Locked-in syndrome
http://www.100md.com
《英国医生杂志》
1 National Rehabilitation Hospital, Dun Laoghaire, County Dublin, Ireland
Correspondence to: E Smith eimear.smith@nrh.ie
Introduction
We gathered information for this article through searches in Medline and Taylor and Francis Health Sciences, identifying relevant case series reviews on the locked-in syndrome and other brain stem strokes. Our own experience is also incorporated.
Clinical features
Locked-in syndrome has been classified into three categories11:
Classic—Quadriplegia and anarthria with preserved consciousness and vertical eye movement
Incomplete—The same as classic but with remnants of voluntary movement other than vertical eye movement
Total—Total immobility and inability to communicate, with full consciousness.
This classification has been referred to in previous case series reports,3 8 9 but no management details are given which differentiate the categories. Each of the three categories has been subdivided into transient and chronic forms.11 Patients with transient locked-in syndrome improve neurologically; an episode of locked-in syndrome that lasted only a few minutes, with good recovery, has been reported.12
Diagnosis and early management
Early literature, primarily relying on autopsy findings, reported that long term survival was rare without neurological recovery. In 1986, mortality was estimated at 60%, being greatest in the first four months and higher in patients with vascular insult than non-vascular causes.13 Survivors tended to be younger at age of onset. Earlier rehabilitation and more effective nursing care have recently been reported to reduce mortality from acute locked-in syndrome.9 Casanova et al reported that patients with locked-in syndrome who began rehabilitation within one month of the acute event had a mortality of only 14% at five years.9 Although most survivors remain either in a chronic locked-in state or severely impaired, early signs of recovery can be exploited through multidisciplinary rehabilitation.2 3 8 9 13 14 In our rehabilitation programme, we monitor for recovery of thumb, finger, head, and neck movement; evidence of independent swallow; and improvement in respiratory function. Any movement that may enable the patient to use a buzzer, an environmental control, or a communication device is targeted.15
Patients initially tire quickly when using vertical eye movement to communicate. Furthermore, their attention span may be severely limited in the first few weeks or months. An agreed system of interpretation is necessary, where one upward movement signifies yes and two rapid upward movements, no. Effective questioning skills must be developed, avoiding open ended questions and confirming answers by repeating questions when necessary. Aggressive treatment of infections, respiratory difficulties, pain, or localised problems such as corneal abrasions can enhance physical stamina and communication. Because patients with classic locked-in syndrome are unable to call for attention or initiate conversation, they should frequently be given the opportunity to communicate and, indeed, end dialogue.
The only aspect of locked-in syndrome recovery for which we found a classification is motor recovery (box).13 No specific classification systems exist for vocal, dysphagia, cognitive, emotional, or behavioural recoveries. A retrospective review of 53 patients with other brain stem strokes used the modified Barthel index to measure functional outcomes such as limb weakness, ataxia, dysarthria, dysphasia, and urinary continence, but emotional and behavioural recoveries remain unclear.16
Table 2 summarises the findings from three studies examining life expectancy and functional recovery in patients with locked-in syndrome.8 9 14 This overview suggests that with return of greater respiratory effort, some swallowing ability, and improved continence, the need for tracheostomies, gastrostomies, and urinary catheters reduces with time.8 9 All patients with locked-in syndrome should be rehabilitated in a national or regional specialist centre that has specific multidisciplinary rehabilitation experience with the condition. Although cognitive ability should not be overestimated, survivors' views regarding the focus of acute treatment, rehabilitation goals, and life choices should be formally sought.
Table 2 Findings from three case series of patients with locked-in syndrome. Values are numbers (percentages) unless stated otherwise
Classification of recovery of motor function13
No recovery—No return of motor function, total dependence for all activities of daily living
Minimal recovery—Minimal motor return, total dependence for all activities of daily living
Moderate recovery—Moderate motor return, independence in some but not all activities of daily living
Full recovery—Independence in all activities of daily living but some minimal neurological deficit
No neurological deficit—No reported residual deficits
Development of communication devices
Many patients choose to return to live at home, which presumably enables greater social interaction with family and friends.8 9 14 Return to home life may positively influence the patient's desire to live.14 However, it places a long term physical and psychological burden on the family. Limited funding means that community care is often scarce and the carers are poorly supported.
