Brain Herniation Syndromes - 2 Nursing CEs Author: Kristi Hudson RN MSN CCRN Course Objectives Upon completion of this

course, the student will be able to:

Discuss the major components of the brain Describe how the different compartments of the brain are separated Explain the theory behind the Monroe-Kellie Doctrine List the three types of Supratentorial Herniation Describe 3 early symptoms of Uncal herniation and 3 late stage symptoms of Uncal herniation Discuss nursing care and management of the patient with increased intracranial pressure List the 3 goals of therapy for increased intracranial pressure

Definition of Herniation The definition of brain herniation is the protrusion of the brain tissue from one compartment (of higher pressure) to a compartment of lower pressure. With this shift of brain tissue, blood and oxygen supplies are diminished causing herniating tissue to suffer from ischemia, hypoxia and finally cell death. To better understand the different herniation syndromes, lets take a look at what the brain is comprised of and how it is divided. The components of the brain include:

Parenchyma - The essential tissue (Brain in this case) of an organ distinct from its surrounding connective tissue. This is the largest intracranial volume (80%). Blood vessel - The volume of blood in the cerebral arteries is an important determinant of intracranial pressure (10%).
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Cerebral Spinal Fluid - CSF is comprised of fluid in the ventricles and subarachnoid spaces and is produced by the choroid plexus. Approximately 500 cc is produced daily (10%).

The Monroe-Kellie Doctrine simply states that in a nonexpandable, non-contractable, freely communicating space, the pressure of the fluid contents and the brain itself, must be directly porportional to eachother in order to maintain a constant pressure. If one of these pressures increases, another must decrease in order to compensate. In addition to the parenchyma, blood vessels and CSF, the brain is also comprised of several individual compartments that are separated by folds of fibrous and relatively rigid dura mater. Two of the major dura folds include: Falx Cerebri which is a double fold of dura mater that drops into the longitudinal fissure and partially, divides the supratentorial space into the left and right side. Tentoruim Cerebelli this is also a double fold of the dura mater that forms a tent like partition (higher in the middle), between the cerebrum and cerebellum. (The area above the tentorium is the supratentorium space and the area below is known as the infratentorial space). Note: Because it is surrounded by strong bone and it is comprised of several small compartments divided by dura mater, the brain is at greater risk then other body organs for herniation. Types and Location of Herniation Syndromes Supratentorial Herniation (descending) There are three major types of herniation that are caused by expanding supratentorial lesions, these include: Cingulate Herniation This occurs when the cingulated gyrus is forced under the falx cerebri, displacing it to the opposite side. Cingulate herniation alone is not
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considered to be life threatening, but because it displaces the falx cerebri and can cause compression and/or loss to local blood supply and surrounding cerebral tissue, it can lead to increases in ICP, which can contribute other and more devastating forms of herniation. Signs and symptoms from Cingulate Herniation are not well defined, but thought to include:

Increased ICP Cerebral Edema Altered LOC Change in Neuro Exam

Note: Though Cingulate Herniation is without defined symptomatology; it is often thought to be a precursor to other more serious herniation syndromes. Central Transtentorial Herniation (Central Syndrome) This occurs when a downward shift or displacement, pushes the cerebral hemispheres, basal ganglia, diencephalons, mid-brain and finally the medulla through the tentorial incisura (notch). Progression of signs and symptoms vary depending on whether herniation is in the early (reversible) or late (irreversible) stage. These include: Early herniation to the diencephalons region (reversible):

Change in LOC (decreased alertness, agitation, drowsiness) Pupils are small (1-3mm) but reactive Conjugate or slightly disconjugate pupils at rest Early hemiparesis or hemiplegia develop bilateral signs Purposeful to painful stimuli Babinskis sign absent Respiratory will show deep sigh, yawns and occasional pauses

Herniation to the Midbrain/Upper Pons (Middle phase of Central Herniation):

Deep Coma Pupils midpoint (3-5mm) and non-reactive Disconjugate pupils with limited horizontal movement Decorticate posturing changes to decerebrate posturing Babinskis sign present Gradual respiratory change to sustained hyperventilation Diabetes Insipidus (not seen early) Hyperthermia (not seen early)

Herniation to the Medulla (Late phase of Central Herniation and irreversible):

Deep Coma Pupils dilated and non-reactive Absent pupil movement Flaccid extremities at rest, occasional flexor response to deep pain Slow irregular respiratory rate, ataxia and periods of apnea Cushings Triad (Hypertension, Bradycardia, Irregular Respirations)

Note: The underlying pathophysiology is that in the early phase of Central Transtentorial Herniation, ischemia and compression, which are, both thought to be reversible conditions, are the basis for signs and symptoms of diencephalic involvement. In the later phase after the midbrain becomes involved, it is thought that infarction of brain tissue has begun and it is at this point that the damage is irreversible. Uncal Transtentorial Herniation (Uncal Syndrome) This occurs when the uncus or hippocampal gyrus (or both), shift from the middle fossa through the tentorial notch
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and into the posterior fossa. When this occurs, there is compression of the ipsilateral third cranial nerve, then contralateral third cranial nerve and finally the mesencephalon. When Uncal Herniation occurs, the diencephalons and mid-brain are shifted to the opposite side of the brain. As with Central Transtentorial Herniation, signs and symptoms of Uncal herniation are considered to be reversible in the early stages and irreversible in the late stages. These signs and symptoms include: Early herniation to the diencephalons region (potentially reversible):

LOC may not be impaired initially Unilateral sluggish, dilating pupil (Ipsilateral to primary lesion) Full extraocular movement Pupils react to Cold Carolics No motor function abnormality Babinskis sign absent Normal respiratory pattern and rate

Herniation to the Midbrain/Upper Pons (Middle phase of Uncal Herniation):

Deep Coma Pupils contralaterally fixed and either completed dilated or enlarged to 5-6mm No extraocular movement Bilateral decerebration Bilateral positive Babinskis sign Hyperventilation (usually with rate 20-4- per minute)

Herniation to the Medulla (Late phase of Uncal Herniation and irreversible):

Deep Coma Bilateral and fully dilated and fixed pupils

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No extraocular movement Flaccid extremities with occasional flexor response to deep painful stimuli Slow and irregular respirations with occasional gasps and periods of apnea Hyperthermia Cushings Triad (Hypertension, Bradycardia, Irregular Respirations)

Note: The result of continued and unresolved downward displacement from any of the supratentorial herniation syndromes is ultimate brainstem herniation. When the medulla, which controls such vital functions as respiration and cardiac function, herniates into the foramen magnum, death is immediate. Infratentorial Herniation (ascending) Lesions of the infratentorial compartment that contribute to herniation are much less frequent then supratentorial herniation syndromes. Included in the infratentorial compartment are the brainstem and the cerebellum. There are three possible effects of an expanding lesion in the infratentorial compartment and they include:

Direct compression of the brainstem, cerebellum or the vascular supply to each area Upward transtentorial herniation of the brainstem and cerebellum Downward herniation of one or both cerebellum tonsils through the foramen magnum, to the cervical spine. When this occurs, the medulla is compressed and death is immediate.

