Encephalopathy

Definitions

Encephalopathy is a general term for any diffuse disease of the brain that alters brain function or structure. The hallmark of encephalopathy is an altered mental status.

It is important to document the presence of “encephalopathy” to accurately reflect severity of illness and complexity of care.
There are many types of encephalopathy as reviewed below. It is important to document the type, as well as underlying cause, if known.

Encephalopathy Types in Children

    
                Type
                Description and Causes
            
                Metabolic
                Abnormalities of water, electrolytes, vitamins, and other chemicals with adverse brain function. Causes: infections, toxins, sepsis, multiple organ failure, brain tumor, brain metastasis, uremia, cerebral ischemia or cerebral infarction. Can be seen with severe DKA.
            
                Septic
                An end-organ failure (in this case the brain) caused by a systemic inflammatory response due to an infection somewhere in the body. Indicative of SEVERE sepsis.
            
                Toxic
                Due to drugs; also may be called toxic metabolic encephalopathy. Often seen in drug overdoses.
            
                Infectious
                Bacteria, viruses, and/or fungi causing encephalitis with altered mental status by infection and inflammation of the brain tissue or meninges lining the brain and spinal cord.
            
                Traumatic/ Post Concussion
                GCS <13 and/or moderate to severe symptoms** and/or symptoms persisting beyond expected timeline (includes patients with post-concussive syndrome).
            
                Hepatic
                Severe liver disease causing a build-up of bloodstream toxins, leading to brain damage. Causes: conditions that reduce liver function (i.e. cirrhosis, hepatitis, etc.) or conditions in which blood circulation does not enter the liver.
            
                Neoplastic disease
                Can be directly related to cancer or a secondary result from treatment. Generally also encompassed in another category listed here.
            
                Hypertensive
                Hypertension severe enough to affect brain function. Causes: acute nephritis, crises in chronic essential hypertension; sudden withdrawal of antihypertensive treatments.
            
                Anoxic
                Brain tissue oxygen deprivation with a global loss of brain function. Causes: cardiac arrest, prolonged seizures without adequate breathing, status asthmaticus, trauma, etc.
            
                Ischemic
                Brain tissue small blood vessel narrowing with a generalized decrease in brain blood flow.
            
    **Moderate to severe symptoms with Traumatic Encephalopathy:
    Typically interfere with ADLs & routine activities.
Significant agitation, irritability, or combativeness.
Significant behavior or personality changes.
Significant memory loss (beyond amnesia of events of accident).
Significant balance problems, dizziness, or coordination problems.
Physical symptoms: headaches, vomiting, and seizures with severity/persistence beyond expected course.

Neonatal Encephalopathy

Hypoxic Ischemic Encephalopathy (HIE) is brain injury due to asphyxia. The primary causes of condition are systemic hypoxemia and/or reduced cerebral blood flow [12].

4 diagnostic criteria of HIE (Neosource)

Guidelines from the American Academy of Pediatrics (AAP) and the American College of Obstetrics and Gynecology (ACOG) for hypoxic-ischemic encephalopathy indicate all of the following must be present for the designation of perinatal asphyxia or HIE:

Profound metabolic or mixed acidemia (pH < 7) in an umbilical artery blood sample
Persistence of an Apgar score of 0-3 for longer than 5 minutes
Neonatal neurologic sequelae (i.e. post-concussive syndrome)
Multiple organ involvement (eg, kidney, lungs, liver, heart, intestines)

Kernicterus or Bilirubin Encephalopathy is a neurologic syndrome resulting from the deposition of unconjugated (indirect) bilirubin in the basal ganglia and brainstem nuclei. [7]

Common initial signs are lethargy, poor feeding, and loss of the moro reflex [7]

Infectious Encephalopathy is the result of many types of bacteria, viruses and fungi which can cause encephalitis by infection and inflammation of the brain tissue or meninges that line the brain and spinal cord.[6]

Possible complications/symptoms: irritability, poor feeding, hypotonia, floppy baby syndrome, seizures, death

Grading System for Pre-cooling Exam

    
        
                Category
                Normal/Mild
                Moderate
                Severe
            

        
                1. Level of Consciousness
                1
                2 = Lethargic
                3 = Stupor/coma
            

                2. Spontaneous Activity
                1
                2 = Decreased activity
                3 = No activity
            

                3. Posture
                1
                2 = Distal flexion, complete extension
                3 = Decerebrate
            

                4. Tone
                1
                2 = Hypotonia (focal or general)
                3 = Flaccid
            

                5. Primitive Reflexes (any)
                Suck: 1
                Suck: 2 = Weak
                Suck: 3 = Absent
            

                Moro: 1
                Moro: 2 = Incomplete
                Moro: 3 = Absent
            

                6. Autonomic System (any)
                Pupils: 1
                Pupils: 2 = Constricted
                Pupils: 3 = Deviation/dilated/non-reactive to light
            

                Heart rate: 1
                Heart rate: 2 = Bradycardia
                Heart rate: 3 = Variable HR
            

                Respiration: 1
                Respiration: 2 = Periodic breathing
                Respiration: 3 = Apnea
            

    