We found only two references to patients who returned to work. The first was a lawyer who used morse code blinks to provide legal opinions and the second was someone who taught maths and spelling using a mouth stick to trigger an electronic voice device.14
Patient information
Bauby J-D. The diving bell and the butterfly: a memoir of life in death. New York: Vintage, 1996.
Association for the Locked-in Syndrome (ALIS) (www.club-internet.fr/alis)
National Institute of Neurological Disorders and Stroke (www.ninds.nih.gov/disorders/lockedinsyndrome/lockedinsyndrome.htm)
In the only review of quality of life, the authors found that a series of seven patients with locked-in syndrome had a worse quality of life on the Spitzer quality of life index than cancer patients but better than terminally ill patients; of the five who completed the general health questionnaire, three were depressed, but they all stated that they would want antibiotics if they developed pneumonia.18 In the longest surviving group of patients reviewed (after 11 years) 54% had never considered euthanasia, 46% had previously considered it, and none had a "not for resuscitation" order.14 The finding that locked-in syndrome survivors who remain severely disabled rarely want to die14 counters a popular misconception that such patients would have been better off dead.
Contributors: MD had the idea for the article. ES did the literature search and wrote the article with input from MD. ES is the guarantor.
Competing interests: None declared.
References
Plum F, Posner JB. The diagnosis of stupor and coma. Philadelphia, PA: FA Davis, 1966.
Haig AJ, Katz RT, Sahgal V. Mortality and complications of the locked-in syndrome. Arch Phys Med Rehabil 1987;68: 24-7.
Katz RT, Haig AJ, Clark BB, Di Paolo RJ. Long-term survival, prognosis and life-care planning for 29 patients with chronic locked-in syndrome. Arch Phys Med Rehabil 1992;73: 403-8.
Garrard P, Bradshaw D, Jager HR, Thompson AJ, Losseff N, Playford D. Cognitive dysfunction after isolated brain stem insult. An underdiagnosed cause of long term morbidity. J Neurol Neurosurg Psychiatry 2002;73: 191-4.
Leon-Carrion J, van Eeckhout P, Dominguez-Morales Mdel R, Perez Santamaria FJ. The locked-in syndrome: a syndrome looking for a therapy. Brain Inj 2002;16: 571-82.
Allain P, Joseph PA, Isambert JL, Le Gall D, Emile J. Cognitive functions in chronic locked-in syndrome: a report of two cases. Cortex 1998;34: 629-34.
Zeman A. What is consciousness and what does it mean for the persistent vegetative state? Adv Clin Neurosci Rehabil 2003;3: 12-4.
Richard I, Pereon Y, Guiheneu P, Nogues B, Perrouin-Verbe B, Mathe JF. Persistence of distal motor control in the locked-in syndrome. Review of 11 patients. Paraplegia 1995;33: 640-6.
Casanova E, Lazzari RE, Latta S, Mazzucchi A. Locked-in syndrome: improvement in the prognosis after an early intensive multi-disciplinary rehabilitation. Arch Phys Med Rehabil 2003;84: 862-7.
Fishburn MJ, Marino RJ, Ditunno JF. Atelectasis and pneumonia in acute spinal cord injury. Arch Phys Med Rehabil 1990;71: 197-200.
Bauer G, Gerstenbrand F, Rumpl E. Varieties of locked-in syndrome. J Neurol 1979;221: 77-91.
Khurana RK, Genut AA, Yannakis GD. Locked-in syndrome with recovery. Ann Neurol 1980;8: 439-41.
Patterson JR, Grabois M. Locked-in syndrome:a review of 139 cases. Stroke 1986;17: 758-64.
Doble JE, Haig AJ, Anderson C, Katz R. Impairment, activity, participation, life satisfaction and survival in persons with locked-in syndrome for over a decade. J Head Trauma Rehabil 2003;5: 435-44.
S?derholm S, Meinander M, Alaranta H. Augmentative and alternative communication methods in locked-in syndrome. J Rehabil Med 2001;33: 235-9.
Chua KSG, Kong KH. Functional outcome in brain stem stroke patients after rehabilitation. Arch Phys Med Rehabil 1996;77: 194-7.
Wu Y, Voda JA. User-friendly communication board for non-verbal, severely physically, disabled individuals. Arch Phys Med Rehabil 1985;66: 82-8.
Anderson C, Dillon C, Burns R Life-sustaining treatment and locked-in syndrome. Lancet 1993;342: 867-8.