The signs and symptoms of an Infratentorial Herniation vary widely depending on the brainstem involvement. In addition to medullary compression, an infratentorial lesion can also encroach on a portion of the ventricular system (3rd and 4th ventricle), which causes acute hydrocephalus.

Nursing Care and Management of Increased Intracranial Pressure:

Monitor LOC using Glasgow Coma Scale (or other objective scale) Assess motor response (bilaterally) Check for positive Babinskis sign Assess sensory responses (place emphasis on side opposite of injury) Include the following in assessment of pupils: Comparing pupil size, shape and equality bilaterally Check pupils with the direct light reflex (check each eye individually) Check that pupils are equal, round reactive to light accommodation (PERRLA) Assess 6 cardinal fields of gaze (Cranial Nerves 3, 4 and 6) Assess for Dolls eye phenomenon in unconscious patients (indicates brain stem damage) Monitor vital signs (Notify MD for deviation from set parameters) Provide nursing measures r/t respiratory care including: Suctioning ABGs Providing O2 Monitoring O2 saturation Monitoring ventilator settings Position pt to maintain venous outflow from brain Elevate HOB to 30 degrees (except for dural tear) No pillow under head (can interfere with venous flow) Turn by logrolling q 2 hrs Administer prescribed medications Control noise and stimuli Provide rest/activity balance (stagger tasks) Maintain desired temperature range (use antipyretics or hypothermia blanket) Provide nursing care to prevent damage to eyes,
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skin, oral mucous Provide education and emotional support to family

Nursing Diagnosis

Altered tissue perfusion r/t impaired cerebral circulation Altered sensory perception Impaired gas exchange Self-care deficits Altered body image Potential for skin breakdown Potential for injury Ineffective coping (family) Knowledge deficit Anxiety (fear) Altered Nutrition

Major Goals

Maintain normal ICP Vital signs and ABGs normal for patient Improvement in LOC

References Marini, J., J. & Wheeler, A., P. (2006). Critical care medicine: the essentials.(3rd ed.). Lippencott, Williams and Wilkes. Philadelphia Arbour, R., (2004). Intracranial hypertension: Monitoring and nursing assessment. Critical Care Nurse. Oct. 2004. Hickey, J., V., (2002). The Clinical Practice of Neurological and Neurosurgical Nursing. (5th ed.) Lippencott. Philadelphia S Peterman, G., M.D., & Smirniotopoulos, J., G., M.D. (2005). Brain herniation. Retrieved on December 5, 2006 at:
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http://rad.usuhs.mil/rad/herniation/herniation.html Neurological Nursing NU 454. (2000). Retrieved on December 20, 2004 at: http://www.muw.edu/nursing/acuteneuro.html Brain Herniation Causes of Different Types of Hernias

Table of Contents

What is a brain herniation? Different Types of Brain Herniation Causes of Brain Herniation The cranial cavity houses the brain and related structures but it is a rigid compartment that will not accommodate expansion of the tissues and fluid within. The dural folds are also rigid dividers that compartmentalize the different parts of the brain. The softer tissue of the brain therefore presses against the bone or dural folds or protrudes through any openings should there be swelling or bleeding within the parenchyma or cranial cavity. Read more on swelling of the brain and bleeding in the brain. What is a brain herniation? A brain herniation syndrome is when the brain tissue is pushed from its normal position and protrudes into adjacent compartments or may even push out of the skull if there is an opening present. In order to understand how a herniation develops and the different types of brain herniation syndromes, it is important to have a basic knowledge of intracranial anatomy. The brain is housed in the hard and bony cranial cavity of the skull. The opening at the base of the skull through which the medulla oblongata, an extension of the spinal cord, exits the skull is known as the foramen magnum. It is one opening for brain tissue to protrude through. The brain is covered by the meninges, which comprise three layers pia, arachnoid and dura mater. The dura mater thickens and folds on itself to project into two sites :

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1. between the left and right hemispheres of the brain (cerebrum) where it is known as the falx cerebri. 2. between the occipital lobe of the brain (cerebrum) and cerebelllum where it is known as thetentorium cerebelli.

Different Types of Brain Herniation There are four different types of brain herniation : 1. 2. 3. 4. 5. Uncinate/Uncal rranstentorial herniation Central transtentorial herniation Subfalcine (cingulate) herniation Extracranial (transcalvarial) herniation Upward cerebellar herniation
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6. Tonsillar (downward cerebellar) herniation

Picture from Wikimedia Commons The four main types of brain herniation syndromes include an uncinate and central transtentorial herniations, as well as a subfalcine or tonsillar herniations. An uncinate or uncal transtentorial herniation (1) occurs when the inner part (medial aspect) of the temporal lobe is pushed against the free margin of the tentorium. In severe cases, it can compress the brainstem. Another type, known as a central transtentoral herniation, or just acentral herniation (2), occurs when there is downward pressure centrally. It may cause a bilateral uncal herniation, meaning an uncal herniation (1) on both sides. A subfalcine herniation (3) occurs when there is uneven, usually one-sided expansion of the cerebral hemisphere which pushes a portion of the brain tissue known as the cingulate gyrus under the falx cerebri. Hence this type of herniation is also known as a cingulate herniation. The fourth main type of brain herniation is known as the tonsillar herniation and involves the cerebellum. Swelling or bleeding within the cerebellum pushes the cerebellar tonsils downwards into the foramen magnum. It is also known as the downward cerebellar herniation and can be life-threatening because it compresses the brainstem. A less commonly seen cerebellar herniation is
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theupward cerebellar herniation (5) where pressure from the back of the cranial cavity pushes the cerebellar tissue upwards. An extracranial herniation (4) is when the brain tissue pushes through an opening in the cranial cavity formed by trauma or at a surgical site. It is also known as a transcalvarial herniation. Causes of Brain Herniation A brain herniation is a consequence of raised intracranial pressure where the force pushes the brain tissue in a specific direction and angle. This may be diffuse meaning that it is occurring throughout the brain tissue or cranial cavity or focal (localized) where it is only occurring at a specific portion of the brain or cranial cavity. 1. Diffuse Causes o Generalized swelling of the brain o Hydrocephalus 2. Focal Causes o Abscess o Tumor o Intracranial hematoma Ads by Google Homeopathy-Brain Proven Success w/ All Types www.DrPBanerji.com/BrainTumor Top Brand ofBrain Shoes Tumors. Consult On Us Tumor Now! Sale

