TY - JOUR T1 - Locked-in: don’t judge a book by its cover JF - Journal of Neurology, Neurosurgery & Psychiatry JO - J Neurol Neurosurg Psychiatry SP - 2 LP - 2 DO - 10.1136/jnnp.2007.125294 VL - 79 IS - 1 AU - M Bruno AU - J L Bernheim AU - C Schnakers AU - S Laureys Y1 - 2008/01/01 UR - http://jnnp.bmj.com/content/79/1/2.abstract N2 - Amyotrophic lateral sclerosis (ALS; also called motor neuron disease) is a devastating medical condition that progressively robs patients of their ability to move, speak and eventually breathe. At present, many physicians are hesitant to propose tracheostomy and respiratory support in the terminal phase of ALS. In accordance with the principle of patient autonomy, physicians should respect the right of the ALS patient to accept or refuse any treatment, including mechanical ventilation. Also, in environments where euthanasia or physician-assisted death is legal, such requests can be acceptable. At least two conditions are necessary for full autonomy. To have a claim on full autonomy, people need to have intact cognitive abilities, and to exercise this right they must be able to communicate. In the past, the first condition was in doubt (indeed, overlap with other neurodegenerative diseases is sometimes suspected and some patients with ALS are thought to have associated frontotemporal dementia) and the second was severely compromised in patients with devastating motor impairment (communication being limited to the twitch of a finger or the blink of an eye).In this issue of J … ER -