Locked-In Syndrome in Children: Report of Five Cases and Review of the Literature

Bruno, Marie-Aurélie; Schnakers, Caroline; Damas, François; Pellas, F.; Lutte, Isabelle; Bernheim, Jan L.; Majerus, Steve; Moonen, Gustave; Goldman, Serge; Laureys, Steven

doi:10.1016/j.pediatrneurol.2009.05.001

Cited by 61 publications

(60 citation statements)

References 57 publications

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“…This problem was recently highlighted in a study showing that the clinical consensus diagnosis could be incorrect in more than 40% of patients considered to be vegetative [18]. Locked-in syndrome patients may also be mistakenly considered unconscious [37,38]. These studies should aid clinicians to use standardized validated behavioural scales of consciousness when making a diagnosis in these challenging patients.…”

Section: Clinical Bedside Assessment Using Consciousness Scalesmentioning

confidence: 99%

From unresponsive wakefulness to minimally conscious PLUS and functional locked-in syndromes: recent advances in our understanding of disorders of consciousness

et al. 2011

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Functional neuroimaging and electrophysiology studies are changing our understanding of patients with coma and related states. Some severely brain damaged patients may show residual cortical processing in the absence of behavioural signs of consciousness. Given these new findings, the diagnostic errors and their potential effects on treatment as well as concerns regarding the negative associations intrinsic to the term vegetative state, the European Task Force on Disorders of Consciousness has recently proposed the more neutral and descriptive term unresponsive wakefulness syndrome. When vegetative/unresponsive patients show minimal signs of consciousness but are unable to reliably communicate the term minimally responsive or minimally conscious state (MCS) is used. MCS was recently subcategorized based on the complexity of patients' behaviours: MCS? describes highlevel behavioural responses (i.e., command following, intelligible verbalizations or non-functional communication) and MCS-describes low-level behavioural responses (i.e., visual pursuit, localization of noxious stimulation or contingent behaviour such as appropriate smiling or crying to emotional stimuli). Finally, patients who show nonbehavioural evidence of consciousness or communication only measurable via para-clinical testing (i.e., functional MRI, positron emission tomography, EEG or evoked potentials) can be considered to be in a functional locked-in syndrome. An improved assessment of brain function in coma and related states is not only changing nosology and medical care but also offers a better-documented diagnosis and prognosis and helps to further identify the neural correlates of human consciousness.

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Section: Clinical Bedside Assessment Using Consciousness Scalesmentioning

confidence: 99%

From unresponsive wakefulness to minimally conscious PLUS and functional locked-in syndromes: recent advances in our understanding of disorders of consciousness

et al. 2011

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“…La détection de signes de conscience non ambigus au chevet des patients ayant un état de conscience altérée reste délicate car elle dépend des difficultés motrices ou verbales des patients, mais égale-ment des critères diagnostiques utilisés, des échelles ou de l'expertise du clinicien [10][11][12]. Le nombre de patients diagnostiqués par erreur comme étant inconscients est particulièrement important (40 %) [10].…”

Section: Resultsunclassified

“…Cependant, les comportements observés varient fortement d'un patient à l'autre et peuvent être très discrets. Dès lors, la détection clinique de signes comportementaux de conscience est difficile [10,11] et ce pour plusieurs raisons : la présence de défi-cits moteurs et/ou langagiers, la fluctuation du niveau de vigilance des patients [12], le manque d'expertise des cliniciens, le manque de consensus dans la littérature ou encore l'utilisation de critères ou d'échelles diagnostiques d'une sensibilité insuffisante [10,11,13]. Une étude récente révèle que le diagnostic d'EV est erroné dans 40 % des cas [10], ce qui peut avoir de graves conséquences sur les décisions théra-peutiques ou de fin de vie.…”

Section: Paradigmes Passifs Et Stimulations Sensoriellesunclassified

La neuro-imagerie : un outil diagnostique des états de conscience altérée

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“…5 There is no definitive treatment for this condition, and care is mostly supportive, with anticipation of difficulties encountered during feeding, ambulation and phonation. As the posterior pons containing the reticular activating system, is uninvolved-sleep-wake cycle and consciousness are unaffected.…”

Section: Discussionmentioning

confidence: 99%