Flat 10% Off Today. Buy Now!Flip-Flops, Wedges, Sandals & More. Shopping.indiatimes.com/ShipFree Free Mind Power e-Course Self-Hypnosis will help you www.hyptalk.com Win Prizes Daily Overview of Coma and Impaired Consciousness toRealize Your Unlimited Potential

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Coma is unresponsiveness from which the patient cannot be aroused. Impaired consciousness refers to similar, less severe disturbances of consciousness; these disturbances are not considered coma. The mechanism for coma or impaired consciousness involves dysfunction of both cerebral hemispheres or of the reticular activating system (also known as the ascending arousal system). Causes may be structural or nonstructural (eg, toxic or metabolic disturbances). Damage may be focal or diffuse. Diagnosis is clinical; identification of cause usually requires laboratory tests and neuroimaging. Treatment is immediate stabilization and specific management of the cause. For long-term coma, adjunctive treatment includes passive range-of-motion exercises, enteral feedings, and measures to prevent pressure ulcers. Decreased or impaired consciousness or alertness refers to decreased responsiveness to external stimuli. Severe impairment includes

Coma: The patient usually cannot be aroused, and the eyes do not open in response to any stimulation. Stupor: The patient can be awakened only by vigorous physical stimulation. Less severely impaired levels of consciousness are often labeled as lethargy or, if more severe, obtundation. However, differentiation between less severely impaired levels is often imprecise; the label is less important than a precise clinical description (eg, the best level of response is partial limb withdrawal to nail bed pressure). Delirium differs because cognitive disturbances (in attention, cognition, and level of consciousness) fluctuate more; also, delirium is usually reversible (see Delirium and Dementia). Pathophysiology Maintaining alertness requires intact function of the cerebral hemispheres and preservation of arousal mechanisms in the reticular activating system (RASalso known as the ascending arousal system)an extensive network of nuclei and interconnecting fibers in the upper pons, midbrain, and posterior diencephalon. Therefore, the mechanism of impaired consciousness must involve both cerebral

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hemispheres or dysfunction of the RAS. To impair consciousness, cerebral dysfunction must be bilateral; unilateral cerebral hemisphere disorders are not sufficient, although they may cause severe neurologic deficits. However, rarely, a unilateral massive hemispheric focal lesion (eg, left middle cerebral artery stroke) impairs consciousness if the contralateral hemisphere is already compromised or if it results in compression of the contralateral hemisphere (eg, by causing edema). Usually, RAS dysfunction results from a condition that has diffuse effects, such as toxic or metabolic disturbances (eg, hypoglycemia, hypoxia, uremia, drug overdose). RAS dysfunction can also be caused by focal ischemia (eg, certain upper brain stem infarcts), hemorrhage, or direct, mechanical disruption. Any condition that increases intracranial pressure (ICP) may decrease cerebral perfusion pressure, resulting in secondary brain ischemia. Secondary brain ischemia may affect the RAS or both cerebral hemispheres, impairing consciousness. When brain damage is extensive, brain herniation (see Fig. 1: Coma and Impaired Consciousness: Brain herniation. and Table 1: Coma and Impaired Consciousness: Effects of Brain Herniation ) contributes to neurologic deterioration because it directly compresses brain tissue, increases ICP, may lead to hydrocephalus, and results in neuronal and vascular cell dysfunction. In addition to the direct effects of increased ICP on neuronal and vascular cells, cellular pathways of apoptosis and autophagy, also detrimental to these cells, can become activated. Fig. 1 Brain herniation.

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Because the skull is rigid after infancy, intracranial masses or swelling may increase intracranial pressure, sometimes causing protrusion (herniation) of brain tissue through one of the rigid intracranial barriers (tentorial notch, falx cerebri, foramen magnum). When intracranial pressure is increased sufficiently, regardless of the cause, Cushing reflex and other autonomic abnormalities can occur. Cushing reflex includes systolic hypertension with increased pulse pressure, irregular respirations, and bradycardia. Brain herniation is life threatening. Transtentorial herniation: The medial temporal lobe is squeezed by a unilateral mass under the tentlike tentorium that supports the temporal lobe. The herniating lobe compresses the following structures:

Ipsilateral 3rd cranial nerve (often first) and posterior cerebral artery As herniation progresses, the ipsilateral cerebral peduncle In about 5% of patients, the contralateral 3rd cranial nerve and cerebral peduncle
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Eventually, the upper brain stem and the area in or around the thalamus Subfalcine herniation: The cingulate gyrus is pushed under the falx cerebri by an expanding mass high in a cerebral hemisphere. In this process, one or both anterior cerebral arteries become trapped, causing infarction of the paramedian cortex. As the infarcted area expands, patients are at risk of transtentorial herniation, central herniation, or both. Central herniation: Both temporal lobes herniate because of bilateral mass effects or diffuse brain edema. Ultimately, brain death occurs. Upward transtentorial herniation: This type can occur when an infratentorial mass (eg, tumor, cerebellar hemorrhage) compresses the brain stem, kinking it and causing patchy brain stem ischemia. The posterior 3rd ventricle becomes compressed. Upward herniation also distorts the mesencephalon vasculature, compresses the veins of Galen and Rosenthal, and causes superior cerebellar infarction due to occlusion of the superior cerebellar arteries. Tonsillar herniation: Usually, the cause is an expanding infratentorial mass (eg, cerebellar hemorrhage). The cerebellar tonsils, forced through the foramen magnum, compress the brain stem and obstruct CSF flow. Table 1 Effects of Brain Herniation Type of Mechanism* Findings Herniation Transtentorial Compression of ipsilateral 3rd cranial nerve Unilateral dilated, fixed pupil Oculomotor paresis

