New Guideline for Minimally Conscious, Vegetative States Released

Batya Swift Yasgur, MA, LSW

August 09, 2018

Three specialty societies have published a new practice guideline for managing prolonged disorders of consciousness (DoC), focusing on accurate diagnosis, prognosis, and treatment.

The guidelines, issued by the American Academy of Neurology, the American Congress of Rehabilitation Medicine (ACRM), and the National Institute on Disability, Independent Living, and Rehabilitation Research, updates the earlier 1994 AAN practice parameter on persistent vegetative state and the 2002 case definition for the minimally conscious state (MCS).

"A major motivation for the new guideline was that, since [publication of] the original guidelines, a lot of work has been done and it became clear that some of the recommendations we've been working from probably no longer hold true," lead author Joseph T. Giacino, PhD, director, rehabilitation neuropsychiatry and associate professor, Department of Physical Medicine and Rehabilitation, Harvard Medical School, Boston, Massachusetts, told Medscape Medical News.

"Misdiagnosis [of DoC] is common because underlying impairments can mask awareness — in fact, there is a 40% rate of misdiagnosis, leading to inappropriate care decisions as well as poor health outcomes." he said.

A summary of the guideline, together with an accompanying literature review on which the guideline was based, was published online August 8 in Neurology.

Recommendation 1: Treatment Setting 

Care for patients with prolonged DoC (ie, lasting ≥28 days) should be provided in a specialized setting and be managed by a multidisciplinary team of knowledgeable clinicians.

The setting should be able to provide effective monitoring and rehabilitative care (level B evidence).

"This is a very complex area, and some clinicians may not have adequate training, so an important call is to ensure that specialized people be involved in the diagnostic and treatment process," Giacino commented.

He encouraged clinicians to "ensure to the extent possible that patients with DoC are moved from the ICU [intensive care unit] or acute care to a setting that will be capable of providing that type of surveillance."

Diagnostic Approaches (Recommendations 2a to 2f)

2a. Use valid and reliable standardized neurobehavioral assessment scales, such as those recommended by ACRM, rather than relying on bedside examination (level B). 

2b. To reduce diagnostic error, serial assessments should be conducted, with reassessment intervals informed by "individual clinical circumstances" (level B).

"We now recognize that a one-time exam increases the risk of misdiagnosis," Giacino noted.

2c. Attempt to increase arousals before performing assessments whenever diminished arousal is suspected or observed (level B).

2d. Before establishing a final diagnosis, Identify and treat any condition that may confound diagnostic accuracy (level B).

2e. In the event of continued ambiguity, use multimodal imaging and electrophysiologic evaluations to assess for evidence of awareness (level C).

2f. If there is no behavioral evidence of consciousness on clinical examination, but neuroimaging or electrophysiologic testing suggests the possibility of preserved conscious awareness, conduct frequent neurobehavioral re-evaluations to identify emerging signs of conscious awareness (Level C).

In these situations, decisions to reduce the intensity of rehabilitation treatment, if relevant, may be delayed (Level C).

Prognostic Issues (3 to 10)

3. When discussing prognosis with caregivers of patients with DoC during the first 28 days after injury, avoid statements suggesting that these patents "have a universally poor prognosis" (level A).

"Approximately 20% of individuals who have disturbance in consciousness from trauma regain functional independence between 2 and 5 years post-injury, even though they may not return to work or pretrauma functioning," Giacino commented.

4. Serial standardized behavioral evaluations should be performed in patients with DoC to "identify trends in the trajectory of recovery that are important for establishing prognosis" (level B).

5. At 2 to 3 months after injury in patients in with posttraumatic vegetative state (VS)/ unresponsive wakefulness syndrome (UWS), use the Disability Rating Scale (level B); assess for the presence of P300 (level C); or assess electroencephalography reactivity (level C) to inform prognostication of 12-month recovery of consciousness.

Perform MRI and single-photon emission computerized tomography 6 to 8 weeks and 1 to 2 months after injury, respectively, to inform prognostication regarding recovery of consciousness and degree of disability/recovery at 12 months (level B).

Blood oxygen level–dependent functional MRI may be used to assess for the presence of higher-level activation of the auditory association in response to a familiar voice speaking the patient's name (level C).

