Headache Australia

Disorders

Vegetative State (Unresponsive Wakefulness Syndrome)

Description

On recovery from the coma state, VS/UWS is characterized by the return of arousal without signs of awareness (Multi-Society Task Force on PVS, 1994). In contrast, a coma is a state that lacks both awareness and wakefulness. Absence of awareness can only be inferred by lack of responsiveness to the environment and not as lack of consciousness that we may not be able to detect by behavioural measures. For this reason, many authors have suggested that the term ‘Unresponsive Wakefulness Syndrome’ (UWS) (Laureys et al., 2010) or ‘post-coma unresponsiveness’ (NHMRC, 2004) are more accurate descriptive terms for VS.

VS/UWS  patients can open their eyelids occasionally and demonstrate sleep-wake cycles, but they completely lack cognitive function. VS/UWS patients' eyes might be in a relatively fixed position, may track moving objects (visual pursuit), or move in a completely unsynchronized manner. Sleep-wake cycles may resume or patients may appear to be in a state of chronic wakefulness. They may grind their teeth, swallow, smile, shed tears, grunt, moan, or scream without any apparent external stimulus. VS/UWS patients do not respond to sound, hunger, or pain. Patients cannot obey verbal commands and lack local motor responses. Additionally VS/UWS patients cannot talk in comprehensible terms and may become noisy, restless, and hypermobile.

One of the most challenging tasks facing clinicians is that of differentiating VS/UWS from minimally conscious (MCS) states (eg Schnakers et al, 2009; Andrews et al, 1996). Whilst neuroimaging is widely used in assessing brain damage and functional abilities, behavioral assessment remains the "gold standard" for detecting signs of consciousness and thereby for determining diagnosis (Majerus et al. 2005).

Treatment

Careful, ongoing assessment of the patient, using empirically validated assessment tools (eg the Coma Recovery Scale-Revised) is essential in order to evaluate and measure signs of progress, improvement or deterioration. Treatment is addressed at presenting symptoms and the patient’s needs; VS/UWS patients require constant monitoring and assistance with feeding, hydration, hygiene, assisted movement and physical therapies (to help prevent ulcers and blood clots in the legs), and elimination of waste products.

Currently no treatment for VS/UWS exists that would satisfy the efficacy criteria of evidence-based medicine. Pharmacological methods, surgery, physical therapy, and various stimulation techniques have been suggested. Pharmacological therapy mainly uses activating substances such as tricyclic antidepressants or methylphenidate (Dolce et al. 2002). Surgical methods (eg. deep brain stimulation) are used infrequently due to the invasiveness of the procedures. Stimulation techniques include sensory stimulation, sensory regulation, music and musicokinetic therapy, social-tactile interaction, etc. While not empirically validated, families have reported benefits from arousal regimes, such as those implemented by Dr Ted Freeman (eg Coma Arousal Therapy). The therapy involves family members taking the patient through a regimen of controlled auditory, visual and physical stimulation for up to six hours a day every day.

Prognosis (outlook for recovery)

Many patients emerge spontaneously from VS/UWS within a few weeks (Jennett, 2007). The chances of recovery depend on the extent of injury to the brain and age, with younger patients having a better chance of recovery than older patients. Generally, adults have a circa 50 percent chance and children a 60 percent chance of recovering consciousness from VS/UWS within the first 6 months in the case of traumatic brain injury. For non-traumatic injuries such as strokes, the recovery rate falls to 14% at one year (Jennett, 2002). After this period the chances that VS/UWS patient will regain consciousness are very low and, of those patients who do recover consciousness, most experience significant disability. The longer a patient is in VS/UWS the more severe the resulting disabilities are likely to be.

Some patients who have entered a vegetative state (UWS) go on to regain a degree of awareness (see Minimally Conscious State). The likelihood of significant functional improvement for VS/UWS patients diminishes over time.