Compression of the Contralateral posterior cerebral artery homonymous hemianopia Absence of blinking in response to visual threat
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in obtunded patients Compression of the Contralateral dilated pupil contralateral 3rd cranial and oculomotor paresis nerve and cerebral Ipsilateral hemiparesis peduncle Compression of the ipsilateral cerebral peduncle Contralateral hemiparesis

Eventually, compression Impaired consciousness of the upper brain stem Abnormal breathing and the area in and patterns around the thalamus Fixed, unequal pupils Further compromise of the brain stem Loss of oculocephalic reflex Loss of oculovestibular reflex Loss of corneal reflexes Decerebrate posturing Leg paralysis

Subfalcine (cingulate)

Trapping of one or both anterior cerebral arteries, causing infarction of the paramedian cortex Expansion of infarcted area

Edema Increased intracranial pressure Increased risk of transtentorial herniation, central herniation, or both Pupils fixed in midposition
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Central

Bilateral, more or less symmetric damage to

the midbrain

Decerebrate posturing Many of the same symptoms as transtentorial herniation Loss of all brain stem reflexes Disappearance of decerebrate posturing Cessation of respirations Brain death Hydrocephalus, which increases intracranial pressure Early: Nausea, vomiting, occipital headache, ataxia Later: Somnolence, breathing abnormalities, patchy and progressive loss of brain stem reflexes

Further compromise of the brain stem

Upward Compression of the transtentorial posterior 3rd ventricle Distortion of the mesencephalon vasculature Compression of the veins of Galen and Rosenthal Superior cerebellar infarction due to occlusion of the superior cerebellar arteries

Posterior fossa mass (eg, Ataxia cerebellar hemorrhage) Progression Increasing somnolence Respiratory irregularities Patchy but progressive loss of brain stem reflexes

Tonsillar

Compression of the brain Acute hydrocephalus stem (with impaired Obstruction of CSF flow consciousness, headache, vomiting, and
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meningismus) Dysconjugate eye movements Later, abrupt respiratory and cardiac arrest *Not all mechanisms occur in every patient. Impaired consciousness may progress to coma and ultimately to brain death (see Coma and Impaired Consciousness: Brain Death). Etiology Coma or impaired consciousness may result from structural disorders, which typically cause focal damage, or nonstructural disorders, which most often cause diffuse damage (see Table 2: Coma and Impaired Consciousness: Common Causes of Coma or Impaired Consciousness ). Table 2 Common Causes of Coma or Impaired Consciousness Cause Examples Focal Structural disorders Brain abscess Brain tumor Head trauma (eg, concussion, cerebral lacerations or contusions, epidural or subdural hematoma) Hydrocephalus (acute) Intraparenchymal hemorrhage
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Subarachnoid hemorrhage Upper brain stem infarct or hemorrhage Nonstructural Seizures (eg, disorders nonconvulsive status epilepticus) or a postictal state caused by an epileptogenic focus Diffuse Metabolic disorders Diabetic ketoacidosis Hepatic encephalopathy Hypercalcemia Hypercapnia Hyperglycemia Hypernatremia Hypoglycemia Hyponatremia Hypoxia Myxedema Uremia Wernicke encephalopathy Encephalitis Meningitis Sepsis Diffuse axonal
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Infections

Other

disorders

injury Hypertensive encephalopathy Hyperthermia or hypothermia Sedatives Other CNS depressants Carbon monoxide

Drugs

Toxins

Psychiatric disorders (eg, psychogenic unresponsiveness) can mimic impaired consciousness but are usually distinguished from true impaired consciousness by neurologic examination. Symptoms and Signs Consciousness is decreased to varying degrees. Repeated stimuli arouse patients only briefly or not at all. Depending on the cause, other symptoms develop (see Table 3: Coma and Impaired Consciousness: Findings by Location* ):

Eye abnormalities: Pupils may be dilated, pinpoint, or unequal. One or both pupils may be fixed in midposition. Eye movement may be dysconjugate or absent (oculomotor paresis). Homonymous hemianopia may be present. Other abnormalities include absence of blinking in response to visual threat (almost touching the eye), as well as loss of the oculocephalic reflex (the eyes do not move in response to head rotation), the oculovestibular reflex (the eyes do not move in response to caloric stimulation), and corneal reflexes. Autonomic dysfunction: Patients may have abnormal breathing patterns (Cheyne-Stokes or Biot respirations), sometimes with hypertension and bradycardia (Cushing reflex). Abrupt respiratory and cardiac arrest may occur. Motor dysfunction: Abnormalities include flaccidity, hemiparesis, asterixis, multifocal myoclonus, decorticate posturing (elbow flexion and shoulder adduction with leg extension), and decerebrate posturing (limb extension and internal shoulder rotation).
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Other symptoms: If the brain stem is compromised, nausea, vomiting, meningismus, occipital headache, ataxia, and increasing somnolence can occur. Table 3 Findings by Location* Location Abnormal Findings

Bilateral hemispheric Symmetric tone and response (flexor or extensor) to pain damage or dysfunction* Myoclonus (possible) Periodic cycling of breathing Supratentorial mass compressing the brain stem Ipsilateral 3rd cranial nerve palsy with unilateral dilated, fixed pupil and oculomotor paresis Sometimes contralateral homonymous hemianopia and absent blinking response to visual threat Contralateral hemiparesis Early abnormal pupillary and oculomotor signs Abnormal oculocephalic reflex Abnormal oculovestibular reflex Asymmetrical motor responses Decorticate rigidity (usually due to an upper brain stem lesion) or decerebrate rigidity (usually due to a bilateral midbrain or pontine lesion) Hyperventilation (due to a midbrain or upper pontine lesion) Spontaneous, conjugate roving eye movements in mild coma Fixed eye position in deeper coma Abnormal oculovestibular reflex Multifocal myoclonus
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Brain stem lesion

