Guideline Update: Disorders of Consciousness

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The American Academy of Neurology (AAN) has released new guidelines for the management of patients in vegetative and minimally conscious states (MCS).¹ The guidelines serve as an update to the 1995 recommendations as well as the 2002 case definition of MCS.

The recommendations focus on individuals with prolonged disorders of consciousness lasting 28 days or longer. Accurate diagnosis and evidence-based continuing medical and rehabilitative care may offer the best chance for recovery from these disorders.

They emphasize specialized care provided by an interdisciplinary team with expertise diagnosing and treating these disorders. In total, they provide 18 recommendations covering overall care and diagnosis of prolonged disorder of consciousness, prognostic counseling recommendations, as well as management recommendations for adults and children.

“People are sometimes misdiagnosed due to underlying impairments that can mask awareness,” guideline lead author Joseph T. Giacino, PhD, of Harvard Medical School and Spaulding Rehabilitation Hospital in Boston, said in a press release.

Misdiagnosis of severe disorders of consciousness consistently hovers around 40%. While outcomes for prolonged disorders of consciousness vary widely, about 20% of people with severe brain injury eventually recover enough to live at home and care for themselves, according to the guidelines.

“An inaccurate diagnosis can lead to inappropriate care decisions and poor health outcomes. Misdiagnosis may result in premature or inappropriate treatment withdrawal, failure to recommend beneficial rehabilitative treatments and worse outcome. That is why an early and accurate diagnosis is so important,” Dr Giacino said.

Relying on a single exam is more likely to increase misdiagnosis, so the guidelines recommend serial exams conducted by trained specialists who can pick up subtle and often inconsistent signs of consciousness. For these exams, clinicians should use standardized, validated neurobehavioral assessments, such as the Coma Recovery Scale–Revised (CRS-R). Serial exams should take place early in recovery, and particularly during the initial three months following brain injury. However, insufficient evidence exists to recommend how far apart these exams should be spaced, and how often they should be done.

Moderate evidence suggests that individuals who suffer from brain trauma may have a better chance at recovery than those with brain injury from other causes. Sparse evidence exists to support treatments for disorders of consciousness, with the exception of the drug amantadine. Moderate evidence suggests amantadine can speed recovery when given within four months after traumatic brain injury.

Additional key points

• Identify and treat confounding conditions, optimize arousal, and perform serial standardized assessments to improve diagnostic accuracy in adults and children with prolonged disorders of consciousness (level B)

• Counsel families that for adults, the following are associated with more favorable outcomes: MCS compared to vegetative state/unresponsive wakefulness syndrome, and traumatic compared to nontraumatic brain injury (Level B)

• When prognosis is poor, discuss long-term care (Level A), acknowledging that prognosis is not universally poor (Level B)

• Structural MRI, SPECT, and the Coma Recovery Scale–Revised can assist prognostication in adults (Level B)

• No tests are shown to improve prognostic accuracy in children

• Always assess and treat pain (Level B) and discuss evidence supporting treatment approaches (Level B)

• Prescribe amantadine (100 mg to 200 mg bid) for adults who are 4 to 16 weeks post-injury with traumatic vegetative state/unresponsive wakefulness syndrome or MCS to hasten functional recovery and reduce disability early in recovery (Level B)

• Family counseling for children should acknowledge that natural history of recovery, prognosis, and treatment are not established (Level B)

• Recent evidence indicates that the term chronic vegetative state/unresponsive wakefulness syndrome should replace permanent vegetative state, with duration specified (Level B)

The guidelines were drawn up by an interdisciplinary group of experts convened by the AAN, American Congress of Rehabilitation Medicine and the National Institute on Disability, Independent Living, and Rehabilitation Research. They were endorsed by the American Academy of Physical Medicine and Rehabilitation, American College of Surgeons Committee on Trauma, and Child Neurology Society

References:

Reference

1. Giacino JT, Katz DI, Schiff ND, et al. Practice guideline update recommendations summary: Disorders of consciousness: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology; the American Congress of Rehabilitation Medicine; and the National Institute on Disability, Independent Living, and Rehabilitation Research. Neurology. 2018;91:450-460.