Further Information and Support

Click here for access to the Australian Register for Disorders of Consciousness (ARDoC)

For information about Coma Arousal Therapy, see Dr Edward (Ted) Freeman (1987) The Catastrophe of Coma. Publisher: David Bateman

Information

Coma Science Group
coma.ulg.ac.be

Families4Families – Acquired Brain Injury support network
Phone: 0433 388 250
Email:  This e-mail address is being protected from spambots. You need JavaScript enabled to view it
Web: families4families.org.au 

National Institute of Neurological Disorders and Stroke
www.ninds.nih.gov/health_and_medical/disorder_index.htm


ComaCare
comacare.com

ReNew Neurobehavioural Services
renewservices.org.au 

Brain Injury Resource Center
www.headinjury.com/coma

Reviewed by: Shannan Keen, MBMSc, Brain and Mind Research Institute, University of Sydney

 

Minimally Conscious State

Description

The minimally conscious state is a defined as severely altered consciousness in which minimal but definite, sustained and/or reproducible behavioral evidence of awareness of self or environment is demonstrated.

To make the diagnosis of MCS, limited but clearly discernible evidence of self or of environmental awareness must be demonstrated on a reproducible or sustained basis by one or more of the following behaviors:

  1. Follow simple commands (eg touch your nose, look up).
  2. Gestural or verbal yes/no responses (regardless of accuracy).
  3. Intelligible verbalization.
  4. Purposeful behavior that is not due to reflexive activity. Eg appropriate smiling or crying in response to emotional but not to neutral topics or stimuli – reaching for objects demonstrating a clear relationship between object location and direction of reach – touching or holding objects appropriately in relation to the size and shape of the object – pursuit eye movement or sustained fixation that occurs in direct response to moving or salient stimuli.

Treatment

As defined by Giacino et al (2002), “evaluation and management decisions will differ depending on the prognosis and the needs of the patient. In all circumstances, the patient should be treated with dignity, and caregivers should be cognizant of the patient’s potential for understanding and perception of pain. In early MCS, prevention of complications and maintenance of bodily integrity should be emphasized because of the likelihood of further improvement.” While not empirically validated, families have reported benefits from arousal regimes, such as those implemented by Dr Ted Freeman (eg Coma Arousal Therapy). The therapy involves family members taking the coma patient through a regimen of controlled auditory, visual and physical stimulation for up to six hours a day every day.

Giacino and colleagues report that “efforts should be made to establish functional communication and environmental interaction when possible. A person with experience in neurologic assessment of patients with impaired consciousness should be primarily responsible for establishing the diagnosis and prognosis and for coordinating clinical management. An additional opinion of a physician or other professional with particular expertise in the evaluation, diagnosis, and prognosis of patients in VS and MCS is recommended when the assessment will impact critical management decisions. Such decisions include, but are not limited to, those regarding changes in level of care, disputed treatment decisions, and withdrawal of life-sustaining treatment.”

Prognosis (outlook for recovery)

To date, the natural history and long-term outcome of MCS has not been adequately investigated. MCS may occur in a variety of neurologic conditions, such as traumatic brain injury, stroke, progressive degenerative disorders, tumors, neurometabolic diseases, and congenital or developmental disorders. Following an acute injury, MCS can be a transitional or permanent state. Although it is not known how many patients will emerge from MCS 12 months or more after injury, most patients in MCS for this length of time remain severely disabled as measured by the Glasgow Outcome Scale (Jennett & Bond, 1975). As with Vegetative State patients the likelihood of significant functional improvement for MCS patients diminishes over time.

Further Information and Support

Click here for access to the Australian Register for Disorders of Consciousness (ARDoC)

For information about Coma Arousal Therapy, see Dr Edward (Ted) Freeman (1987) The Catastrophe of Coma. Publisher: David Bateman

Information

Coma Science Group
coma.ulg.ac.be

Families4Families – Acquired Brain Injury support network
Phone: 0433 388 250
Email:  This e-mail address is being protected from spambots. You need JavaScript enabled to view it
Web: families4families.org.au  

National Institute of Neurological Disorders and Stroke
www.ninds.nih.gov/health_and_medical/disorder_index.htm


ComaCare
comacare.com

ReNew Neurobehavioural Services
renewservices.org.au

Brain Injury Resource Center
www.headinjury.com/coma

Reviewed by: Shannan Keen, MBMSc, Brain and Mind Research Institute, University of Sydney

 

Locked-in Syndrome (LiS)

Description

Locked-in Syndrome (LiS) results from a lesion to the brainstem, most frequently an ischemic pontine lesion. It results in severe impairments due to the complete disruption of the motor pathways controlling eyes, face, trunk and limb movements, including breathing, swallowing and phonation. However consciousness and cortical functions are preserved.