Toxic-metabolic dysfunction*

Asterixis (may be considered a type of negative myoclonus) Decorticate and decerebrate rigidity or flaccidity *Not all of the findings occur in all cases. Brain stem reflexes and pupillary light responses may be intact in patients with bilateral hemispheric damage or dysfunction or toxic-metabolic dysfunction; however, hypothermia, sedative overdose, or use of an anesthetic can cause partial loss of brain stem reflexes. Diagnosis History General physical examination Neurologic examination, including eye examination Laboratory tests (eg, pulse oximetry, bedside glucose measurement, blood and urine tests) Immediate neuroimaging Sometimes measurement of ICP If diagnosis is unclear, lumbar puncture or EEG Impaired consciousness is diagnosed if repeated stimuli arouse patients only briefly or not at all. If stimulation triggers primitive reflex movements (eg, decerebrate or decorticate posturing), impaired consciousness may be deepening into coma. Diagnosis and initial stabilization (airway, breathing, and circulation) should occur simultaneously. Glucose levels must be measured at bedside to identify low levels, which should be corrected immediately. If trauma is involved, the neck is immobilized until clinical history, physical examination, or imaging tests exclude an unstable injury and damage to the cervical spine. History: Medical identification bracelets or the contents of a wallet or purse may provide clues (eg, hospital identification card, drugs). Relatives, paramedics, police officers, and any witnesses should be questioned about the circumstances and environment in which the patient was found; containers that may have held food, alcohol, drugs, or poisons should be examined and saved for identification (eg, drug
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identification aided by a poison center) and possible chemical analysis. Relatives should be asked about the onset and time course of the problem (eg, whether seizure, headache, vomiting, head trauma, or drug ingestion was observed, how quickly symptoms appeared, whether the course has been progressive or waxing and waning), baseline mental status, recent infections and possible exposure to infections, recent travel, ingestions of unusual meals, psychiatric problems and symptoms, drug history, alcohol and other substance use, previous illnesses, the last time the patient was normal, and any hunches they may have about what might be the cause (eg, possible occult overdose, possible occult head trauma due to recent intoxication). Medical records should be reviewed if available. General physical examination: Physical examination should be focused and efficient and should include thorough examination of the head and face, skin, and extremities. Signs of head trauma include periorbital ecchymosis (raccoon eyes), ecchymosis behind the ear (Battle sign), hemotympanum, instability of the maxilla, and CSF rhinorrhea and otorrhea. Scalp contusions and small bullet holes can be missed unless the head is carefully inspected. If unstable injury and cervical spine damage have been excluded, passive neck flexion is done; stiffness suggests subarachnoid hemorrhage or meningitis. Fever, petechial or purpuric rash, hypotension, or severe extremity infections (eg, gangrene of one or more toes) may suggest sepsis or CNS infection. Needle marks may suggest drug overdose (eg, of opioids or insulin

). A bitten tongue suggests seizure. Breath odor may suggest alcohol, other drug intoxication, or diabetic ketoacidosis. Neurologic examination: The neurologic examination determines whether the brain stem is intact and where the lesion is located within the CNS (see Approach to the Neurologic Patient: Neurologic
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Examination). The examination focuses on the following:

Level of consciousness Eyes Motor function Deep tendon reflexes Level of consciousness is evaluated by attempting to wake patients first with verbal commands, then with nonnoxious stimuli, and finally with noxious stimuli (eg, pressure to the supraorbital ridge, nail bed, or sternum). The Glasgow Coma Scale (see Table 4: Coma and Impaired Consciousness: Glasgow Coma Scale* ) was developed to assess patients with head trauma. For head trauma, the score assigned by the scale is valuable prognostically. For coma or impaired consciousness of any cause, the scale is used because it is a relatively reliable, objective measure of the severity of unresponsiveness and can be used serially for monitoring. The scale assigns points based on responses to stimuli. Eye opening, facial grimacing, and purposeful withdrawal of limbs from a noxious stimulus indicate that consciousness is not greatly impaired. Asymmetric motor responses to pain or deep tendon reflexes may indicate a focal hemispheric lesion. Table 4 Glasgow Coma Scale* Area Response Assessed Eye Open opening spontaneously; open with blinking at baseline

Points 4

Open to verbal 3 command, speech, or shout Open in response 2 to pain applied to the limbs or
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sternum None Verbal Oriented 1 5

Confused 4 conversation but able to answer questions Inappropriate responses; words discernible 3

Incomprehensible 2 speech None Motor 1

Obeys commands 6 for movement Responds to pain 5 with purposeful movement Withdraws from 4 pain stimuli Responds to pain 3 with abnormal flexion (decorticate posturing) Responds to pain 2 with abnormal extension (decerebrate posturing)
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None *Combined scores < 8 are typically regarded as coma.

Adapted from Teasdale G, Jennett B: Assessment of coma and impaired consciousness. A practical scale. Lancet 2:8184; 1974. Clinical Calculator

As impaired consciousness deepens into coma, noxious stimuli may trigger stereotypic reflex posturing. Decorticate posturing indicates hemispheric damage with preservation of motor centers in the upper portion of the brain stem (eg, rubrospinal tract). Decerebrate posturing indicates that the upper brain stem motor centers, which facilitate flexion, have been damaged and that only the lower brain stem centers (eg, vestibulospinal tract, reticulospinal tract), which facilitate extension, are responding to sensory stimuli. Flaccidity without movement indicates that the lower brain stem is not affecting movement, regardless of whether the spinal cord is damaged. It is the worst possible motor response. Asterixis and multifocal myoclonus suggest metabolic disorders such as uremia, hepatic encephalopathy, hypoxic encephalopathy, and drug toxicity. Psychogenic unresponsiveness can be differentiated because although voluntary motor response is typically absent, muscle tone and deep tendon reflexes remain normal, and all brain stem reflexes are preserved. Vital signs are usually not affected. Eye examination: The following are evaluated:

Pupillary responses
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Extraocular movements Fundi Other neuro-ophthalmic reflexes Pupillary responses and extraocular movements provide information about brain stem function (see Table 5: Coma and Impaired Consciousness: Interpretation of Pupillary Response and Eye Movements ). One or both pupils usually become fixed early in coma due to structural lesions, but pupillary responses are often preserved until very late when coma is due to diffuse metabolic disorders (called toxic-metabolic encephalopathy), although responses may be sluggish. If one pupil is dilated, other causes of anisocoria should be considered (see Symptoms of Ophthalmologic Disorders: Etiology). The fundi should be examined. Papilledema may indicate increased ICP but may take many hours to appear. Increased ICP can cause earlier changes in the fundi, such as disk hypermia, dilated capillaries, blurring of the medial disk margins, and sometimes hemorrhages. Subhyaloid hemorrhage may indicate subarachnoid hemorrhage. Table 5 Interpretation of Pupillary Response and Eye Movements Area Finding Interpretation Assessed Pupils Sluggish light Diffuse cellular cerebral reactivity retained dysfunction (toxic-metabolic until all other brain encephalopathy) stem reflexes are lost Unilateral pupillary 3rd cranial nerve compression dilation, pupil (eg, in transtentorial herniation), unreactive to light usually due to an ipsilateral lesion (see Symptoms of Ophthalmologic Disorders: Anisocoria) Pupils fixed in midposition Midbrain dysfunction due to structural damage (eg, infarction,
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hemorrhage) Central herniation Prolonged metabolic depression by drugs or toxins Constricted pupils (1 mm wide) Massive pontine hemorrhage Toxicity due to opioids or certain insecticides (eg, organophosphates, carbamates) Primary brain stem lesion

Eye Early abnormal movement pupillary and s oculomotor signs

Spontaneous, Early toxic-metabolic encephalopathy conjugate roving eye movements but intact brain stem reflexes Gaze preference to one side Brain stem lesion on the opposite side Cerebral hemisphere lesion on the same side Further testing required (eg, oculocephalic and oculovestibular reflexes) Possibly toxicity due tophenobarbital

Absent eye movements

or phenytoin

, Wernicke encephalopathy, botulism, or brain death In an unresponsive patient, the oculocephalic reflex is tested by the doll's-eye maneuver: The eyes are observed while the head is passively
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rotated from side to side or flexed and extended. This maneuver should not be attempted if cervical spine instability is suspected.

If the reflex is present, the maneuver causes the eyes to move in the opposite direction of head rotation, flexion, or extension, indicating that the oculovestibular pathways in the brain stem are intact. Thus, in a supine patient, the eyes continue to look straight up when the head is turned side to side. If the reflex is absent, the eyes do not move and thus point in whatever direction the head is turned, indicating the oculovestibular pathways are disrupted. The reflex is also absent in most patients with psychogenic unresponsiveness because visual fixation is conscious. If the patient is unconscious and the oculocephalic reflex is absent or the neck is immobilized, oculovestibular (cold caloric) testing is done. After integrity of the tympanic membrane is confirmed, the patient's head is elevated 30, and with a syringe connected to a flexible catheter, the examiner irrigates the external auditory canal with 50 mL of ice water over a 30-sec period.

If both eyes deviate toward the irrigated ear, the brain stem is functioning normally, suggesting mildly impaired consciousness. If nystagmus away from the irrigated ear also occurs, the patient is conscious and psychogenic unresponsiveness is likely. In conscious patients, 1 mL of ice water is often enough to induce ocular deviation and nystagmus. Thus, if psychogenic unresponsiveness is suspected, a small amount of water should be used because cold caloric testing can induce severe vertigo, nausea, and vomiting in conscious patients. If the eyes do not move or movement is dysconjugate after irrigation, the integrity of the brain stem is uncertain and the coma is deeper. Prognosis may be less favorable. Sidebar 1 Pearls & Pitfalls

If muscle tone and deep tendon reflexes are normal and doll's-eye reflex is
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absent, suspect psychogenic unresponsiveness and check the oculovestibular reflex by instilling only 1 mL of ice water in the ear. Certain patterns of eye abnormalities and other findings may suggest brain herniation (see Fig. 1: Coma and Impaired Consciousness: Brain herniation. and see Table 1: Coma and Impaired Consciousness: Effects of Brain Herniation ). Respiratory patterns: The spontaneous respiratory rate and pattern should be documented unless emergency airway intervention is required. It may suggest a cause. Periodic cycling of breathing (Cheyne-Stokes or Biot respiration see Approach to the Pulmonary Patient: Inspection) may indicate dysfunction of both hemispheres or of the diencephalon. Hyperventilation (central neurogenic hyperventilation) with respiratory rates of > 40 breaths/min may indicate midbrain or upper pontine dysfunction. An inspiratory gasp with respiratory pauses of about 3 sec after full inspiration (apneustic breathing) typically indicates pontine or medullary lesions; this type of breathing often progresses to respiratory arrest. Testing: Initially, pulse oximetry, fingerstick plasma glucose measurements, and cardiac monitoring are done. Blood tests should include a comprehensive metabolic panel (including at least serum electrolytes, BUN, creatinine, and Ca levels), CBC with differential and platelets, liver function tests, and ammonia level. ABGs are measured, and if carbon monoxide toxicity is suspected, carboxyhemoglobin level is measured. Blood and urine should be obtained for culture and routine toxicology screening; serum ethanol level is also measured. Additional toxicology tests (eg, additional toxicology screening, serum drug levels) are done based on clinical suspicion. ECG (12-lead) should be done.