((Eimear Smith, specialist registrar in re)
Correspondence to: E Smith eimear.smith@nrh.ie
Introduction
We gathered information for this article through searches in Medline and Taylor and Francis Health Sciences, identifying relevant case series reviews on the locked-in syndrome and other brain stem strokes. Our own experience is also incorporated.
Clinical features
Locked-in syndrome has been classified into three categories11:
Classic—Quadriplegia and anarthria with preserved consciousness and vertical eye movement
Incomplete—The same as classic but with remnants of voluntary movement other than vertical eye movement
Total—Total immobility and inability to communicate, with full consciousness.
This classification has been referred to in previous case series reports,3 8 9 but no management details are given which differentiate the categories. Each of the three categories has been subdivided into transient and chronic forms.11 Patients with transient locked-in syndrome improve neurologically; an episode of locked-in syndrome that lasted only a few minutes, with good recovery, has been reported.12
Diagnosis and early management
Early literature, primarily relying on autopsy findings, reported that long term survival was rare without neurological recovery. In 1986, mortality was estimated at 60%, being greatest in the first four months and higher in patients with vascular insult than non-vascular causes.13 Survivors tended to be younger at age of onset. Earlier rehabilitation and more effective nursing care have recently been reported to reduce mortality from acute locked-in syndrome.9 Casanova et al reported that patients with locked-in syndrome who began rehabilitation within one month of the acute event had a mortality of only 14% at five years.9 Although most survivors remain either in a chronic locked-in state or severely impaired, early signs of recovery can be exploited through multidisciplinary rehabilitation.2 3 8 9 13 14 In our rehabilitation programme, we monitor for recovery of thumb, finger, head, and neck movement; evidence of independent swallow; and improvement in respiratory function. Any movement that may enable the patient to use a buzzer, an environmental control, or a communication device is targeted.15
Patients initially tire quickly when using vertical eye movement to communicate. Furthermore, their attention span may be severely limited in the first few weeks or months. An agreed system of interpretation is necessary, where one upward movement signifies yes and two rapid upward movements, no. Effective questioning skills must be developed, avoiding open ended questions and confirming answers by repeating questions when necessary. Aggressive treatment of infections, respiratory difficulties, pain, or localised problems such as corneal abrasions can enhance physical stamina and communication. Because patients with classic locked-in syndrome are unable to call for attention or initiate conversation, they should frequently be given the opportunity to communicate and, indeed, end dialogue.
The only aspect of locked-in syndrome recovery for which we found a classification is motor recovery (box).13 No specific classification systems exist for vocal, dysphagia, cognitive, emotional, or behavioural recoveries. A retrospective review of 53 patients with other brain stem strokes used the modified Barthel index to measure functional outcomes such as limb weakness, ataxia, dysarthria, dysphasia, and urinary continence, but emotional and behavioural recoveries remain unclear.16
Table 2 summarises the findings from three studies examining life expectancy and functional recovery in patients with locked-in syndrome.8 9 14 This overview suggests that with return of greater respiratory effort, some swallowing ability, and improved continence, the need for tracheostomies, gastrostomies, and urinary catheters reduces with time.8 9 All patients with locked-in syndrome should be rehabilitated in a national or regional specialist centre that has specific multidisciplinary rehabilitation experience with the condition. Although cognitive ability should not be overestimated, survivors' views regarding the focus of acute treatment, rehabilitation goals, and life choices should be formally sought.
Table 2 Findings from three case series of patients with locked-in syndrome. Values are numbers (percentages) unless stated otherwise
Classification of recovery of motor function13
No recovery—No return of motor function, total dependence for all activities of daily living
Minimal recovery—Minimal motor return, total dependence for all activities of daily living
Moderate recovery—Moderate motor return, independence in some but not all activities of daily living
Full recovery—Independence in all activities of daily living but some minimal neurological deficit
No neurological deficit—No reported residual deficits
Development of communication devices
Many patients choose to return to live at home, which presumably enables greater social interaction with family and friends.8 9 14 Return to home life may positively influence the patient's desire to live.14 However, it places a long term physical and psychological burden on the family. Limited funding means that community care is often scarce and the carers are poorly supported.
