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DISEASES OF THE
CENTRAL NERVOUS
SYSTEM
KANWAL KHAN
LECTURER
ZCPT
Introduction
Infection of the nervous system can involve:
meninges (meningitis) or the brain substance
itself (encephalitis), or both
(meningoencephalitis) or causing brain abscess
Additionally, infections can be acute or chronic.
Also The organisms that are involved in infection
are bacterial, parasitic or viral or can be prions
Chronic (granulomatous)
meningitis
Slowly evolving meningitis.
Fewer signs of meningeal inflammation (headache, neck
stiffness, etc), and more findings of focal neurologic
damage (cranial nerves, focal sensorimotor deficit,
cognitive deterioration, etc).
Infectious and non-infectious forms of chronic
meningitis.
Most common infectious types are tuberculous and
cryptococcal meningitis.
Chronic (granulomatous)
meningitis
Atypical bacteria, such as brucella, spirochetes (syphilis
and Lyme) may produce subacute or chronic infections
and uncommon parasites such as ehrlichia can invade
the meninges.
Non-infectious types include carcinomatous meningitis
and some other granulomatous forms, like sarcoid.
With the diversity of causes and the nonspecificity of
presentation, diagnosis may be extremely difficult.
Tuberculous meningitis
occurs most often in children and debilitated and immune
incompetent adults.
results from seeding of a tuberculoma in the brain or
meninges.
The tuberculoma, in turn, arises from the hematogenous
spread from a primary focus (usually in the lung).
The patients present with headache, malaise, and fever.
Weight loss may be prominent.
A physical examination may show normal results, or nuchal
rigidity may be present.
Tuberculous meningitis
The thick basilar meningitis may produce hydrocephalus, cranial nerve palsies, or an
arteritis of the small penetrating arteries of the brain stem.
The CSF shows a moderate pleocytosis (usually fewer than 300 WBCs/cu mm), mostly
lymphocytes.
The level of protein is high, and the amount of sugar low (these changes may be mild
early in the course).
The organism is occasionally demonstrable by acid-fast bacillus (AFB) strains of the CSF
sediment
Routine cultures are negative, but specialized cultures may take 4 to 8 weeks to grow.
The chest x-ray and tuberculin skin test may be helpful
Polymerase chain reaction testing is rapid and fairly accurate.
Tuberculous meningitis
If the diagnosis of tuberculous meningitis is suspected
on clinical grounds, treatment should be instituted.
Isoniazide (INH), streptomycin, rifampin and
pyrazinamide are used in combination.
Ethambutol may be useful if given in high doses.
Treatment is continued for at least 6-9 months.
Corticosteroids may be helpful in reducing the
inflammatory response, which itself can contribute to
the patient's symptoms.
Sequelae are common
Pressure
Cells
(10 / l)
PMN
Lactate
(mmol/l)
Normal
< 20 cm
1-2
<1
> .5
<2
Acute
pyogenic
>20 cm
>1000
> 50%
< .4
(>.2)
>4
Chronic
variable
> 1000
Vary
< .4
> 0.45
>2
Aseptic
(viral)
< 20 cm
< 1000
<50
> .4
Vary
<2
Viral Encephalitis
Meningeal involvement is present in most forms of
encephalitis; however, the clinical picture is dominated by
evidence of brain dysfunction.
In addition to headache and fever, patients often have
strikingly depressed levels of consciousness, and seizures are
common.
Behavioral changes and focal neurologic signs are
sometimes present.
The CSF contains a moderate number of cells. The level of
protein is normal or high, and the amount of sugar is usually
normal.
Viral Encephalitis
The
a.
b.
c.
d.
