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Locked-in syndrome is a rare disease defined by the association of tetraplegia and palsy of most bulbar muscles without loss of consciousness. Vertical eye movements and blinking are the only means of communication. Locked-in syndrome is due to a pontine lesion, exceptionally to mesencephalic or bilateral lesions in capsula interna. The etiology is generally vascular secondary to basilar artery thrombosis. Rarely, the syndrome may be caused by hemorrhage, tumor, central pontine myelinolysis, infections, trauma, heroin abuse... It may be difficult to differentiate Locked-in syndrome from other types of tetraplegia associated with loss of consciousness (akinetic mutism or vegetative state) because of the vigilance fluctuations in patients, especially in acute stage. Management requires specialized care. Therapy includes etiological treatment, supportive psychotherapy and physiotherapy. Morbidity and mortality are significant, nevertheless partial recovery may occur. |