnetic resonance imaging scan performed 22 months after his stroke that shows a substantial infarct in the ventral pons.
The video shows an awake, attentive patient with a gaze preference to the left and minimal horizontal eye movements to the right. Vertical eye movements and blinking were preserved. Twenty-two months later, he is shown ambulating with an ataxic gait. He has difficulty with tandem walking. There is a mild residual horizontal ophthalmoplegia and left facial weakness in an upper motor neuron pattern.
Locked-in syndrome is due to a ventral pons lesion, which deafferentiates the patient for most motor function (absent horizontal eye movements, no grimacing, no swallowing, no head movements, and no limb movements). Patients with locked-in syndrome have their eyes open, blink, and may have spontaneous vertical eye movements. A large proportion of patients die of pulmonary complications or withdrawal of support, but patients may survive for decades. Using serial videos, we were able to document that a patient with a nearly classic syndrome (but incomplete owing to some bulbar function) may improve dramatically over a prolonged period. Perhaps the presence of some oropharyngeal function early may be the prelude to recovery of speech and motor function. Imbalance due to spastic ataxia remained but most other motor functions recovered well.
We documented the clinical trajectory of a patient with a large pontine and cerebellar infarct and were able to confirm substantial improvement. Such an improvement may be more likely in incomplete forms of the syndrome.
Corresponding Author: Sara Hocker, MD, Division of Critical Care Neurology, Mayo Clinic, 200 First St SW, Rochester, MN 55905 (firstname.lastname@example.org).
Conflict of Interest Disclosures: None reported.
Hocker S, Wijdicks EFM. Recovery From Locked-in Syndrome. JAMA Neurol. 2015;72(7):832-833. doi:10.1001/jamaneurol.2015.0479