If the cause is not immediately apparent, noncontrast head CT should be done as soon as possible to check for masses, hemorrhage, edema, and hydrocephalus. Initially, noncontrast CT rather than contrast CT is
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preferred to rule out brain hemorrhage. MRI can be done instead if immediately available, but it is not as quick as newer-generation CT scanners. Contrast CT can then be done if noncontrast CT is not diagnostic. MRI or contrast CT may detect isodense subdural hematomas, multiple metastases, sagittal sinus thrombosis, herpes encephalitis, or other causes missed by noncontrast CT. A chest x-ray should also be taken. If coma is unexplained after neuroimaging and other tests and if there is no obstruction in the CSF flow or ventricular system that would significantly increase ICP, lumbar puncture is done to check opening pressure and to exclude infection, subarachnoid hemorrhage, and other abnormalities. Lumbar puncture is not done until after imaging studies are done to exclude an intracranial mass and obstructive hydrocephalus because if either is present, suddenly lowering CSF pressure by lumbar puncture could trigger brain herniation. CSF analysis includes cell and differential counts, protein, glucose, Gram staining, cultures, and sometimes, based on clinical suspicion, specific tests (eg, cryptococcal antigen, Venereal Disease Research Laboratory [VDRL] tests, PCR for herpes simplex, visual or spectrophotometric determination of xanthochromia). If increased ICP is suspected, pressure is measured. Hyperventilation, managed by an ICU specialist, should be considered. Hyperventilation causes hypocapnia, which in turn decreases cerebral blood flow globally through vasoconstriction. Reduction in PCO2 from 40 mm Hg to 30 mm Hg can reduce ICP by about 30%. PCO2 should be maintained at 25 mm Hg to 30 mm Hg, but aggressive hyperventilation to < 25 mm Hg should be avoided because this approach may reduce cerebral blood flow excessively and result in cerebral ischemia. If pressure is increased, it is monitored continuously (see Approach to the Critically Ill Patient: Intracranial Pressure Monitoring). If diagnosis remains uncertain, EEG may be done. In most comatose patients, EEG shows slowing and reductions in wave amplitude that are nonspecific but often occur in toxic-metabolic encephalopathy. However, EEG monitoring (eg, in the ICU) is increasingly identifying
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nonconvulsive status epilepticus. In such cases, the EEG may show spikes, sharp waves, or spike and slow complexes. Prognosis Prognosis depends on the cause, duration, and depth of the impairment of consciousness. For example, absent brain stem reflexes indicates a poor prognosis after cardiac arrest, but not always after a sedative overdose. In general, if unresponsiveness lasts < 6 h, prognosis is more favorable. After coma, the early return of speech (even if incomprehensible), spontaneous eye movements, or ability to follow commands is a favorable prognostic sign. If the cause is a reversible condition (eg, sedative overdose, some metabolic disorders such as uremia), patients may lose all brain stem reflexes and all motor response and yet recover fully. After trauma, a Glasgow Coma Scale score of 3 to 5 may indicate fatal brain damage, especially if pupils are fixed or oculovestibular reflexes are absent. After cardiac arrest, clinicians must exclude major confounders of coma, including sedatives, neuromuscular blockade, hypothermia, metabolic derangements, and severe liver or kidney failure. If brain stem reflexes are absent at day 1 or lost later, testing for brain death is indicated. Prognosis is poor if patients have any of the following:

Myoclonic status epilepticus (bilaterally synchronous twitching of axial structures, often with eye opening and upward deviation of the eyes) that occurs within 24 to 48 h after cardiac arrest No pupillary light reflexes 24 to 72 h after cardiac arrest No corneal reflexes 72 h after cardiac arrest Extensor posturing or no response elicited by painful stimuli 72 h after cardiac arrest No N20 on somatosensory evoked potentials (SEP) or a serum neuron-specific enolase level of > 33 g/L If patients were treated with hypothermia, 72 h should be added to the times above because hypothermia slows recovery. If none of the above criteria is met, outcome is usually (but not always) poor; thus, whether to withdraw life support may be a difficult decision.
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Treatment Immediate stabilization (airway, breathing, circulation, or ABCs) Supportive measures, including, when necessary, control of ICP Admission to an ICU Treatment of underlying disorder Airway, breathing, and circulation must be ensured immediately. Hypotension must be corrected (see Shock and Fluid Resuscitation: Cardiogenic shock). Patients are admitted to the ICU so that respiratory and neurologic status can be monitored. Because some patients in coma are undernourished and susceptible to Wernicke encephalopathy, thiamin 100 mg IV or IM should be given routinely. If plasma glucose is low, patients should be given 50 mL of 50% dextrose IV. If opioid overdose is suspected,naloxone

2 mg IV is given. If trauma is involved, the neck is immobilized until damage to the cervical spine is ruled out. If a recent (within about 1 h) drug overdose is possible, gastric lavage can be done through a largebore orogastric tube (eg, 32 Fr) after endotracheal intubation. Activated charcoal can then be given via the orogastric tube. Endotracheal intubation: Patients with any of the following require endotracheal intubation to prevent aspiration and ensure adequate ventilation:

Infrequent, shallow, or stertorous respirations Low O2 saturation (determined by pulse oximetry or ABG measurements) Impaired airway reflexes Severe unresponsiveness (including most patients with a Glasgow Coma Scale score 8 If increased ICP is suspected, intubation should be done via rapidsequence oral intubation (using a paralytic drug) rather than via nasotracheal intubation; nasotracheal intubation in a patient who is breathing spontaneously causes more coughing and gagging, thus increasing ICP, which is already increased because of intracranial
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abnormalities. To minimize the increase in ICP that may occur when the airway is manipulated, some clinicians recommend giving lidocaine

1.5 mg/kg IV 1 to 2 min before giving the paralytic. Patients are sedated before the paralytic is given. Etomidate

is a good choice in hypotensive or trauma patients because it has minimal effects on BP; IV dose is 0.3 mg/kg for adults (or 20 mg for an average-sized adult) and 0.2 to 0.3 mg/kg for children. Alternatively, if hypotension is absent and unlikely and if propofol

is readily available, propofol

0.2 to 1.5 mg/kg may be used. Succinylcholine

1.5 mg/kg IV is typically used as a paralytic. However, use of paralytics is minimized and, whenever possible, avoided because they can mask neurologic findings and changes. Pulse oximetry and ABGs (if possible, end-tidal CO2) should be used to assess adequacy of oxygenation and ventilation. ICP control: If ICP is increased, intracranial and cerebral perfusion pressure should be monitored (see Approach to the Critically Ill Patient: Intracranial Pressure Monitoring), and pressures should be controlled. The goal is to maintain ICP at 20 mm Hg and cerebral perfusion pressure at 50 to 70 mm Hg. Cerebral venous drainage can be enhanced (thus lowering ICP) by elevating the head of the bed to 30 and by keeping the patient's head in a midline position.

