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ENCEPHALITIS

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WHAT IS ENCEPHALITIS?

Acute inflammation of the brain.


Children, elderly and those with a weak immune
system are those who are more prone to
encephalitis.
The treatment given and the chances to
recovery depend completely on the virus
involved and how severe the inflammation is.
Acute encephalitis : affects brain directly
Para - infectious : brain and spinal chord inflates
two weeks into contracting the virus or bacteria.

CAUSES

The exact cause of encephalitis is unknown.


Usually caused by a virus or sometimes even a bacterial
infestation as well as non infectious inflammatory
conditions may cause encephalitis .
Few viruses that may be instrumental in causing
encephalitis includes
1)Herpes simplex
2) Polio viruses
3) Mosquito borne viruses
4)Tick borne
5) Rabies
6) childhood viruses

RATE OF INCIDENCE

There were no studies that specifically addressed the


incidence of AES. However, there were studies from
various countries that mentioned the incidence of
encephalitis in different settings. Those studies
suggested an IR of 1.77 to 6.34 for tropical countries
and an IR of 0.51-7.4 for Western industrialized
countries.
An IR of 145-185 was recorded during an epidemic in
Nepal in 1997. A hospital-based study from Andhra
Pradesh, India, suggested an IR of 1. A review article
suggested that the minimum IR must be fixed at 6.0
based on earlier studies

Biological Base
Just as a thief with a copied key can open a lock that is not his,
a virus begins to enter a cell via a similar type of crime.
Outermost layer of virus has special protiens that latch onto a
cell and pick the molecular locks on its surface.
These locks, which cover the surface of a cell, are called
receptors.

When there is no virus, receptors are used to


convey important signals from the outside
world to the inside of the cell.
Unrelated viruses can attach (bind) at different
times to the same kind of receptor on a cell
surface.
A viruss preference for infecting a certain type
of cell (like a neuron) is called tropism, and is
caused by the specific interaction of the virus
with a receptor found on that kind of cell.

In addition to binding to a cell surface receptor,


a virus often has to bind to a second cell
surface receptor, called co-receptor. In these
cases, its only after this second interaction that
a virus can enter into a cell.

TYPES OF ENCEPHALITIS

Infectious encephalitis
Autoimmune encephalitis
Chronic encephalitis
Limbic encephalitis
HIV encephalitis
Encephalitis Lethargica

Infectious Encephalitis
Viruses are the most common agents that cause
Infectious Encephalitis. Within the British Isles
herpes simplex virus (the cold sore virus) is the
virus most frequently identified.
Some of the known types of Infectious
Encephalitis are:
o Herpes Simplex Encephalitis
o West Nile Encephalitis
o Tick Borne Encephalitis
o Japanese Encephalitis

Autoimmune Encephalitis
Autoimmune Encephalitis may be triggered by infection
in which case the term "Post-infectious Encephalitis"
is used. ADEM( Acute Disseminated Encephalomyelitis
) is a Post-infectious Encephalitis. The illness usually
follows in the wake of a mild viral infection (such as
those that cause rashes in childhood) or immunisations.
Typically there is a delay of days to two to three weeks
between the triggering infection and development of the
Encephalitis.
It has recently been recognised that there are other types
of Autoimmune Encephalitis resulting from an attack of
the brain by the body's immune system.

Some of the known types of Autoimmune


Encephalitis are:
o Acute Disseminated Encephalomyelitis
(ADEM)
o NMDA Receptor associated Encephalitis
[N-methyl D-aspartate (NMDA) ]
o Hashimotos Encephalopathy
o Rasmussen Encephalitis

Chronic Encephalitis
SSPE: A Chronic Encephalitis as a result of Measles
SSPE stands for Subacute Sclerosing Panencephalitis and refers
specifically to a type of Encephalitis which can follow natural
(wild) measles virus infection. After the initial measles infection,
the virus lies passive in brain cells. It does not cause SSPE for
several years (average 6 years) when eventually an inflammatory
response is initiated against the infected cells. It is more common
in children younger than 2 years who have had primary measles
infection, although the condition (SSPE) manifests itself much
later- older children and adults.
Unfortunately SSPE is a progressive form of Encephalitis without
a cure. Despite multiple attempts, no satisfactory treatment has
been developed. In a few cases there has been remission
following use of a certain drug or drug combination. However
most of those affected die within about 5 years of diagnosis.

Limbic Encephalitis
The term Limbic Encephalitis (LE) is used
when the limbic areas of the brain are inflamed
(swollen) and consequently not functioning
properly.

