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locked-in syndrome; cognitive-motor dissociation
Etiology:
1) lesions involving the ventral pons bilaterally
2) basilar artery thrombosis or embolism
3) traumatic brain injury [3]
Clinical manifestations:
1) quadriplegia
2) loss of lower cranial nerve function
3) wakefulness & awareness of the environment
4) vertical eye movements & blinking are often preserved
- may be only mechanism for patient to communicate with the outside world
5) preserved tendon reflexes
6) Babinski's sign
7) abnormal pupillary reflexes
Laboratory:
- electroencephalography (EEG) is often normal
Radiology:
- 25% of patients with traumatic brain injury show MRI &/or EEG evidence of cognitive-motor dissociation [3]
Differential diagnosis:
- cataplexy
General
disease/disorder primarily affecting brain
syndrome
References
- Saunders Manual of Medical Practice, Rakel (ed),
WB Saunders, Philadelphia, 1996, pg 1074
- Medical Knowledge Self Assessment Program (MKSAP) 11, American
College of Physicians, Philadelphia 1998
- George J
One in Four Brain Injury Patients Who Appear Unresponsive Respond Covertly.
Functional MRI and EEG detect awareness in coma or vegetative states.
MedPage Today, August 14, 2024
https://www.medpagetoday.com/neurology/generalneurology/111520
- Bodien TG et al
Cognitive Motor Dissociation in Disorders of Consciousness.
Engl J Med. 2024. Aug 14.
PMID: 39141852
https://www.nejm.org/doi/full/10.1056/NEJMoa2400645