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Viral infections are produced by both
conventional viruses which contain DNA or RNA and unconventional (slow /
prions ) viruses which contain neither DNA nor RNA.
They gain access from the skin, the
gastrointestinal tract or the respiratory system to the CNS via neural (axoplasmic
and perineural lymphatics ) and more commonly, hematogenous routes.
They may cause only relatively mild systemic
illnesses; however if they invade the CNS, they may be fatal or cause
severe morbidity.
The most common acute viral encephalitis that
requires neurosurgical intervention is herpes simplex.
The most common post or para infectious viral
encephalopathy encountered in neurosurgery is Reye's syndrome.
The most common unconventional (slow ) viral
disorder of interest to the neurosurgeons is Creutzfeldt-Jakob disease.
A virus etiology of CNS tumors is an area of
interest to the neurosurgeons.
HERPES
SIMPLEX :
It is the
commonest virus encephalitis and caused by Herpes Simplex Virus- type I
(oral) with spread to CNS.
Latent
infection of trigeminal ganglia is common (oral-facial herpes).
Congenital
infection caused by type 2 (genital) virus produces a neonatal
meningoencephalitis. All mothers with genital herpes should have a
cesarean section before their amniotic membranes have ruptured or within 4
hrs of rupture.
Pathology:
There is
acute necrotizing (often hemorrhagic) encephalitis and mild meningitis
with mild meningeal and perivascular lymphocytic infiltration. Microglial
activation and nodule formation are also seen. There may be neuronal
necrosis and loss (Neuronophagia ) and brain edema can be severe. Cowdry
type A intranuclear inclusions may be seen.
The
temporal lobes, orbital regions, insular cortices, cingulate gyri
and brain stem are more commonly involved.
Diagnosis:
Clinically,
there may be fever, personality changes, fluctuating level of
consciousness and seizures, mimicking a bacterial meningitis.
CSF shows
mononuclear leucocytosis, reduced glucose and elevated protein levels.
Recently developed PCR test for Herpes Simplex Virus in CSF is highly
sensitive.
EEG may
show periodic high-voltage discharges localized to the temporal lobes.
CT and
MRI scans demonstrate edema, hemorrhage in the temporal lobes and in the
orbital-frontal regions.
Biopsy
can confirm diagnosis by culture, histology or immunohistochemistry, but
rarely required these days.
Treatment:
Acyclovir
(10mg/kg intravenously every 8 hrs for three days ) is relatively
effective.
Sub-temporal
decompression may be life saving at times.
The
mortality is high and death may occur in 1-3 weeks. The survivors often
have severe neurological deficits due to extensive tissue necrosis and
involvement of temporal lobes often results in memory disorders and/or
seizures.
HERPES
ZOSTER (VARICELLA):
It is
often a reactivation of latent chickenpox virus infection and may occur in
all age groups but predominantly the elderly. It may also involve the
dorsal root ganglia, the trigeminal and the geniculate ganglion. The
virus spreads through the nerve to the nerve endings localized in the
dermatome corresponding to the root ganglion in which the infection is
reactivated. More than one dermatome may be involved at a time. Motor
involvement such as facial weakness or localized wasting of muscles may
occur occasionally.
Trigeminal
nerve involvement is the commonest of ' cephalic zoster '.
Facial
nerve involvement is called 'Ramsay-Hunt Syndrome '.
'
Otitc zoster ' involves the vestibulocochlear nerve.
Very
painful skin eruptions are called ' Shingles '.
Rarely
the virus will spread centripetally to cause an encephalomyelitis (usually
in severely immunocompromised patients).
Transverse
myelitis, Guillain-Barre syndrome, Reye's syndrome and acute cerebellar
ataxia may be associated, as in any other virus infection.
Acyclovir
( 5mg / kg intravenously every 8 hrs) is effective and the recovery
is usual with few residual effects. Post herpetic neuralgia may be
distressing.
Reye's
syndrome:
It is a
para infectious process and usually affects children on recovery from a
viral illness. Clinically, they develop headache, vomiting and confusional
states. There is severe brain edema and liver failure. If untreated , it
is fatal. Investigations reveal hypoglycemia and elevated serum ammonia.
Use of
aspirin in children and also in adults with viral infections is
blamed lately.
Creutzfeldt-Jakob
Disease:
It is a
presenile dementia caused by an unconventional virus which induces a
subacute spongiform encephalopathy. It usually affects the middle aged
with a mean survival time of 5 months.
