Locked in syndrome: A Case Report and Discussion
Sep 17 2012-
- Details
Abstract
Locked in syndrome: A Case Report and Discussion
Authors:
1. Thomas Dewhurst: FY2, Royal Gwent Hospital
2. Yaqoob Bhat: Consultant Stroke Physician, Royal Gwent Hospital, Newport, UK
Introduction
Locked-in Syndrome is the devastating result of insult to the brainstem. It has recently become well known thanks to public figures such as Jean-Dominique Bauby, with his autobiographical account The Diving Bell and the Butterfly, and Tony Nicklinson, who has recently lost his ‘right-to-die’ case in the High Court (Nicklinson 2012). This raised awareness is welcome when early diagnosis is essential for a good prognosis.
Case Report
53 year old Mr P was admitted to the Emergency Department AT Royal Gwent Hospital in Newport, with occipital headache, mild paresthesia on left side and epigastric pain. He was smoker but has no significant past medical history apart from chronic bronchitis. He was triaged as a medical patient because of associated epigastric pain and was investigated for gastrointestinal pathology.
Four hours later Mr P developed profound weakness on left sided and examination showed reduced power and impaired sensation on the left side. Urgent CT head revealed a chronic left temporal lobe infarct but no acute changes. By now he was outside the thrombolysis window and was treated with antiplatelet therapy.
His condition deteriorated overnight and arrest call was made. He was intubated and was sent to intensive care unit. It became apparent clinically that he had developed quadriplegia likely secondary to brain stem infarct. He began biting down on the endotracheal tube due to increasing tone in his masseter muscles so therefore received a tracheostomy.
|
Fig. 1 |
Once consistent communication was confirmed, Mr P was diagnosed with Locked-in Syndrome. He was transferred to the Acute Stroke Ward with PEG tube, oxygenated tracheostomy, cathether and IV access in situ. He remains unable to move all four limbs but has retained consciousness and insight, confirmed by communicating by vertical gaze: up for “Yes”, down for “No”. He had recurrent chest infections during his admission likely due to poor oral hygiene secondary to increased tone in masseter muscles bilaterally. Despite bilateral botox injections to the masseters, his mandible is clamped down and his oral cavity remains inaccessible (locked mouth).
He was discharged to nursing home after prolonged admission.
Discussion
The term “locked-in” was coined by Plum and Posner in 1966. Over the years definitions have evolved and the American Congress of Rehabilitation Medicine in 1995 agreed on the following attributes:
1. Sustained eye opening (bilateral ptosis should be ruled out as a complicating factor)
2. Preserved basic cognitive abilities
3. Aphasia or sever hypophonia
4. Quadriplegia or quadriparesis
5. A primary mode of communication that uses vertical or lateral eye movement or blinking of the upper eyelid (ACRM 1995).
Despite the clear definition, there still remains great variability in the syndrome. Bauer et al. (1979) subdivided the syndrome into three types: Classical: Defined by total immobility except for vertical eye movements or blinking. Incomplete: Where remnants of voluntary movements remain. Total: Where there is complete immobility including eye movements. The underlying feature is a preserved consciousness.
The commonest cause of Locked-in Syndrome is stroke but trauma, neoplasia, autoimmune, metabolic and infectious mechanisms can all lead to Locked-in Syndrome. In each case corticospinal and corticobulbar tracts are disrupted, preventing voluntary movement and speech, while consciousness and cognition, controlled by anterior areas of the brain, is preserved. As the tegmentum of the pons is also spared, patients can achieve vertical eye movements and/or blinking (Smith and Delargy 2005). This becomes the patient’s sole mode of communication and allows family and healthcare professionals to recognise consciousness.
Good management depends on a swift diagnosis, which by definition relies on the patient’s ability to communicate. León-Carrión et al. (2002) found that 55% of the time relatives are the first to recognise awareness, and the average time until diagnosis is 78 days. Standard acute stroke screening is essential (blood tests, ECG, Chest Xray, CT/MRI head), and a CT/MR angiogram of aortic arch/carotids will confirm posterior circulation arteriopathy. The airway must be protected, reversible medical causes and risk factors must be addressed, and immobility, dysphagia and incontinence must be assessed by the multidisciplinary team in an appropriate setting, such as an Acute Stroke Unit.
A 139 patient review (Patterson and Grabois 1986) suggested 60% mortality, greatest in the first 4 months, with vascular causes doing worse than non-vascular causes. Another study has revealed that 40-70% of patients die in the early stages, usually of pulmonary complications of dysphagia, immobility and respiratory muscle dysfunction (Haig et al, 1987). Those who survive are usually younger, and those who commenced rehabilitation within one month had a mortality of only 14% at five years (Casanova et al. 2003). This only highlights the importance of rapid diagnosis and referral for neurorehabilitation. With recent advances in neurorehabilitation ten-year survival rates of up to 80% have been reported and motor recovery, respiratory improvement, emergence of swallow, basic verbal communication and re-established continence have all been documented in survivors (Smith and Delargy 2005).
|
Fig. 2 (Smith and Delargy 2003) |
The aim of rehabilitation is to regain some degree of a fulfilling life. Physiotherapists must monitor for recovery of thumb, finger, head and neck movement and help to improve respiratory function. Speech and Language Therapists must aim for an independent swallow and develop communication. Advances with communication boards (such as the AEIOU alphabet board, Fig. 2) and innovative computer devices have lead to patients with Lock-in Syndrome leading interactive lives.
This Case Report has been presented to highlight the importance of early diagnosis because Mr P missed the early diagnosis due to associated epigatric pain. His background history of chronic bronchitis and locked mouth secondary to increased tone masseter muscles made it impossible to discharge him early because of repeated chest infection. Once the diagnosis is made it is important to use the communication tools because of preserved perception and are patients receive appropriate rehabilitation before medical complications obstruct their road to recovery. Mr P missed the early diagnosis because of associated epigatsrtic pain Thus the role of the multidisciplinary team is to unlock the patient.
References
Nicklinson, T. 2012. My Right to Choose When I Die. The Independent [Online]. Available at:
[Accessed 13/03/12]
Plum F. and Posner J. B. 1966. The Diagnosis of Stupor and Coma. Philadelphia, PA: FA Davis.
American Congress of Rehabilitation Medicine (ACRM) 1995. Recommendations for use of uniform nomenclature pertinent to patients with severe alterations of consciousness. Arch. Phys. Med. Rehabil. 76 Pg 205-209
Bauer, G. et al. 1979. Varieties of Locked-in Syndrome. J Neurol. 221 Pg 77-91
Smith, E. and Delargy, M. 2005. Clinical Review Locked-in Syndrome. BMJ 330 Pg 406-409
León-Carrión, J. et al. 2002. The Locked-in Syndrome: a Syndrome Looking for a Therapy. Brain Inj. 16(7) Pg 571-582
Patterson, J. R. and Grabois, M. 1986. Locked-in Syndrome: a Review of 139 Cases. Stroke 17 Pg 758-764
Haig, A. J. et al. 1987. Mortality and Complications of the Locked-in Syndrome. Arch. Phys. Med. Rehabil. 68(1) Pg 24-27
Casanova, E. et al. 2003. Locked-in Syndrome: Improvement in the Prognosis after an Intensive Multidisciplinary Rehabilitation. Arch. Phys. Med. Rehabil. 84(6) Pg 862-867
