Encephalopathy

Clinicians often struggle with the distinction between delirium and encephalopathy.
By Richard Pinson, MD, FACP

The diagnosis and documentation of encephalopathy can be confusing and controversial.

Clinicians struggle with, for example, the distinction between delirium and encephalopathy,
and with recognizing encephalopathy when a patient with dementia seems to have an acute
alteration in baseline mental status.
A specific, authoritative definition of encephalopathy is hard to come by. Non-specific
descriptions abound online: any brain disease, disorder or disease of the brain, dysfunction of
the brain, degeneration of brain function, and brain damage or malfunction. The National
Institute of Neurological Disorders and Stroke (NINDS) has described encephalopathy as a
term for “any diffuse disease of the brain that alters brain function or structure” and says the
“hallmark of encephalopathy is an altered mental status.” It then proceeds to list a hodge-
podge of acute and chronic causes.
Categories of encephalopathy
There are 2 distinct categories of encephalopathy: acute and chronic (see Table). Many
sources confuse and confound these categories, lumping them together as one. The chronic
encephalopathies are characterized by chronic mental status alteration that, in most cases, is
slowly progressive (anoxic encephalopathy being an exception). They result from permanent,
usually irreversible, structural changes within the brain itself. Some may be halted or
reversed by early detection and treatment. Examples of the chronic encephalopathies include
anoxic brain injury; chronic traumatic encephalopathy; heavy metals (lead, arsenic, mercury,
etc); HIV-related; hereditary enzyme deficiencies; Korsakoff; and spongiform.
Acute encephalopathy is characterized by an acute or subacute global, functional alteration of
mental status due to systemic factors. It is reversible when these abnormalities are corrected,
with a return to baseline mental status. Acute encephalopathy may be further identified as
toxic, metabolic, or toxic-metabolic. Toxic encephalopathy describes acute mental status
alteration due to medications, illicit drugs, or toxic chemicals. Metabolic encephalopathy is
caused by any of a large number of metabolic disturbances. Toxic-metabolic encephalopathy
describes a combination of toxic and metabolic factors. Causes of acute toxic and metabolic
encephalopathy include acute organ failure such as hepatic and renal; alcohol; dehydration;
electrolyte imbalance; fever; hypertension; hypoxemia; illicit drugs; infections including
sepsis; medications; toxic chemicals; and Wernicke (thiamine deficiency).
 Acute intra-cranial processes (such as stroke or traumatic lesions) alone should not be
classified as acute encephalopathy but are more correctly considered an alteration of
consciousness (stupor or coma) or concussion.
In contrast to the generic term “encephalopathy,” the acute toxic and metabolic
encephalopathies as a group are well defined and well described. The 2013 Neurocritical
Care Society Practice Update states that “acute encephalopathy is synonymous with acute
confusional state, acute organic brain syndrome or delirium...[it] describes the clinical
presentation of a global cerebral dysfunction induced by systemic factors.”
Delirium vs. acute encephalopathy
Delirium and acute encephalopathy are essentially 2 different terms describing the same
condition. Delirium represents the mental manifestation while encephalopathy identifies the
underlying pathophysiologic process. This is why the American Psychiatric Association's
Diagnostic and Statistical Manual of Mental Disorders, Fifth Edition (DSM-5), classifies
acute toxic and metabolic encephalopathic states as delirium and does not use encephalopathy
in its definitions.
The coding classifications (ICD-9 and ICD-10) use “encephalopathy” to classify what DSM-
5 calls delirium. ICD relegates delirium to a symptom of lesser importance. To permit correct
coding for these cases, the term encephalopathy is needed to capture a true picture of the
patient's condition. Clinicians may continue to follow DSM definitions using delirium but
should also incorporate the necessary ICD terminology to prevent understating the severity of
illness of patients. Examples include:
 Toxic encephalopathy due to phenytoin, causing delirium
 Delirium due to metabolic encephalopathy
In fact, DSM-5 acknowledges this imperative in a “coding note” for delirium: “Include the
name of the other [underlying] medical condition in the name of the delirium (e.g., 293.0
[F05] delirium due to hepatic encephalopathy). The other medical condition should also be
coded and listed separately immediately before the delirium [hepatic encephalopathy first,
delirium second].”
