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Locked-in syndrome

MedGen UID:
7378
•Concept ID:
C0023944
•
Disease or Syndrome
Synonyms: Cerebromedullospinal Disconnection; Cerebromedullospinal Disconnections; Locked In Syndrome; Locked-In Syndrome; Locked-In Syndromes; Ventral Pontine Syndrome; Ventral Pontine Syndromes
SNOMED CT: Locked in syndrome (38023001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0016567
Orphanet: ORPHA2406

Definition

Locked-in syndrome (LIS) is a neurological condition characterized by the presence of sustained eye opening, quadriplegia or quadriparesis, anarthria, preserved cognitive functioning and a primary code of communication that uses vertical eye movements or blinking. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLocked-in syndrome
Follow this link to review classifications for Locked-in syndrome in Orphanet.

Professional guidelines

PubMed

Locked-in syndrome responding to endovascular treatment.
Song J, Huang J, Li L, Yang J, Yue C, Liu S, Kong W, Luo X, Liao J, Du J, Song B, Liu J, Tian X, Tan X, Li F, Zi W
J Neurointerv Surg 2023 Aug;15(8):808-813. Epub 2022 Aug 19 doi: 10.1136/jnis-2022-019112. PMID: 35985839Free PMC Article
Minimally Conscious State: Evolution of Concept, Diagnosis and Treatment.
Hodelín-Tablada R
MEDICC Rev 2016 Oct;18(4):43-46. doi: 10.37757/MR2016.V18.N4.9. PMID: 27829654
Disorders of consciousness: differential diagnosis and neuropathologic features.
Giacino JT
Semin Neurol 1997 Jun;17(2):105-11. doi: 10.1055/s-2008-1040919. PMID: 9195652

Recent clinical studies

Etiology

Brain-Computer interfaces for communication: preferences of individuals with locked-in syndrome, caregivers and researchers.
Branco MP, Pels EGM, Nijboer F, Ramsey NF, Vansteensel MJ
Disabil Rehabil Assist Technol 2023 Aug;18(6):963-973. Epub 2021 Aug 12 doi: 10.1080/17483107.2021.1958932. PMID: 34383613Free PMC Article
The clinical and cognitive spectrum of locked-in syndrome: 1-year follow-up of 100 patients.
Kumral E, Dorukoğlu M, Uzunoğlu C, Çetin FE
Acta Neurol Belg 2022 Feb;122(1):113-121. Epub 2021 May 13 doi: 10.1007/s13760-021-01675-5. PMID: 33987814
Single-paradigm and hybrid brain computing interfaces and their use by disabled patients.
de Neeling M, Van Hulle MM
J Neural Eng 2019 Oct 23;16(6):061001. doi: 10.1088/1741-2552/ab2706. PMID: 31163412
"Look at my classifier's result": Disentangling unresponsive from (minimally) conscious patients.
Noirhomme Q, Brecheisen R, Lesenfants D, Antonopoulos G, Laureys S
Neuroimage 2017 Jan 15;145(Pt B):288-303. Epub 2015 Dec 12 doi: 10.1016/j.neuroimage.2015.12.006. PMID: 26690804
Validation of a new coma scale: The FOUR score.
Wijdicks EF, Bamlet WR, Maramattom BV, Manno EM, McClelland RL
Ann Neurol 2005 Oct;58(4):585-93. doi: 10.1002/ana.20611. PMID: 16178024

Diagnosis

Therapeutic Approaches in Locked-in Syndrome.
Papadopoulou SL, Dionyssiotis Y, Krikonis K, Lаgopati N, Kamenov I, Markoula S
Folia Med (Plovdiv) 2019 Sep 30;61(3):343-351. doi: 10.3897/folmed.61.e39425. PMID: 32337919
Locked-In Syndrome #303.
Maiser S, Kabir A, Sabsevitz D, Peltier W
J Palliat Med 2016 Apr;19(4):460-1. Epub 2016 Mar 7 doi: 10.1089/jpm.2016.0016. PMID: 26950052
Locked-in syndrome.
Cardwell MS
Tex Med 2013 Feb 1;109(2):e1. PMID: 23378122
Validation of a new coma scale: The FOUR score.
Wijdicks EF, Bamlet WR, Maramattom BV, Manno EM, McClelland RL
Ann Neurol 2005 Oct;58(4):585-93. doi: 10.1002/ana.20611. PMID: 16178024
Locked-in syndrome.
Smith E, Delargy M
BMJ 2005 Feb 19;330(7488):406-9. doi: 10.1136/bmj.330.7488.406. PMID: 15718541Free PMC Article