6. The Coma Recovery Scale–Revised in nontraumatic, postanoxic VS/UWS (level B) and the somatosensory evoked potentials (level C) can assist in prognostication regarding recovery of consciousness at 24 months.

7. Patients with nontraumatic VS/UWS frequently recover consciousness after 3 months, and after 12 months in patients with traumatic VS/UWS. In light of this, use of the term "permanent VS" should be discontinued.

After these time points, the term "chronic VS/UWS" should be used, accompanied by the duration of the VS/UWS (level B).

Prognostic Counseling (Recommendations 8 to 10)

8. Counsel families that MCSs diagnosed within 5 months of injury and of traumatic cause are associated with more favorable outcomes, while VS/UWS and nontraumatic DoC cause are associated with poorer outcomes.

However, individual outcomes vary and prognosis is not "universally poor" (level B), the authors caution.

9. Counsel family members of patients with severe long-term disability to seek assistance in establishing goals of care; medical decision-making; applying for disability benefits; and starting estate, caregiver, and long-term care planning (level A).

10. When patients enter the chronic phase of VS/UWS (3 months after nontraumatic brain injury [TBI] and 12 months after TBI), offer prognostic counseling emphasizing the likelihood of permanent severe disability and the need for long-term assistive care (level B).

Care and Treatment of Prolonged DoC (Recommendations 11 to 15)

11. Identify patient and family preferences early and throughout provision of care (level A).

12. Be vigilant to the medical complications that commonly occur during the first few months after injury (level B).

13. Assess patients for evidence of pain or suffering and treat them when there is "reasonable cause" to suspect that the patient is experiencing pain (level B).

14. Patients with traumatic VS/UWS or MCS 4 to 16 weeks after injury should receive amantadine (100 to 200 mg twice daily) if there are no medical contraindications (level B).

 "Amantadine accelerates the pace of recovery and is effective for patients with traumatic disorders of consciousness; however, it has not yet been formally tested in non-traumatic injury," Giacino said.

15. Clinicians should counsel families about the limitations of existing evidence concerning treatment effectiveness and the potential risks and harms associated with interventions that lack evidentiary support (Level B).

A "Landmark Publication"

Commenting on the guideline for Medscape Medical News, Joseph Fins, MD, chief, Division of Medical Ethics, Weill Cornell Medical College, New York City, who was not involved in guideline authorship, called it a "landmark publication" that "establishes a standard of care for people with DoC."

The guideline "represents maturation of science and the evolution of a field" and is "a real step forward for this population that has historically been marginalized and remains vulnerable," said Fins, who is the coauthor of an accompanying editorial.

"It will no longer be sufficient to tolerate unfortunate diagnostic errors that afflict this population — for example, often patients who are conscious are thought to be unconscious and it is assumed that they feel no pain," he said.

Moreover, the guideline "suggests that brain states are not static, but dynamic, and that people can improve over time."

He pointed to one caveat. "The authors excluded studies with fewer than 20 subjects and, although this approach is rigorous, it excluded preliminary proof-of-principle diagnostic methods as well as small emerging therapies."

Thus, "there's more potentiality in the field than is represented in the guidelines," he said.

He added that "broader healthcare system has to catch up with these guidelines," and is not "currently prepared to meet the needs of these patients, who are often abandoned in the chronic are sector.'"

Giacino added, "Our efforts are beginning now, as we set out to disseminate the initiative and ensure that the information is unpacked for healthcare professionals and the public."

The practice guideline was funded by the American Academy of Neurology, the American Congress of Rehabilitation Medicine, and the National Institute on Disability, Independent Living, and Rehabilitation Research. Giacino reports multiple sources of funding. His disclosures and those of the other guideline authors are listed on the original paper . Fins receives royalties for his book Rights Come to Mind: Brain Injury, Ethics and the Struggle for Consciousness (Cambridge Univ Press, 2015) and A Palliative Ethic of Care: Clinical Wisdom at Life's End (Jones and Bartlett, 2006). His other disclosures and those of his coauthor are listed on the original editorial.

Neurology. Published online August 8, 2018. Guideline, Literature review, Editorial

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