Locked-in syndrome and the current scientific insights

Transcription

1 Locked-in syndrome and the current scientific insights Eerste Lustrumsymposium Bieke Wittebolsfonds November , Leuven, BELGIË Ah Athena Demertzi & Steven Laureys Coma Science Group, Centre de Recherches du Cyclotron and CHU Neurology Department, Liège University Hospital Belgium

2 Overview Definition Diagnosis, prognosis, outcome Communication Quality of life Conclusions Le scaphandre et le papillon (2007) Direction: Julian Schnabel

3 Definition

4 disorders of consciousness behavioural evaluation electrophysiology neuroimaging methods, ethics & quality of life perspectives Locked-in syndrome (LIS) Presence of sustained eye opening Aphonia or severe hypophonia Ocular mode of communication Quadriplegia or quadriparesis-types: Classical i l Incomplete Total Preserved cognitive abilities Amercian Congress of Rehabilitation Medicine, 1995 Laureys et al, Prog in Brain Resc, 2005 Bauer et al, J Neurol, 1979

5 Cognitive function N=10 (evaluated 1-6 yrs after insult) Neuropsychological tests (adapted) Pure brainstem lesions intact cognitive levels Additional brain injuries associated cognitive deficits Healthy controls LIS Schnakers et al., J Neurol, 2008

6 Diagnosis, prognosis, outcome

7 Diangnosis Laureys, Owen and Schiff, Lancet Neurology, 2005

8 Misdiagnosis Person who made the diagnosis Number of patients (n=84) (% of total) Medical doctor 52 (62%) Family member 28 (33%) Other 4 (5%) Misdiagnosis explain by : - Rarity of LIS - Recognize signs of consciousness - Fluctuation of vigilance - Cognitive/sensory deficits Bruno et al., Pediatric Neurology, 2009 Laureys et al., Prog Brain Res, 2005

9 Electrophysiology Total locked-in syndrome (n=1) Pz (µv) Passive other name ms Count other name Passive own name Count own name Schnakers et al., Neurocase, 2009

10 disorders of consciousness behavioural evaluation electrophysiology neuroimaging methods, ethics & quality of life perspectives Neuroimaging g (PET) Bruno, Pellas et al., Revue de Neurologie, 2008 Laureys, Pellas et al., Prog Brain Res, 2005

11 Prognosis Laureys, Pellas et al., Prog Brain Res, 2005 Bruno, Pellas et al., Revue de Neurologie, 2008

12 Outcome Motor recovery Number of patients (n=84) (% of total) Head 87 (92%) Upper extremities 62 (65%) Lower extremities 70 (74%) Questionnaire ALIS 2006

13 Communication

14 Communication Vertical eye closure ESARIN - Consonant/ vowel method E-S-A-R-I-N-T-U-L-O-M-D-P-C-F-B-V-H-G-J-Q-Z-Y-X-K V C1 C2 C3 A B J R E C K S I D L T O F M V U G N W Y H P X Q Z Le scaphandre et le papillon (2007) Direction: Julian Schnabel

15 Communication Vertical eye closure ESARIN - Consonant/ vowel method Eye tracking system

16 Communication Vertical eye closure ESARIN - Consonant/ vowel method Eye tracking system Brain-computer interfaces (BCIs) Electrophysiology (ERPs)

17 Brain-computer interfaces (ERPs) - System of communication - Different applications - Different techniques

18 Brain-computer interfaces (ERPs) - Autobiographical questions - Choose letter, form word and sentences

19 Art becomes possible

20 Communication Vertical eye closure ESARIN - Consonant/ vowel method Eye tracking system Brain-computer interfaces (BCIs) Electrophysiology (ERPs) Functional MRI

21 fmri: active paradigms Boly et al, NeuroImage 2007 Owen, Coleman, Boly, Davis, Laureys & Pickard, Science 2006

22 Brain Computer Interfaces - 3 mental tasks - 3 durations - 3 onsets 27 different dynamic cerebral activations i // letters of the alphabet Sorger et al, PBR, 2009

23 Quality of life

24 Quality of life Short Form-36 in brainstem stroke LIS patients (n=17; duration 6±4 y) Reintegration to Normal Living Index in ALS patients (n=30; duration 6±5 y) Laureys et al., Prog Brain Res, 2005 Lulé et al., Prog Brain Res, 2009

25 Quality of life Unable to move around in community 60 Limited social activities 20 Pain Euthanasia 5 Suicidal thoughts 27 Reanimation ALIS Questionnaire 2007

26 disorders of consciousness Definition behavioural Diagnosis, evaluation prognosis, electrophysiology outcome Communication neuroimaging Quality methods, of ethics life & Conclusions quality of life perspectives Quality of Life Case 1 24-year-old girl LIS for 6 years Good social interactions Occasionally depressed No suicidal thoughts Never considered euthanasia No Reanimation in case of cardiac arrest X Control subjects n=820 X Locked-in patients n=65 Bernheim et al, 1999 Bruno et al, in preparation Bruno et al, Pediatic Neurology, 2009

27 disorders of consciousness Definition behavioural Diagnosis, evaluation prognosis, electrophysiology outcome Communication neuroimaging Quality methods, of ethics life & Conclusions quality of life perspectives Quality of Life Case 2 17-year-old girl LIS for 1 year Good social interactions Occasionally depressed No suicidal thoughts Never considered euthanasia Reanimation in case of cardiac arrest X Control subjects n=820 X Locked-in patients n=65 Bernheim et al, 1999 Bruno et al, in preparation Bruno et al, Pediatic Neurology, 2009

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29 Conclusions

30 Conclusions Preserved cognitive capacities Misdiagnosis still happens Objective evaluation (PET, fmri, ERPs) Technology-based communication Good quality of life Don t judge a book by its cover!

31 THANK YOU Slides can be downloaded from PhD candidates: Audrey Vanhaudenhuyse ARC Marie-Aurélie Bruno FNRS Olivia Gosseries FNRS Camille Chatelle FNRS Marie Thonnard non-fria Victor Cologan FRIA Jean-Floris Tshibanda MD Pierre Boveroux MD CHU Muriel Kirsch MD Audrey Maudoux MD FNRS Isabelle Lutte MD ULB Collaborations: NY J Giacino, N Schiff, J Fins Cambridge A Owen Milano M Massimini Wisconsin G Tononi Tubingen & Wurzburg A Kübler Paris L Puybasset Hangzou China H Di Salzburg M Schabus Lyon F Perrin PhDs: Melanie Boly MD Didier Ledoux MD Caroline Schnakers Quentin Noirhomme Engineering Andrea Soddu Physics Betina Sorger Maastricht Dorothée Lulé Tubingen Visiting fellows: Natalia Lapitskaia, Remy Lehembre, Jonathan Orban, Francisco Gomez

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