Ann Rehabil Med.  2014 Oct;38(5):702-706. 10.5535/arm.2014.38.5.702.

Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report

• Sohn MK
• Nam JH

• KMID: 2266507
• DOI: http://doi.org/10.5535/arm.2014.38.5.702

Abstract

Central pontine myelinolysis (CPM) classically occurs in alcoholics, malnourished individuals, chronic liver diseases, and rapid correction of hyponatremia. This report presents locked-in syndrome due to CPM following rapid correction of hyponatremia. A 44-year-old male came to the hospital due to a short period of loss of consciousness. He was alert and had no focal neurological abnormalities at admission. The serum sodium concentration was 118 mEq/L and was corrected to 134 mEq/L in the first 18 hours. One week later, progressive weakness in limbs developed and he progressed to a complete quadriplegic state and bulbar palsy, with only eye blinking preserved. Brain magnetic resonance imaging revealed a characteristic hyperintense signal abnormality in both pons, so he was diagnosed to locked-in syndrome caused by CPM. The patient gradually improved following continuous intensive rehabilitation for more than 2 years. He was able to move all joint muscles against gravity in generally and he could gait under supervision.

Keyword

Central pontine myelinolysis; Quadriplegia; Recovery of function

MeSH Terms

Adult
Alcoholics
Blinking
Brain
Bulbar Palsy, Progressive
Extremities
Gait
Gravitation
Humans
Hyponatremia
Joints
Liver Diseases
Magnetic Resonance Imaging
Male
Muscles
Myelinolysis, Central Pontine*
Organization and Administration
Pons
Quadriplegia*
Recovery of Function
Rehabilitation
Sodium
Unconsciousness
Sodium

Figure

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  Fig. 1 Changes in serum sodium (Na) concentrations during the first day.

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  Fig. 2 (A, B) Brain MRI shows T2- and diffusion-weighted imaging high signal intensity lesion in the bilateral pons, osmotic demyelination syndrome in pons 10 days after onset of symptoms. (C, D) Encephalomalacia at the site of initial high signal intensity lesion in pons after 1 year.

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  Fig. 3 (A) Somatosensory evoked potential (SEP) study stimulating both tibial nerves and median nerves shows absent potentials, bilaterally at first admission. (B) SEP study stimulating the tibial nerve shows prolonged latency of P37, N45 potentials, bilaterally and SEP study stimulating both median nerves shows prolonged latency of N19, P23 potentials at 3 years after onset.

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