The vegetative state is a chronic condition that preserves the ability to maintain blood pressure (BP), respiration, and cardiac function, but not cognitive function. Hypothalamic and medullary brain stem functions remain intact to support cardiorespiratory and autonomic functions and are sufficient for survival if medical and nursing care is adequate. The cortex is severely damaged (eliminating cognitive function), but the reticular activating system (RAS) remains functional (making wakefulness possible). Midbrain or pontine reflexes may or may not be present. Patients have no awareness of self and interact with the environment only via reflexes. Seizure activity may be present but not be clinically evident.
Traditionally, a vegetative state that lasts > 1 month is considered to be a persistent vegetative state. However, a diagnosis of persistent vegetative state does not imply permanent disability because in very rare cases (eg, after traumatic brain injury), patients can improve, reaching a minimally conscious state or a higher level of consciousness.
The most common causes of a vegetative state and minimally conscious state are
Diffuse cerebral hypoxia
However, any disorder that results in brain damage can cause a vegetative state. Typically, a vegetative state occurs because the function of the brain stem and diencephalon resumes after coma, but cortical function does not.
In the minimally conscious state, unlike the vegetative state, there is evidence that patients are aware of themselves and/or their environment. Patients also tend to improve (ie, gradually become more conscious), but improvement is limited. This state may be the first indication of brain damage or may follow a vegetative state as people recover some function. Patients can transition between the vegetative state and minimally conscious state, sometimes for years after the original brain damage.
Symptoms and Signs
Patients in a vegetative state show no evidence of awareness of self or environment and cannot interact with other people. Purposeful responses to external stimuli are absent, as are language comprehension and expression.
The following are present in patients in a vegetative state:
Signs of an intact reticular formation (eg, eye opening) and an intact brain stem (eg, reactive pupils, oculocephalic reflex)
Sleep-wake cycles, not necessarily reflecting a specific circadian rhythm nor associated with the environment
More complex brain stem reflexes, including yawning, chewing, swallowing, and, uncommonly, guttural vocalizations
Sometimes arousal and startle reflexes (eg, loud sounds or blinking with bright lights may elicit eye opening)
Sometimes watering and tearing of the eyes
Sometimes the appearance of a smile or frown
Spontaneous roving eye movements—usually slow, of constant velocity, and without saccadic jerks
The spontaneous roving eye movements may be misinterpreted as volitional tracking and can be misinterpreted by family members as evidence of awareness.
Patients cannot react to visual threat and cannot follow commands. The limbs may move, but the only purposeful motor responses that occur are primitive (eg, grasping an object that contacts the hand). Pain usually elicits a motor response (typically decorticate or decerebrate posturing) but no purposeful avoidance. Patients have fecal and urinary incontinence. Cranial nerve and spinal reflexes are typically preserved.
Rarely, brain activity, detected by functional MRI or electroencephalography (EEG), indicates a response to questions and commands even though there is no behavioral response (covert consciousness). The extent of patients' actual awareness is not yet known. In most patients who have such brain activity, the vegetative state resulted from traumatic brain injury, not hypoxic encephalopathy.
Minimally conscious state
Fragments of meaningful interaction with the environment are preserved. Patients in a minimally conscious state may do the following:
Establish eye contact
Purposefully grasp at objects
Respond to commands in a stereotypic manner
Answer with the same word
Clinical criteria after sufficient observation
A vegetative state is suggested by characteristic findings (eg, no purposeful activity or comprehension) plus signs of an intact reticular formation. Diagnosis is based on clinical criteria. However, neuroimaging is indicated to rule out treatable disorders.
The vegetative state must be distinguished from the minimally conscious state. Both states can be permanent or temporary, and the physical examination may not reliably distinguish one from the other. Sufficient observation is needed. If observation is too brief, evidence of awareness may be overlooked. Some patients with severe Parkinson disease are misdiagnosed as being in a vegetative state.
CT or MRI can differentiate an ischemic infarct, an intracerebral hemorrhage, and a mass lesion involving the cortex or the brain stem. Magnetic resonance angiography can be used to visualize the cerebral vasculature after exclusion of a cerebral hemorrhage. Diffusion-weighted MRI is becoming the preferred imaging modality for following ongoing ischemic changes in the brain.
Positron emission tomography (PET) and single-photon emission computed tomography (SPECT) can be used to assess cerebral function (rather than brain anatomy). If the diagnosis of persistent vegetative state is in doubt, PET, SPECT, or functional MRI should be done.
EEG is useful in assessing cortical dysfunction and identifying occult seizure activity.
Prognosis varies somewhat by cause and duration of the vegetative state. Prognosis may be better if the cause is a reversible metabolic condition (eg, toxic encephalopathy) than if the cause is neuronal death due to extensive hypoxia and ischemia or another condition. Also, younger patients may recover more motor function than older patients but not more cognition, behavior, or speech.
Recovery from a vegetative state is unlikely after 1 month if brain damage is nontraumatic and after 12 months if brain damage is traumatic. Even if some recovery occurs after these intervals, most patients are severely disabled. Rarely, improvement occurs late; after 5 years, about 3% of patients recover the ability to communicate and comprehend, but even fewer can live independently; no patients regain normal function.
If a vegetative state persists, most patients die within 6 months of the original brain damage. The cause is usually pulmonary infection, urinary tract infection, or multiple organ failure, or death may be sudden and of unknown cause. For most of the rest, life expectancy is about 2 to 5 years; only about 25% of patients live > 5 years. A few patients live for decades.
Minimally conscious state
Most patients tend to recover consciousness but to a limited extent depending on how long the minimally conscious state has lasted. The longer it has lasted, the less chance of patients recovering higher cortical function. Prognosis may be better if the cause is traumatic brain injury.
Rarely, patients regain clear but limited awareness after years of coma, called awakenings by the news media.
Supportive care is the mainstay of treatment for patients in a vegetative state or minimally conscious state; it should include the following:
Preventing systemic complications due to immobilization (eg, pneumonia, urinary tract infection, thromboembolic disease)
Providing good nutrition
Providing physical therapy to prevent limb contractures
Vegetative state has no specific treatment. Decisions about life-sustaining care should involve social services, the hospital ethics committee, and family members. Maintaining patients, especially those without advanced directives Advance Directives Advance directives are legal documents that extend a person's control over health care decisions in the event that the person becomes incapacitated. They are called advance directives because... read more to guide decisions about terminating treatment, in a prolonged vegetative state raises ethical and other (eg, resource utilization) questions.
Most patients in a minimally conscious state do not respond to specific treatments. However, in some cases, treatment with zolpidem, apomorphine, or amantadine can lead to improvement in neurologic responsiveness for as long as the drug is continued.
A growing number of studies are evaluating the effects of providing music interventions during disorders of consciousness (1 Treatment reference A vegetative state is absence of responsiveness and awareness due to overwhelming dysfunction of the cerebral hemispheres, with sufficient sparing of the diencephalon and brain stem to preserve... read more ). Some studies show that music therapy may lead to positive behavioral effects and return to normal physiologic responses. Results should be interpreted with caution because research in this area has thus far been limited.
Vegetative state is typically characterized by absence of responsiveness and awareness due to overwhelming dysfunction of the cerebral hemispheres, intact brain stem function, and sometimes the simulation of awareness despite its absence.
Minimally conscious state differs from vegetative state in that patients have some interaction with the environment and tend to improve over time.
Diagnosis requires exclusion of other disorders and often prolonged observation, particularly to differentiate vegetative state, minimally conscious state, and Parkinson disease.
Prognosis tends to be poor, particularly for patients in a vegetative state.
Treatment is mainly supportive.