Spotlight on Disorders of consciousness

Clinical disorders of consciousness have attracted extensive scientific and media attention. The persistent vegetative state (also known as unresponsive wakefulness syndrome) and the minimally conscious state are being reconsidered and redefined since their original descriptions in 1972 (persistent vegetative state) and 2002 (minimally conscious state). The results of functional neuroimaging and electrophysiological studies suggest that some degree of consciousness or awareness that has not been or could not be determined by behavioral evaluations alone may be present in some of these patients. This raises multiple therapeutic and ethical questions, such as:

  1. Do the findings of these research evaluations truly represent consciousness, and if they are present on fMRI, PET, or EEG in a patient who cannot otherwise demonstrate conscious behavior, are they adequate and appropriate neural correlates?
  2. Do these patients appreciate pain?
  3. Should the usual duration of aggressive rehabilitation therapies be extended given the multiple reports of continued improvement of patients in persistent vegetative state well past the standard estimates of permanency of 3 months for nontraumatic and 12 months for traumatic etiologies?
  4. Should end-of-life or right-to-life issues be reconsidered given the aforementioned concerns?

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Spotlight on Breath-holding spells

Breath-holding spells, described more than 400 years ago, are paroxysmal clinical events that occur between the ages of 6 months and 4 to 6 years in which vigorous crying is interrupted by end-expiratory apnea, followed by cyanosis or pallor, loss of consciousness, and occasionally by a clonic seizure or myoclonic movements. Though virtually always triggered by a stimulus (pain, fear, or anger), the misconception still exists that the child “does it on purpose.” The spells are terrifying to parents or caregivers but are often dismissed by clinicians in a cavalier manner due to their benign long-term outcome and the misconception that they occur in “spoiled children.”

In this article, Dr. Felicia Gliksman of Hackensack University Medical Center, Hackensack Meridian Health School of Medicine discusses studies regarding presumed autonomic dysregulation, rare occurrences of asystole, and seizures. Dr. Gliksman also expands on recent studies proposing possible treatment options in children with breath-holding spells.

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Spotlight on Locked-in syndrome

In the purest sense, locked-in syndrome occurs when a patient becomes “de-efferented” due to a lesion occurring in the brainstem, classically in the ventral pons. Cortical function remains unimpaired, but leads to quadriplegia with retained consciousness. In this clinical summary, Nicole Reams MD, of the Department of Neurology at the University of Michigan, discusses the presentation, etiology, prognosis, diagnostic evaluation, and management of locked-in syndrome.

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