Acute encephalopathy complicating dementia
A common clinical conundrum is presented by the patient with preexisting dementia who is
admitted with an altered mental status. Patients with dementia often experience transient,
episodic mental status fluctuation, and it may be difficult to determine if any change in
baseline has actually occurred. On the other hand, patients with dementia are particularly
vulnerable to acute encephalopathic changes with any metabolic or toxic stress.
When a significant alteration of mental status has occurred and systemic toxic or metabolic
factors are present, it is a rather simple matter to diagnose toxic or metabolic encephalopathy
when mental status returns to baseline as these abnormalities are corrected. If delirium due to
these underlying medical conditions is diagnosed, toxic or metabolic encephalopathy should
also be identified as its cause (see DSM-5 coding note above).
The chronic encephalopathies (such as Korsakoff, anoxic, or traumatic) are distinctly
different from acute toxic or metabolic encephalopathy. They result from permanent, usually
irreversible, structural changes within the brain itself. 

Toxic encephalopathy
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Toxic encephalopathy
Specialty Neurology 
Toxic encephalopathy is a neurologic disorder caused by exposure to neurotoxic organic
solvents such as toluene, following exposure to heavy metals such as manganese, as a side
effect of melarsoprol treatment for African trypanosomiasis, or exposure to extreme
concentrations of any natural toxin such as cyanotoxins found in shellfish or freshwater
cyanobacteria crusts. Toxic encephalopathy can occur following acute or chronic exposure to
neurotoxicants, which includes all natural toxins. Exposure to toxic substances can lead to a
variety of symptoms, characterized by an altered mental status, memory loss, and visual
problems. Toxic encephalopathy can be caused by various chemicals, some of which are
commonly used in everyday life, or cyanotoxins which are bio-accumulated from harmful
algal blooms (HABs) which have settled on the benthic layer of a waterbody. Toxic
encephalopathy can permanently damage the brain and currently treatment is mainly just for
the symptoms.

Contents
 1Signs and symptoms
 2Causes
 3Diagnosis
 4Treatment
 5Prognosis
 6Research
 7See also
 8Notes
 9References
 10External links
Signs and symptoms[edit]
"Encephalopathy" is a general term describing brain malfunctions and "toxic" asserts that the
malfunction is caused by toxins on the brain. The most prominent characteristic of toxic
encephalopathy is an altered mental status. Acute intoxication is a reversible symptom of
exposure to many synthetic chemical neurotoxicants. Acute intoxication symptoms include
lightheadedness, dizziness, headache and nausea, and regular cumulative exposure to these
toxic solvents over a number of years puts the individual at high risk for developing toxic
encephalopathy. Chronic exposure to low levels of neurotoxic chemicals can also cause
reversible changes in mood and affect which resolve with cessation of exposure. Acute and
chronic toxic encephalopathy on the other hand, are persistent changes in neurological
function that typically occur with exposure to higher concentrations and longer durations
respectively. The symptoms of acute and chronic toxic encephalopathy do not resolve with
cessation of exposure and can include memory loss, small personality changes/increased
irritability, insidious onset of concentration difficulties, involuntary movements
(parkinsonism), fatigue, seizures, arm strength problems, and depression.[1] A paper by
Feldman and colleagues described neurobehavioral effects in a 57-year-old house painter
with regular exposure to large amounts of solvents.[2].
Magnetic Resonance Imaging (MRI) analyses have also demonstrated increased rates
of dopamine synthesis in the putamen, reduced anterior and total corpus callosum volume,
demyelination in the parietal white matter, basal ganglia, and thalamus, as well as atypical
activation of frontal areas of the brain due to neural compensation. A thorough and standard
diagnostic process is paramount with toxic encephalopathy, including a careful occupational
history, medical history, and standardized imaging/neurobehavioral testing.
Causes[edit]
In addition, chemicals, such as lead, that could instigate toxic encephalopathy are sometimes
found in everyday products such as cleaning products, building materials, pesticides, air
fresheners, and even perfumes. These harmful chemicals can be inhaled (in the case of air
fresheners) or applied (in the case of perfumes).[3][4] The substances diffuse into the brain
rapidly, as they are lipophilic and readily transported across the blood–brain barrier. This is a
result of increased membrane solubility and local blood flow, with central nervous system
(CNS) solvent uptake being further increased with high levels of physical activity.[5] When
they are not detoxified immediately, the symptoms of toxic encephalopathy begin to emerge.
