Locked-in syndrome: improvement in the prognosis after an early intensive multidisciplinary rehabilitation

doi:10.1016/S0003-9993(03)00008-X

Archives of Physical Medicine and Rehabilitation

Volume 84, Issue 6, June 2003, Pages 862-867

Presented in part at the National Congress of PM&R, October 2000, Napoli, Italy; the National Congress of Neurorehabilitation, October 2000, Riva del Garda, Italy; and the 4th World Congress on Brain Injury, May 2001, Torino, Italy.

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Abstract

Casanova E, Lazzari RE, Lotta S, Mazzucchi A. Locked-in syndrome: improvement in the prognosis after an early intensive multidisciplinary rehabilitation.

Objective:

To evaluate prognosis and recovery in patients with locked-in syndrome (LIS) receiving early intensive rehabilitative care.

Design:

Consecutive sample and follow-up for 5 months to 6 years.

Setting:

Three rehabilitation centers in Italy.

Participants:

Fourteen patients with LIS who underwent the same treatment and subsequently recovered.

Interventions:

Intensive nursing care and intensive and early rehabilitative program, including physiotherapy and respiratory, swallowing, and speech training. For 4 patients, occupational therapy was performed; 4 subjects also had oculomotor training. After discharge, rehabilitative maintenance care continued for each patient.

Main Outcome Measures:

Motor recovery according to the Patterson and Grabois classification, functional improvement, and mortality rate.

Results:

A significant motor recovery was found in 21% of subjects, within 3 to 6 months of onset of the morbid event; complete swallow recovery in 42%; verbal communication in 28%; communication through devices in 42%; effective bladder and bowel control in 35%; and good breathing patterns in 50%. At follow-up, the mortality rate was 14% and only 2 complications were reported.

Conclusions:

Intensive and early rehabilitation, begun within about 1 month of the morbid event, improved the functional recovery and reduced the mortality rate, which, as reported in the literature, had been 60% about 10 years ago. Further studies are necessary to confirm these data.

Section snippets

Participants

From 1995 until 2001, 14 patients with LIS, at different times and at different periods after the morbid event, were hospitalized in the 3 participating centers and selected for this study. The follow-up has continued for a range of time between 5 months and 6 years—at the beginning during the hospitalization and subsequently by telephone contact.

We decided not to establish a control group, for ethical reasons and because of the rarity of LIS. Hence, all patients with LIS were admitted to the

Results

The subjects with LIS were 9 men and 5 women (table 3); their mean age at onset was 44.7 years (range, 16–71y). The etiology was vascular in 11 cases and traumatic in the remaining 3 cases. Disorders of protein S, unknown before the illness, were found in 2 patients, and intake of drugs, such as hormone, was discovered in the history of 2 others. Moreover, before the onset of LIS, 1 patient was positive for HIV (human immunodeficiency virus) and 2 others had a diagnosis of hypertension.

Discussion

The population with LIS analyzed in our 3 centers does not appear substantially dissimilar from patients with LIS described elsewhere in the literature. In fact, in our population, the mean age at onset was 44.7 years; 9 were men (64%) and 5 (35%) women, and the etiology was vascular in 11 cases (78%) and traumatic in 3 (21%). In Patterson and Grabois’s literature review,4 the mean age of patients with LIS was 52 years, 85 (62%) were men and 52 (38%) were women, and the most common etiologic

Conclusions

After studying a consecutive sample of 14 patients with LIS hospitalized in our centers, and after comparing our results with the data reported in the literature, it seems that an intensive and early multidisciplinary rehabilitative treatment, begun, when possible, within about 1 month of the onset of the morbid event, improved health status at follow-up and reduced the mortality rate below 60%, as reported by several researchers about 10 years ago. In addition, in our case series, the recovery

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There are more references available in the full text version of this article.

Cited by (82)

Transient Incomplete Locked-In Syndrome Secondary to Supratentorial Gunshot Wound
2019, World Neurosurgery
Locked-in syndrome (LIS) is a rare neurologic disorder characterized as quadriplegia with anarthria. The diagnosis of LIS is challenging and requires a high index of suspicion. The syndrome is typically caused by an infratentorial lesion to the ventral pons, regardless of etiology. LIS secondary to supratentorial injury is extremely rare, and to our knowledge, this is the first reported case.
We report the case of a 26-year-old woman who sustained a gunshot to the left suboccipital area, with supratentorial extension. A diagnosis of incomplete LIS was made on the day of admission, with eye movement preservation. Imaging studies confirmed bilateral injury of the motor homunculus. The clinical course was that of progressive improvement, aided by intensive care unit (ICU) supportive care and early physiotherapy rehabilitation. Her condition improved, and she was discharged to a rehabilitation facility at the end of week 7 postadmission.
This is a unique case of incomplete LIS after supratentorial injury. Initial ICU care and early rehabilitation likely played a major role in the full recovery of this patient. The influence of etiology and site of injury on outcome prognosis is also suggested. Although severe diffuse brain injury may occur in the face of an unremarkable computed tomography (CT) scan, the emerging role of magnetic resonance imaging in optimally evaluating traumatic brain injury with discordant clinical and CT information is highlighted and is useful in cases of LIS where prognosis prediction is important.
Consciousness in the Locked-In Syndrome
2015, The Neurology of Consciousness: Cognitive Neuroscience and Neuropathology
Patients in a locked-in syndrome (LIS) are selectively de-efferented (i.e., they have no means of producing speech, limb, or face movements). Usually the anatomy of the responsible lesion in the brainstem is such that locked-in patients are left with the capacity to use vertical eye movements and blinking to communicate with the external world. The syndrome is subdivided as: (i) classical LIS, which is characterized by total immobility except for vertical eye movements or blinking; (ii) incomplete LIS, which allows remnants of voluntary motion such as head or finger movements; and (iii) total LIS, which is a complete immobility including all eye movements. In all three cases, consciousness is usually fully preserved. Eye-controlled computer-based communication and brain-computer interface technology currently allows these patients to control their environment, use a word processor coupled to a speech synthesizer and access the world wide net. Available literature raises important ethical considerations in terms of quality of life and end-of-life decisions in such challenging patients.
Transcranial direct current stimulation for a patient with locked-in syndrome
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2024, American Journal of Case Reports
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2023, Therapeutic Advances in Neurological Disorders
Demographic, Medical, and Clinical Characteristics of a Population-Based Sample of Patients With Long-lasting Locked-In Syndrome
2023, Neurology

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ArticleLocked-in syndrome: improvement in the prognosis after an early intensive multidisciplinary rehabilitation1

Abstract

Objective:

Design:

Setting:

Participants:

Interventions:

Main Outcome Measures:

Results:

Conclusions:

Section snippets

Participants

Results

Discussion

Conclusions

J Neurol Sci

The diagnosis of stupor and coma

Varieties of locked-in syndrome

J Neurol

Long-term survival, prognosis, and life-care planning for 29 patients with chronic locked-in syndrome

Arch Phys Med Rehabil

Locked-in syndromea review of 139 cases

Stroke

Article
Locked-in syndrome: improvement in the prognosis after an early intensive multidisciplinary rehabilitation¹