Therapy

Locked-in syndrome responding to endovascular treatment.
Song J, Huang J, Li L, Yang J, Yue C, Liu S, Kong W, Luo X, Liao J, Du J, Song B, Liu J, Tian X, Tan X, Li F, Zi W
J Neurointerv Surg 2023 Aug;15(8):808-813. Epub 2022 Aug 19 doi: 10.1136/jnis-2022-019112. PMID: 35985839Free PMC Article
Sleep in the completely locked-in state (CLIS) in amyotrophic lateral sclerosis.
Malekshahi A, Chaudhary U, Jaramillo-Gonzalez A, Lucas Luna A, Rana A, Tonin A, Birbaumer N, Gais S
Sleep 2019 Dec 24;42(12) doi: 10.1093/sleep/zsz185. PMID: 31665518
Locked-in syndrome responding to thrombolytic therapy.
Johnson TM, Romero CS, Smith AT
Am J Emerg Med 2018 Oct;36(10):1928.e5-1928.e7. Epub 2018 Jul 3 doi: 10.1016/j.ajem.2018.07.003. PMID: 29983216
Stable long-term BCI-enabled communication in ALS and locked-in syndrome using LFP signals.
Milekovic T, Sarma AA, Bacher D, Simeral JD, Saab J, Pandarinath C, Sorice BL, Blabe C, Oakley EM, Tringale KR, Eskandar E, Cash SS, Henderson JM, Shenoy KV, Donoghue JP, Hochberg LR
J Neurophysiol 2018 Jul 1;120(1):343-360. Epub 2018 Apr 25 doi: 10.1152/jn.00493.2017. PMID: 29694279Free PMC Article
The locked-in syndrome: a syndrome looking for a therapy.
León-Carrión J, van Eeckhout P, Domínguez-Morales Mdel R
Brain Inj 2002 Jul;16(7):555-69. doi: 10.1080/02699050110119466. PMID: 12119075

Prognosis

Locked-in syndrome responding to endovascular treatment.
Song J, Huang J, Li L, Yang J, Yue C, Liu S, Kong W, Luo X, Liao J, Du J, Song B, Liu J, Tian X, Tan X, Li F, Zi W
J Neurointerv Surg 2023 Aug;15(8):808-813. Epub 2022 Aug 19 doi: 10.1136/jnis-2022-019112. PMID: 35985839Free PMC Article
Locked-in syndrome.
Cardwell MS
Tex Med 2013 Feb 1;109(2):e1. PMID: 23378122
EEG in connection with coma.
Wilson JA, Nordal HJ
Tidsskr Nor Laegeforen 2013 Jan 8;133(1):53-7. doi: 10.4045/tidsskr.11.1432. PMID: 23306996
Validation of a new coma scale: The FOUR score.
Wijdicks EF, Bamlet WR, Maramattom BV, Manno EM, McClelland RL
Ann Neurol 2005 Oct;58(4):585-93. doi: 10.1002/ana.20611. PMID: 16178024
Locked-in syndrome.
Smith E, Delargy M
BMJ 2005 Feb 19;330(7488):406-9. doi: 10.1136/bmj.330.7488.406. PMID: 15718541Free PMC Article

Clinical prediction guides

Locked-in syndrome responding to endovascular treatment.
Song J, Huang J, Li L, Yang J, Yue C, Liu S, Kong W, Luo X, Liao J, Du J, Song B, Liu J, Tian X, Tan X, Li F, Zi W
J Neurointerv Surg 2023 Aug;15(8):808-813. Epub 2022 Aug 19 doi: 10.1136/jnis-2022-019112. PMID: 35985839Free PMC Article
"Look at my classifier's result": Disentangling unresponsive from (minimally) conscious patients.
Noirhomme Q, Brecheisen R, Lesenfants D, Antonopoulos G, Laureys S
Neuroimage 2017 Jan 15;145(Pt B):288-303. Epub 2015 Dec 12 doi: 10.1016/j.neuroimage.2015.12.006. PMID: 26690804
Validation of a new coma scale: The FOUR score.
Wijdicks EF, Bamlet WR, Maramattom BV, Manno EM, McClelland RL
Ann Neurol 2005 Oct;58(4):585-93. doi: 10.1002/ana.20611. PMID: 16178024
Consciousness.
Zeman A
Brain 2001 Jul;124(Pt 7):1263-89. doi: 10.1093/brain/124.7.1263. PMID: 11408323
Locked-in syndrome.
Sepcić J, Sepić-Grahovac D, Strenja-Grubesić J, Antonelli L, Andrasević D
Neurol Croat 1992;41(3):157-61. PMID: 1463800

Recent systematic reviews

Massive body-brain disconnection consequent to spinal cord injuries drives profound changes in higher-order cognitive and emotional functions: A PRISMA scoping review.
Moro V, Beccherle M, Scandola M, Aglioti SM
Neurosci Biobehav Rev 2023 Nov;154:105395. Epub 2023 Sep 20 doi: 10.1016/j.neubiorev.2023.105395. PMID: 37734697
Functional Near-Infrared Spectroscopy in the Study of Speech and Language Impairment Across the Life Span: A Systematic Review.
Butler LK, Kiran S, Tager-Flusberg H
Am J Speech Lang Pathol 2020 Aug 4;29(3):1674-1701. Epub 2020 Jul 8 doi: 10.1044/2020_AJSLP-19-00050. PMID: 32640168Free PMC Article
Effect of Exercise on Physical Recovery of People with Locked-In Syndrome after Stroke: What Do We Know from the Current Evidence? A Systematic Review.
Law YM, Feng LF, Liang Q, Meng LJ, Shen P, Yu SJ, Pao WY
Cerebrovasc Dis Extra 2018;8(2):90-95. Epub 2018 Jul 13 doi: 10.1159/000490312. PMID: 30007974Free PMC Article

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