[4]
 However, in chronic situations, these effects may not become severe enough to be noticed
until much later. Increased exposure time and increased concentration of the chemicals will
worsen the effects of toxic encephalopathy, due to the associated structural CNS damage and
direct functional impairment consequences.[5]
Diagnosis[edit]
Rapid diagnosis is important to attempt to prevent further damage to the brain and further
neurologic deficits. It is a diagnosis of exclusion, so a full work up for other possible
etiologies (hepatic, uremic, infectious, oncologic) should be performed. Screening for heavy
metals, as well as other toxins, should be done immediately as those are some of the most
common causes and the patient can then remove themselves from the dangerous
environment. In addition, a full examination of blood (CBC) and metabolites (CMP) should
be done.
Treatment[edit]
Treatment is mainly for the symptoms that toxic encephalopathy brings upon victims, varying
depending on how severe the case is. Diet changes and nutritional supplements may help
some patients. To reduce or halt seizures, anticonvulsants may be prescribed. Dialysis or
organ replacement surgery may be needed in some severe cases.[6]
Management of affected individuals consists of immediate removal from exposure to the
toxic substance(s), treatment of the common clinical manifestation of depression if present,
and counselling for the provision of life strategies to help cope with the potentially
debilitating condition.[5]
Prognosis[edit]
Toxic encephalopathy is often irreversible. If the source of the problem is treated by
removing the toxic chemical from the system, further damage can be prevented, but
prolonged exposure to toxic chemicals can quickly destroy the brain.[7] Long term studies
have demonstrated residual cognitive impairment (primarily attention and information-
processing impairment resulting in dysfunction in working memory) up to 10 years following
cessation of exposure.[5] Severe cases of toxic encephalopathy can be life-threatening.[8]

Encephalopathy means brain disease, disorder, or damage. The term refers to temporary or
permanent conditions that affect the brain's structure or function.
The main symptom of encephalopathy is a change to a person's mental state.
There are different types of encephalopathy, with distinct causes, and encephalopathy can be
a complication of another condition.
This article discusses types, causes, and symptoms. It also discusses how doctors can treat
encephalopathy.
Causes and types of encephalopathy
Share on PinterestEncephalopathy is when the brain changes due to damage, disease, or a
disorder.
The word encephalopathy can refer to all kinds of brain damage and diseases.
There are many types of encephalopathy, and they can be grouped by their cause.
Broadly, encephalopathy can arise from physical injury, infection, or other medical
conditions.
Below, we explore the types of encephalopathy and their causes:
Physical injury
When encephalopathy results from repeated trauma, it is called chronic traumatic
encephalopathy.
Trauma to the head may lead to nerve damage that affects brain function. Chronic traumatic
encephalopathy can develop following multiple brain traumas or injuries over time. These
may result from a series of accidents or blows to the head.
People in the military or who play contact sports may have a higher risk of chronic traumatic
encephalopathy.
Toxins
If toxins build up in the blood and reach the brain, they can cause damage. This may result
from an underlying condition, infection, or exposure to toxic chemicals.
There are three types of encephalopathy linked with toxins:
  Uremic encephalopathy: This results from uremic toxins building up in the blood
when a person has kidney failure.
 Hepatic encephalopathy: This happens if liver disease causes toxins to build up in a
person's blood.
 Toxic metabolic encephalopathy: This occurs when toxic chemicals, or a chemical
imbalance caused by an infection, affects brain function.
High blood pressure
If a person does not receive treatment for high blood pressure, it can lead to swelling in the
brain. When this results in a brain injury, it is called hypertensive encephalopathy.
Lack of oxygen
If the brain does not get enough oxygen, a person may experience brain damage.
Encephalopathy caused in this way is called hypoxic ischemic encephalopathy.
Vitamin B-1 deficiency
When a person has a vitamin B-1 deficiency, they can develop a type of brain disease called
Wernicke encephalopathy.