HIV Encephalitis

Human Immunodeficiency Virus (HIV) can affect the brain in


different ways.
HIV-meningoencephalitis is infection of the brain and the
lining of the brain by the HIV virus. It occurs shortly after the
person is first infected with HIV and may cause headache,
neck stiffness, drowsiness, confusion and/or seizures.
HIV-encephalopathy (HIV-associated dementia) is the result
of damage to the brain by longstanding HIV infection. It is a
form of dementia and occurs in advanced HIV infection.
Mild Neurocognitive Disorder is problems with thinking
and memory in HIV, however is not as severe as HIVencephalopathy. Unlike HIV-encephalopathy it can occur
early in HIV infection and is not a feature of Aquired
Immune Deficiency Syndrome - AIDS.

Encephalitis Lethargica
Encephalitis Lethargica (EL) is a serious sporadic
form of Encephalitis.
a form of encephalitis caused by a virus and
characterized by headache and drowsiness leading
to coma.
The term "sleeping sickness", where people seem to
fall asleep or freeze whilst eating or working was
first used to describe two cases in Vienna.

SYMPTOMATOLOGY

Initial Signs

Headache
Malaise
Anorexia
Nausea and Vomiting
Abdominal Pain

Symptoms

Fever
Headache
Behavioral changes
Altered level of consciousness
Focal neurological deficits
seizures

Developing Signs
Altered LOC: mild lethargy to
deep coma
Altered Mental Status: confused,
delirium, disorientation
Mental disorders: hallucination,
agitation, personality changes,
behavioral disorders, occasional
psychosis
Focal or general seizures in
>50% of severe cases.

Encephalitis with focus or


diffused neurological symptoms
Behavioral and personality
changes.
Decreased level of
consciousness.
Stiff neck, photophobia and
lethargy.
Generalized or localized seizure.
Acute confusion or amnestic
states.
Flaccid paralysis(10%)

Neurological Signs
Aphasia
Ataxia
Hemiparesis with hyperactive
tendon reflexes
Involuntary movements
Cranial nerve deficits (ocular
palsies, facial weakness)

Viral encephalitis clinical


symptoms
Typical presentation
Acute flu-like prodrome
High fever, severe headache
Altered consciousness (lethargy, drowsiness,
confusion, coma)
Seizures
Focal neurological signs

More subtle presentations

Low grade fever


Speech disturbances (dysphasia, aphasia)
Behavioural changes
Subacute and chronic presentations can be caused
by CMV, VZV, HSV (immuno-compromised)

Possible Complications
Permanent brain damage may occur in
severe cases of encephalitis. It can
affect:
Hearing
Memory
Muscle control
Sensation
Speech
Vision

DIAGNOSIS

DIAGNOSIS
Because the various types of encephalitis
produce similar symptoms, doctors cannot rely on
clinical features to differentiate among the many
causes of brain inflammation. The primary
objective in diagnosing viral encephalitis is to
determine if it is caused by:

Herpes simplex or other conditions that


can be treated with specific medications

Arboviruses or other viruses that can be


managed only by targeting symptoms

IMAGING TECHNIQUES
For suspected encephalitis scanning technique is often
the first diagnostic step.
Computerized tomography (CT) or magnetic resonance
imaging (MRI) scans can show the extent of the
inflammation in the brain and help differentiate
encephalitis from other conditions. MRIs are
recommended over CT scans because they can detect
injuries in parts of the brain that suggest infection with
herpes virus at the onset of the disease, while CT scans
cannot.
Electroencephalogram (EEG), which records brain waves,
may reveal abnormalities in the temporal lobe that
are indicative of herpes simplex encephalitis.

CEREBROSPINAL FLUID TESTS


When encephalitis is suspected, a sample of cerebrospinal
fluid is taken using a lumbar puncture.
The sample is taken to count white blood cells and
identify specific blood cell types, to measure proteins and
blood sugar levels, and to determine spinal fluid pressure.
Doctors use CSF to test for herpes simplex virus,
Epstein-Barr virus, varicella-zoster virus,
enteroviruses, and to look for the presence of antibodies
to the West Nile virus.
While cerebrospinal fluid tests may help diagnose
encephalitis, they cannot provide information on how
severe the disease will be.

BLOOD TESTS
Blood tests may be used to test for West Nile
virus and other arbovirus infections.
Blood and urine tests are used to isolate and
identify viruses.
Enzyme-linked immunosorbent assays
(ELISA), including IgM-capture ELISA (MAC-ELISA)
and IgG ELISA, can identify viruses that cause
encephalitis soon after infection.
Polymerase chain reaction (PCR) can identify
small amounts of viral DNA.

