Sleep in the completely locked-in state (CLIS) in amyotrophic lateral sclerosis.

Persons in the completely locked-in state (CLIS) suffering from amyotrophic lateral sclerosis (ALS) are deprived of many zeitgebers of the circadian rhythm: While cognitively intact, they are completely paralyzed, eyes mostly closed, with artificial ventilation and artificial nutrition, and social communication extremely restricted or absent. Polysomnographic recordings in eight patients in CLIS, however, revealed the presence of regular episodes of deep sleep during night time in all patients. It was also possible to distinguish an alpha-like state and a wake-like state. Classification of rapid eye movement (REM) sleep is difficult because of absent eye movements and absent muscular activity. Four out of eight patients did not show any sleep spindles. Those who have spindles also show K-complexes and thus regular phases of sleep stage 2. Thus, despite some irregularities, we found a surprisingly healthy sleep pattern in these patients.

Transient Incomplete Locked-In Syndrome Secondary to Supratentorial Gunshot Wound.

BACKGROUND: 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. CASE DESCRIPTION: 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. CONCLUSIONS: 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.

Comprehensive, technology-based, team approach for a patient with locked-in syndrome: A case report of improved function & quality of life.

One of the most severe types of stroke is locked-in syndrome (LIS) due to the loss of almost all voluntary motor functions and a high mortality rate. The majority of the literature regarding LIS is based on case reports that utilized multidisciplinary interventions focused on improving functional communication and respiratory care with minimal focus on motor retraining. These reports were neither dynamic nor multi-sensory, and the only technology utilized was in the form of augmentative communication. There are additional types of technology frequently used in the general stroke population that can address similar motor deficits that occur in the LIS population. This case report explains an interdisciplinary approach using motor and communication interventions that are multisensory, progressive, multi-modal, and technology- based. The length of stay was 153 days in acute rehabilitation, after which the patient returned home making significant gains in overall function. In this patient, the FIM changes in motor (+42), cognitive (+29) and total change score of (+71) surpassed what was determined to be a minimal clinically important difference. These results suggest that this treatment program and approach may be a key reason why this patient was able to achieve significant functional gains and report improved quality of life.