Vitamin B-1 deficiency may result from:
 alcohol use disorder
 malnutrition
 problems absorbing food in the gut
Prion diseases
Infectious encephalopathy can be the most serious type. It sometimes results from a rare
group of conditions called prion diseases or transmissible spongiform encephalopathies.
These progressive diseases are linked to the mutation of a protein called prion.
Prion diseases are neurogenerative. This means that they damage the brain and cause the
brain's function to get worse, or deteriorate, over time. The main feature of prion disease is
tiny holes in the brain that give it a spongy appearance.
Examples of prion diseases that can lead to infectious encephalopathy include:
 fatal familial insomnia
 kuru
 chronic wasting disease
 Creutzfeldt-Jakob disease
Inherited conditions
Some types of encephalopathy are related to genetics, and a person is more likely to develop
one if a family member has it.
Two inherited forms of encephalopathy are:
 Hashimoto's encephalopathy: This relates to an autoimmune disease called
Hashimoto's disease, which affects the thyroid. The exact cause of Hashimoto's is
unknown, but doctors believe that it is inherited.
 Glycine encephalopathy: This occurs when there is too much of the amino acid
glycine in the brain. It can affect brain function.
Encephalopathy vs. encephalitis
Encephalopathy and encephalitis both affect the brain, but there are significant differences.
Encephalitis refers to inflammation in the brain, which often results from a viral infection.
Encephalopathy refers to permanent or temporary brain damage, disorder, or disease. It
affects the brain's function or structure and may be degenerative.
Symptoms of encephalopathy
Share on PinterestFatigue, difficulty concentrating, and forgetfulness are potential symptoms
of encephalopathy.
The main symptom of encephalopathy is an altered mental state. This can involve:
 having trouble staying alert
 having problems thinking or processing information
 feeling confused
 forgetting things
 behaving out of character
 finding it hard to concentrate
 feeling very tired
These symptoms may get worse over time. The progression depends on the type of
encephalopathy and how severe it is.
In addition to an altered mental state, a person with encephalopathy may experience:
 involuntary muscle twitching
 involuntary eye movements
 shaking
 muscle weakness
 trouble swallowing or speaking
  seizures
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Diagnosis
If a person has symptoms of encephalopathy, they should see a doctor immediately.
The doctor may use the following to reach a diagnosis:
 blood tests
 a spinal fluid examination
 imaging studies, such as MRI or CT scans
 electroencephalograms, which is a method of recording the electrical activity of the
brain
The results of these tests will help the doctor determine:
 if a person has encephalopathy
 what type of encephalopathy a person has
 what the underlying cause is
Treatments
Share on PinterestA doctor may recommend surgery to treat encephalopathy.
Once a doctor has determined the cause of a person's encephalopathy, they can recommend
the best treatment.
The doctor may suggest various medications to slow the progression of the damage.
In some cases, people may require surgery. If a person is having seizures, the doctor may
prescribe anticonvulsant drugs.
Complications
Severe encephalopathy may lead to loss of consciousness or coma. If this happens, the doctor
will put the person on life support to help them recover.
In rare cases, encephalopathy may lead to permanent brain damage or loss of life.
Prevention
Not all forms of encephalopathy are preventable. However, a person may be able to lower
their risk by:
  eating a healthful, balanced diet
 reducing alcohol consumption
 avoiding exposure to toxic chemicals
Outlook
Encephalopathy refers to a broad range of conditions that affect the brain's function,
including brain damage and disease. The primary symptom is an altered mental status.
Doctors can often treat encephalopathy, and many people make a full recovery. With
treatment, impaired brain function may be reversed.
However, certain types of encephalopathy are life-threatening. Infectious encephalopathy
caused by prion disease is an example of this.
According to the Centers for Disease Control and Prevention (CDC), prion diseases are rare,
but they always lead to loss of life. Research into treatments is ongoing.
When they are severe, all types of encephalopathy can be life-threatening.
Some people with severe encephalopathy may develop permanent brain damage. If this
happens, the doctor will support the person and their family and help them manage the
condition.
A doctor can provide detailed information about an individual's outlook. In some cases,
family members can consult with the doctor on the person's behalf.