We found only two references to patients who returned to work. The first was a lawyer who used morse code blinks to provide legal opinions and the second was someone who taught maths and spelling using a mouth stick to trigger an electronic voice device.14
Patient information
Bauby J-D. The diving bell and the butterfly: a memoir of life in death. New York: Vintage, 1996.
Association for the Locked-in Syndrome (ALIS) (www.club-internet.fr/alis)
National Institute of Neurological Disorders and Stroke (www.ninds.nih.gov/disorders/lockedinsyndrome/lockedinsyndrome.htm)
In the only review of quality of life, the authors found that a series of seven patients with locked-in syndrome had a worse quality of life on the Spitzer quality of life index than cancer patients but better than terminally ill patients; of the five who completed the general health questionnaire, three were depressed, but they all stated that they would want antibiotics if they developed pneumonia.18 In the longest surviving group of patients reviewed (after 11 years) 54% had never considered euthanasia, 46% had previously considered it, and none had a "not for resuscitation" order.14 The finding that locked-in syndrome survivors who remain severely disabled rarely want to die14 counters a popular misconception that such patients would have been better off dead.
Contributors: MD had the idea for the article. ES did the literature search and wrote the article with input from MD. ES is the guarantor.
Competing interests: None declared.
References
Plum F, Posner JB. The diagnosis of stupor and coma. Philadelphia, PA: FA Davis, 1966.
Haig AJ, Katz RT, Sahgal V. Mortality and complications of the locked-in syndrome. Arch Phys Med Rehabil 1987;68: 24-7.
Katz RT, Haig AJ, Clark BB, Di Paolo RJ. Long-term survival, prognosis and life-care planning for 29 patients with chronic locked-in syndrome. Arch Phys Med Rehabil 1992;73: 403-8.
Garrard P, Bradshaw D, Jager HR, Thompson AJ, Losseff N, Playford D. Cognitive dysfunction after isolated brain stem insult. An underdiagnosed cause of long term morbidity. J Neurol Neurosurg Psychiatry 2002;73: 191-4.
Leon-Carrion J, van Eeckhout P, Dominguez-Morales Mdel R, Perez Santamaria FJ. The locked-in syndrome: a syndrome looking for a therapy. Brain Inj 2002;16: 571-82.
Allain P, Joseph PA, Isambert JL, Le Gall D, Emile J. Cognitive functions in chronic locked-in syndrome: a report of two cases. Cortex 1998;34: 629-34.
Zeman A. What is consciousness and what does it mean for the persistent vegetative state? Adv Clin Neurosci Rehabil 2003;3: 12-4.
Richard I, Pereon Y, Guiheneu P, Nogues B, Perrouin-Verbe B, Mathe JF. Persistence of distal motor control in the locked-in syndrome. Review of 11 patients. Paraplegia 1995;33: 640-6.
Casanova E, Lazzari RE, Latta S, Mazzucchi A. Locked-in syndrome: improvement in the prognosis after an early intensive multi-disciplinary rehabilitation. Arch Phys Med Rehabil 2003;84: 862-7.
Fishburn MJ, Marino RJ, Ditunno JF. Atelectasis and pneumonia in acute spinal cord injury. Arch Phys Med Rehabil 1990;71: 197-200.
Bauer G, Gerstenbrand F, Rumpl E. Varieties of locked-in syndrome. J Neurol 1979;221: 77-91.
Khurana RK, Genut AA, Yannakis GD. Locked-in syndrome with recovery. Ann Neurol 1980;8: 439-41.
Patterson JR, Grabois M. Locked-in syndrome:a review of 139 cases. Stroke 1986;17: 758-64.
Doble JE, Haig AJ, Anderson C, Katz R. Impairment, activity, participation, life satisfaction and survival in persons with locked-in syndrome for over a decade. J Head Trauma Rehabil 2003;5: 435-44.
S?derholm S, Meinander M, Alaranta H. Augmentative and alternative communication methods in locked-in syndrome. J Rehabil Med 2001;33: 235-9.
Chua KSG, Kong KH. Functional outcome in brain stem stroke patients after rehabilitation. Arch Phys Med Rehabil 1996;77: 194-7.
Wu Y, Voda JA. User-friendly communication board for non-verbal, severely physically, disabled individuals. Arch Phys Med Rehabil 1985;66: 82-8.
Anderson C, Dillon C, Burns R Life-sustaining treatment and locked-in syndrome. Lancet 1993;342: 867-8.
((Eimear Smith, specialist registrar in re)