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Control of increased ICP involves several strategies:

Sedation: Sedatives may be necessary to control agitation, excessive muscular activity (eg, due to delirium), or pain, which can increase ICP. Propofol

is often used in adults (contraindicated in children) because onset and duration of action are quick; dose is 0.3 mg/kg/h by continuous IV infusion, titrated gradually up to 3 mg/kg/h as needed. An initial bolus is not used. The most common adverse effect is hypotension. Prolonged use at high doses can cause pancreatitis. Benzodiazepines (eg, midazolam

, lorazepam

) can also be used. Because sedatives can mask neurologic findings and changes, their use should be minimized and, whenever possible, avoided. Antipsychotics should be avoided if possible because they can delay recovery. Sedatives are not used to treat agitation and delirium due to hypoxia; O2 is used instead. Hyperventilation: Hyperventilation causes hypocapnia, which causes vasoconstriction, thus decreasing cerebral blood flow globally. Reduction in PCO2 from 40 to 30 mm Hg can reduce ICP about 30%. Hyperventilation that reduces PCO2 to 28 to 33 mm Hg decreases ICP for only about 30 min and is used by some clinicians as a temporary measure until other treatments take effect. Aggressive hyperventilation to < 25 mm Hg should be avoided because it may reduce cerebral blood flow excessively and result in cerebral ischemia. Other measures may be used to control increased ICP (see Traumatic Brain Injury (TBI): Increased intracranial pressure). Hydration: Isotonic fluids are used. Providing free water through IV fluids (eg, 5% dextrose, 0.45% saline) can aggravate cerebral edema and should be avoided. Fluids may be restricted to some degree, but patients should be kept euvolemic. If patients have no signs of dehydration or fluid overload, IV fluids with normal saline can be started at 50 to 75 mL/h. The rate can be increased or decreased
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based on serum Na, osmolality, urine output, and signs of fluid retention (eg, edema). Diuretics: Serum osmolality should be kept at 295 to 320 mOsm/kg. Osmotic diuretics (eg, mannitol

) may be given IV to lower ICP and maintain serum osmolality. These drugs do not cross the blood-brain barrier. They pull water from brain tissue across an osmotic gradient into plasma, eventually leading to equilibrium. Effectiveness of these drugs decreases after a few hours. Thus, they should be reserved for patients whose condition is deteriorating or used preoperatively for patients with hematomas. Mannitol

20% solution is given 0.5 to 1 g/kg IV (2.5 to 5 mL/kg) over 15 to 30 min, then given as often as needed (usually q 6 to 8 h) in a dose ranging from 0.25 to 0.5 g/kg (1.25 to 2.5 mL/kg). Mannitol

must be used cautiously in patients with severe coronary artery disease, heart failure, renal insufficiency, or pulmonary vascular congestion because mannitol

rapidly expands intravascular volume. Because osmotic diuretics increase renal excretion of water relative to Na, prolonged use of mannitol

may result in water depletion and hypernatremia.Furosemide

1 mg/kg IV can decrease total body water, particularly when

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transient hypervolemia associated with mannitol

is to be avoided. Fluid and electrolyte balance should be monitored closely while osmotic diuretics are used. A 3% saline solution is being studied as another potential osmotic agent to control ICP. BP control: Systemic antihypertensives are needed only when hypertension is severe (>180/95 mm Hg). How much BP is reduced depends on the clinical context. Systemic BP needs to be high enough to maintain cerebral perfusion pressure even when ICP increases. Hypertension can be managed by titrating a nicardipine

drip (5 mg/h, increased by 2.5 mg q 5 min to a maximum of 15 mg/h) or by boluses of labetalol

(10 mg IV over 1 to 2 min, repeated q 10 min to a maximum of 150 mg). Corticosteroids: These drugs are usually helpful for patients with a brain tumor or brain abscess, but they are ineffective for patients with head trauma, cerebral hemorrhage, ischemic stroke, or hypoxic brain damage after cardiac arrest. Corticosteroids increase plasma glucose; this increase may worsen the effects of cerebral ischemia and complicate management of diabetes mellitus. After an initial dose of dexamethasone

20 to 100 mg, 4 mg once/day appears to be effective while minimizing adverse effects. Dexamethasone

can be given IV or po.

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If ICP continues to increase despite other measures to control it, the following may be used:

Pentobarbital coma: Pentobarbital

can reduce cerebral blood flow and metabolic demands. However, its use is controversial because the effect on clinical outcome is not consistently beneficial. Coma is induced by giving pentobarbital

10 mg/kg IV over 30 min, followed by 5 mg/kg/h for 3 h, then 1 mg/kg/h. The dose may be adjusted to suppress bursts of EEG activity, which is continuously monitored. Hypotension is common and is managed by giving fluids and, if necessary, vasopressors. Other possible adverse effects include arrhythmias, myocardial depression, and impaired uptake or release of glutamate. Decompressive craniotomy: Craniotomy with duraplasty can be done to provide room for brain swelling. This procedure can prevent deaths, but overall functional outcome may not improve much. It may be most useful for large cerebral infarcts with impending herniation, particularly in patients < 50 yr. Long-term care: Patients require meticulous long-term care. Stimulants, sedatives, and opioids should be avoided. Enteral feeding is started with precautions to prevent aspiration (eg, elevation of the head of the bed); a percutaneous endoscopic jejunostomy tube is placed if necessary. Early, vigilant attention to skin care, including checking for breakdown especially at pressure points, is required to prevent pressure ulcers. Topical ointments to prevent desiccation of the eyes are beneficial. Passive range-of-motion exercises done by physical therapists and taping or dynamic flexion splitting of the extremities may prevent contractures. Measures are also taken to prevent UTIs and deep venous thrombosis. Key Points
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Coma and impaired consciousness require dysfunction of both cerebral hemispheres or dysfunction of the reticular activating system. Manifestations include abnormalities of the eyes (eg, abnormal conjugate gaze, pupillary responses, and/or oculocephalic or oculovestibular reflexes), vital signs (eg, abnormal respirations), and motor function (eg, flaccidity, hemiparesis, asterixis, multifocal myoclonus, decorticate or decerebrate posturing). Taking a complete history of prior events is critical; ask witnesses and relatives about the time course for the change in mental status and about possible causes (eg, recent travel, ingestion of unusual meals, exposure to possible infections, drug or alcohol use, possible trauma). Do a general physical examination, including thorough examination of the head and face, skin, and extremities and a complete neurologic examination (focusing on level of consciousness, the eyes, motor function, and deep tendon reflexes), followed by appropriate blood and urine tests, toxicology screening, and fingerstick plasma glucose measurements. Do noncontrast CT immediately as soon as the patient has been stabilized. Ensure adequate airways, breathing, and circulation. Give IV or IM thiamin and IV glucose if plasma glucose is low and IV naloxone

if opioid overdose is suspected. Control ICP using various strategies, which may include sedatives (as needed) to control agitation, temporary hyperventilation, fluids and diuretics to maintain euvolemia, and antihypertensives to control BP. Last full review/revision September 2012 by Kenneth Maiese, MD Content last modified November 2012 http://www.merckmanuals.com/professional/neurologic_disorders/coma_and_impa ired_consciousness/overview_of_coma_and_impaired_consciousness.html

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