BRAIN BIOPSY
Tiny samples of brain tissue are surgically
removed for examination and testing for the
presence of the virus.
Tissue is prepared using staining techniques
and then viewed under an electron microscope.
In a few cases, the viruses in brain cells are able
to be cultured; that is, the viruses can actually be
made to replicate in samples.
A brain biopsy is the gold standard for
diagnosing rabies.

TREATMENT
The goals of treatment are to provide supportive care (rest, nutrition,
fluids) to help the body fight the infection, and to relieve symptoms.
Reorientation and emotional support for confused or delirious people
may be helpful.

Medications may include:

Antiviral medications, such as acyclovir (Zovirax) and


foscarnet (Foscavir) -- to treat herpes encephalitis or other severe
viral infections (however, no specific antiviral drugs are available to
fight encephalitis)
Antibiotics -- if the infection is caused by certain bacteria
Anti-seizure medications (such as phenytoin) -- to prevent seizures.
Steroids (such as dexamethasone) -- to reduce brain swelling (in
rare cases)
Sedatives -- to treat irritability or restlessness
Acetaminophen -- for fever and headache.

TREATING PROBABLE CAUSES OF


ENCEPHALITIS
Since it is difficult to determine the cause of
encephalitis, and rapid treatment is essential, clinical
guidelines recommend immediately administering
intravenously the antiviral drug acyclovir without waiting
to determine the cause of the illness.
Once the doctor receives results from diagnostic tests, drug
treatment depends on the cause of the encephalitis.
Antiviral drug treatments for specific causes of encephalitis
include:

Herpes Simplex Virus . Acyclovir is recommended.

Varicella-Zoster Virus . Acyclovir is recommended.


Ganciclovir or adjunctive corticosteroids may also be
considered.

Epstein-Barr Virus . Corticosteroids may be


used, although risks may outweigh benefits. (Acyclovir is
not recommended.)

ADDITIONAL TREATMENTS
Other encephalitis treatments are aimed at
reducing symptoms.
Seizures may be prevented by using oral
anticonvulsant drugs or intravenous
lorazepam (Ativan).
Sedatives may be prescribed for irritability or
restlessness.
Simple pain relievers may be used for fever
and headache.

Prognosis

Acute phase of illness different from person to


person
Some recover with slight disability, others
profound disability and a few need residential
care for a life time
Degree and type of damage
cause of inflammation
severity of the infection
area affected
delay in seeking treatment

Post encephalitis
presentation

Personality changes
Physical difficulties
Memory problems
Emotional problems
Problems with pain and
other sensations
Problems with daily
living skills
Fatigue
Hormone problems
Cognitive Problems
Problems with new
learning
Inability to understand
and communicate
Epilepsy
Inappropriate behavior
and poor social skills

Psychosocial Rehabilitation
After acute phase,
safe environment +gentle stimulation
spontaneous recovery
Later phase,
new skills, habits and strategies to cope
Family and Patient requirement of time to cope varies

Barbara Wilson Cutting Edge developments in


Neurological Rehabilitation and possible Future
directions (2011)
Comparison focused therapy (CFT) refocus emotional
responses from self-critical to self acceptance
Music intonation therapy- music engages brain and
behavior functions. Improve attention, memory,
executive functions, unilateral neglect, anxiety,
depression
Virtual reality assessments make more able for
patients to become more able to participate in
community life.
Restitution of working memory deficits by computerized
working memory training
Errorless learning for people with language deficits
Problem solving therapy for people with executive
function deficits

Recent trends
Extensive frontal gray matter volume reduction
related to a possible sequalae of encephalitis
(Evernsel & etal, 2015)
39 year old man
Normal growth till 2 years
At 2, a febrile illness with convulsions- unconscious for a
week. No treatment given
Could walk only at 5, no speech, lacking self-care,
disorganized behavior and mental retardation
Untreated encephalitis at 2 years damaged frontal region

Encephalitis sequalae causes


mental retardation
Frontal Necrosis leads to
disinhibition
Low dose of antipsychotic
and antiepileptic drugs for
symptomatic treatment

Fig 1. MRI of the brain showing


extensive frontal and mild right
parietal gliosis and encephalomalacia.

Encephalitis a service orphan: The need for


more research and access to
neuropsychology (Easton, Alkin & Dowell
2006)
Aimed to spread awareness of neuropsychology in
management of encephalitis
More psychological disorders in people with Acquired
Brain Injury
After acute phase of encephalitis, marked changes in
behavior
Memory problems, personality change, personality and
spatial disorders etc
Family and caregiver outcomes
Social Outcomes
Rehabilitation

Thank You

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