Clinically,
there is progressive dementia with myoclonus which differentiates this
from Alzheimer's.
CT and
MRI scans reveal bilateral cortical atrophy.
Brain
biopsy may be required for diagnosis. The method of transmission is
iatrogenic through contaminated instruments.
OTHERS:
Epilemic
acute encephalitis
is caused by arbovirus (RNA viruses) infections.
It is seasonal (summer). There is widespread meningoencephalitis with no
viral inclusions. The diagnosis often is made only by convalescent
immunoglobulin.
There is
no viral-specific therapy is available. The mortality and morbidity
varies.
Rabies
is caused by bite of a rabid animal (dog, skunk, fox, etc.) There is a
long but variable incubation period - 2 months (average). Rhabdovirus (
RNA virus ) migrates to CNS via peripheral nerves. There is acute
encephalomyelitis (gray matter infection) and the lesions are distributed
in the spinal cord, brain stem, cerebellum and the temporal
lobes.
Viral
inclusions (Negri bodies) in neuronal cytoplasm are diagnostic.
Poliomyelitis
(
Polioencephalomyelitis ) is by poliovirus infection, enteric virus
(seasonal - summer months). The primary enteric infection is
followed by hematogenous spread to the CNS, certain strains are
neuronotropic. there is mild diffuse meningitis with microglial nodules,
neuronophagia and gliosis.
It is
extremely rare since the advent of polio vaccines.
AIDS-RELATED
(OPPORTUNISTIC
) INFECTIONS:
A
clinically important group of CNS infections occur in the
immune-suppressed patient. The advent of the AIDS epidemic has
increased the frequency of many of these diseases, but some are more
commonly seen in patients who are immunosuppressed for other reasons
(transplantation, chemotherapy).
In
addition to HIV infection, AIDS patients(are prone for infections due to
cytomegalovirus, herpes, typical and atypical mycobacteria, the fungi,
such as cryptococcus and toxoplasmosis due to T.gondii .
Primary
HIV infection of the CNS ( HIV-Associated dementia complex ):
HIV
infects macrophages and microglia but does not produce significant
infection of neurons or neuroglia. The principal pathology is aggregates
of macrophages and multinucleated cells in the white matter. Atrophy of
the brain with neuronal loss may occur in later stages.30 to 40 % of the
AIDS patients have neurological involvement.
The
neurologic effects of virus may be mediated by cytokines. The clinical
picture varies from a chronic asymptomatic state to full blown AIDS.
Rarely,
acute reversible encephalitis or meningitis, or acute myelopathy or
neuropathy occurs.
Subacute
encephalitis causes dementia. Chronic meningitis and peripheral neuropathy
have also been reported.
Vacuolar
degeneration of the spinal cord result in paresis, ataxia and
incontinence.
Most
patients develop CSF evidence of HIV infection at the time of
seroconversion.
The early
stages of the disease are pathophysiological and may be self limiting in a
few weeks and most patients die within six months.
Cytomegalovirus
infection:
Most
adults have been infected by the cytomegalic virus by the age of 35 years.
The infection is usually asymptomatic, chorioretinitis often a more
serious problem. Acquired cytomegalic virus disease is very rare except in
those who are immunosuppresed and is the most common opportunistic
infection in AIDS; also common in organ transplant.
Congenital
infection is by transplacental route. It is characterized by low
birth weight, jaundice, hepatosplenomegaly, thrombocytopenia, hemolytic
anemia, chorioretinitis, microcephaly and periventricular calcifications.
There is
nodular encephalomyelitis with predilection for the subependymal germinal
matrix surrounding the lateral ventricles.Calcification within the walls
of these ventricles occurs frequently.
At the
present time, there is no effective treatment or prophylaxis.
Progressive
Multifocal Leukoencephalopathy (PML):
It is a
demyelinating disorder caused by papovavirus (DNA) infection (JC virus)
usually occurring in patients with immunosuppression or lymphoid malignancies
(relatively common in AIDS). It is either a primary infection or
reactivation of latent infection (systemic JC virus infection is common in
the general population, but CNS disease is rare)
The virus
attacks oligodendroglia leading to demyelination; particularly involves
the cerebral hemispheres, but can involve posterior fossa also. There
typically is little inflammatory reaction other than for debris (
laden macrophages ). Oligodendrocytes have glassy intranuclear viral
inclusions. Reactive astrocytes often have enlarged, pleomorphic
nuclei.
There is
no effective therapy and the clinical course typically is 3-9 months until
death.
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