Locked-in Syndrome (De-Efferented State)

KEY FEATURES

• Acute destructive lesions (eg, infarction, hemorrhage, demyelination, encephalitis) involving the ventral pons and sparing the tegmentum

• Patient is mute and quadriparetic but conscious

• Such a patient can mistakenly be regarded as comatose

• Clinicians should recognize that "locked-in" individuals are fully aware of their surroundings

CLINICAL FINDINGS

• Mute, quadriplegic patient

• Conscious state

• Patient is capable of blinking and of voluntary eye movement in the vertical plane

• Preserved pupillary responses to light

DIAGNOSIS

• Brain imaging with CT or MRI scan

• Obtain the expertise of a clinician familiar with this disorder to aid in the diagnosis

TREATMENT

• The prognosis is usually poor, but recovery has occasionally been reported, in rare cases including resumption of independent daily life