Monday, June 21, 2010

Between Life and Death - Part II - Locked-in syndrome

Locked-in syndrome

Locked-In syndrome is a condition in which a patient is aware and awake, but cannot move or communicate due to complete paralysis of nearly all voluntary muscles in the body. It is the result of a brain stem lesion in which the ventral part of the pons is damaged. The condition has been described as 'the closest thing to being buried alive'.Locked-in alive'.Locked-in syndrome is also known as Cerebromedullospinal Disconnection, De-Efferented State, Pseudocoma, and ventral pontine syndrome.

Clinical Presentation

  • Unable to speak
  • unable to move the limbs
  • Awareness and consciousness preserved
  • Able to open eyes and move them and blink, in order to try to communicate.
Causes

Locked-in syndrome is caused by damage to specific portions of the lower brain and brainstem with no damage to the upper brain.

Possible causes of locked-in syndrome include:
- Traumatic brain injury
- Diseases of the circulatory system
- Medication overdose
- Damage to nerve cells, particularly destruction of the myelin sheath, caused by disease (e.g. central pontine myelinolysis secondary to rapid correction of hyponatremia).
- A stroke or brain hemorrhage

Treatment

There is no standard treatment for Locked-In syndrome, nor is there a cure. Stimulation of muscle reflexes with electrodes (Neuromuscular stimulation) has been known to help patients regain some muscle function.
Other courses of treatment are often symptomatic.

Progress

It is extremely rare for any significant motor function to return. The majority of locked-in syndrome patients do not regain motor control, but devices are available to help patients communicate.

References

Medical Encyclopedia from MobileReference
Clinical Neurology, Graeme J Hankley MD, FRCP (Lond), FRCP(Edin), FRACP, Joana M Wardlaw MBchB, MD, FRCP, FRCR
Clinical Neurology, 7e, Roger P. Simson, MD, David A. Greenberg, MD, PHD

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