The American Congress of Rehabilitation Medicine (1995) defines LiS as a syndrome characterized by preserved awareness, relatively intact cognitive functions, and by the ability to communicate while being paralysed and voiceless.

Bauer described three categories of LIS (Bauer et al., 1979).

  • Complete or total LIS: Quadriplegia and anarthria. No eye movement.
  • Classic LIS: Preserved vertical eye movement and blinking.
  • Incomplete LIS: Recovery of some voluntary movements in addition to eye movements

Treatment

Early recognition of LIS state is important for rehabilitation. It is essential that LiS patients play an active role in decision-making processes regarding their rehabilitation. A reliable code to indicate yes and no usually constitutes the basis of communication. Initially many use eye movements and blinking to communicate. Because language skills are usually intact, many employ a system of spelling via an alphabetical code whereby the person with LIS indicates the desired letter by moving their eyes or blinking. (De Serres and Martel, 2008; Beukelman et al., 2007).

Early on, individuals with LIS need to be equipped with a means to indicate their needs or a desire to communicate. Should the patient regain control of a reliable movement, that could enable the activation of a switch enabling access to communication devices (eg computer with synthetic voice).

Initially, patients are essentially fed through a feeding tube. Following the initial assessment of their swallowing ability, most patients gradually start with eating smooth purees and thickened liquids. Tracheostomy, mechanical ventilation, cardiac failure, chronic obstructive pulmonary disease, uncontrolled emotional lability, improper positioning during feeding, undernutrition or dehydration are factors that may lead the healthcare team to remain cautious regarding feeding through the mouth.

To promote functional use of the patient’s emergent voluntary movements, it can be helpful to progressively move the patient into a more vertical position. Exercises to maintain range of motion, as well as breathing, eyes, head, trunk and limb control exercises are performed throughout the rehabilitation process. Stretching exercises, use of antispasmodic drugs or botulinum toxin injections help to decrease spasticity.

Prognosis (outlook for recovery)

According to the International Encyclopedia of Rehabilitation and researchers, recovery of horizontal eye movements prior to the first four weeks is associated with a good neurological prognosis (Bauer et al., 1979). Recovery of the oral-motor area (speech and swallowing) begins later and spreads over a longer period as does recovery of movement in distal parts of limbs of individuals (Janjua et al., 2005; De Serres and Martel, 2008).

The great majority of patients are weaned from their tracheostomy during the first months with one third able to utter isolated comprehensible words after the first year (Casanova et al., 2003). During inpatient rehabilitation, more than 50% of individuals start feeding orally and their gastrostomy is removed during the first year (Bruno et al., 2008; Beaudoin and De Serres, 2008; Casanova et al., 2003).

Level of independence is directly related to motor recovery. The majority of LiS patients can move around independently, most often in powered wheelchair. However very few  reach a level of motor recovery enabling them to feed and perform activities of daily living on their own (Beaudoin and De Serres, 2008). Even after several years, spasticity often remains a significant problem for many. Of note, individuals with LIS usually do not suffer from a pain syndrome during the chronic phase.

Further Information and Support

Click here for  the Australian Register for Disorders of Consciousness (ARDoC)

Click here for access to the blog of LiS individual, Tracey Gibb http://ridingwithlis.wordpress.com/

Information

Coma Science Group
http://www.coma.ulg.ac.be/index.html

Families4Families – Acquired Brain Injury support network
Phone: 0433 388 250
Email:  This e-mail address is being protected from spambots. You need JavaScript enabled to view it
Web: families4families.org.au

National Institute of Neurological Disorders and Stroke
www.ninds.nih.gov/health_and_medical/disorder_index.htm


ComaCare
comacare.com

ReNew Neurobehavioural Services
renewservices.org.au

Brain Injury Resource Center
www.headinjury.com/coma

Neural Signals Inc.
www.neuralsignals.com

The mission of Neural Signals Inc. is to provide brain/computer communication systems for individuals with LiS.
Neural Signals have designed the LISKeyMicro - a tiny device that helps the totally paralyzed use a computer to communicate.  www.LISKeyMicro.com

 

Reviewed by: Shannan Keen, MBMSc, Brain and Mind Research Institute, University of Sydney 

 

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