Viral Encephalitis
Arboviruses
1. Arthropod borne viruses general term for many unrelated
viruses transmitted by arthropods
2. There are at least 5 major encephalitis viruses in the us
4. Transmitted by mosquito bite, and horses and birds are the
reservoir
5. High fever and horrible headache, sometimes paralysis
6. Eliminate vector mosquito (spray and eliminate standing water)
Viral Encephalitis
Rabies
A. Fatal encephalitis
B. Acquired by an animal bite or handling an infected
animal
C. Coyotes ,skunks, raccoons, bats (and others)
different strains
D. Type of infection with rabies: virus has an affinity
for nervous tissue, spreads from site of bite to
nervous tissue. Very slow progression towards the
CNS.
Viral Encephalitis
Polio viral infection
caused by small non-enveloped virus
(naked) called a Picorna virus
A. Enterovirus acquired by contaminated food or
water, fomites, and mechanical vectors too
B. Disease is usually limited to the throat, tonsils,
and lymph nodes, but it can infect the nerve
tissues, especially motor neurons of the spinal
chord, leading to paralysis or even death
Viral Encephalitis
D. Prevention vaccine
a. Salk vaccine inactivated viral vaccine, trivalent
(3strains)
b. Sabin is live attenuated virus
c. Salk for infants and Sabin for later, infant immune
system not as developed and there is some potential for
the attenuated virus become infective
WHO is till working on polio and measles to eradicate
them
COMPLICATIONS
Cerebral edema (may lead to herniation)
Vasculitis
Arteritis (stroke)
Cortical venous thrombosis (stroke, seizures)
Venous sinus thrombosis (increased intracranial pressure)
Hydrocephalus
Cranial nerve palsies
Subdural effusion or empyema
Disseminated intravascular clotting
Lactic acidosis
Inappropriate ADH secretion
Diabetes insipidus
Residual findings
Cranial nerve palsies
Mental retardation
Seizures
Brain Abscess
Can arise either from direct extension from a
parameningeal focus of infection (ear and sinus
infections) or by hematogenous spread.
Pulmonary pathology (especially bronchiectasis) is the
most common source of the hematogenous spread.
Persons with cyanotic congenital heart disease and
pulmonary arteriovenous malformations are also prone to
develop abscesses.
Brain Abscess
Bacteria originating in the bowel and reaching the vena
cava and the right side of the heart via the portal
system, liver, and hepatic veins are short-circuited to
the left side of the heart and systemic circulation.
Thus they miss filtration by the pulmonary macrophage
system. Although subacute and acute bacterial
endocarditis may be associated with mycotic aneurysms
and meningoencephalitis, it is infrequently the cause of
brain abscess.
Brain Abscess
In hematogenously spread abscesses, they are most
likely to occur in areas of ischemic injury to the brain
and most likely to include anaerobic or
microaerophilic organisms (such as come from
the bowel).
On the other hand, aerobic bacteria are frequently
cultured from abscesses that have sinus tracts
connecting them to the exterior, i.e., sinus infections,
middle-ear infections, and skull fractures.
Brain Abscess
There are two stages in the development of a bacterial brain
abscess.
In the first stage, the primary infection is often active,
brain infection is a cerebritis - an inflammatory response with
some tissue breakdown.
patient is usually febrile and may complain of headache.
The intracranial pressure is usually raised.
There may be focal signs, but lesions in the temporal lobes or
frontal lobes may have no symptoms localizing a lesion to the
brain.
Brain Abscess
The CT scan or MRI is usually abnormal and the EEG is usually focally
abnormal.
Arteriography does not show any well-defined mass.
The spinal fluid may show a pleocytosis, with a raised level of protein
and a normal amount of glucose, but it can be entirely normal.
Spinal tap in a patient with potentially elevated intracranial pressure
should only be done after scanning to ascertain the risk of potential
herniation
Brain Abscess
Treatment with antibiotics alone at this stage may
produce complete resolution and surgery is not
recommended due to the lack of clear margins or a
defined wall to the infection.
Brain Abscess
In the second stage, the region of the cerebritis
becomes organized and walled off, and a true abscess
forms.
Fever often subsides. There may be signs of an
expanding mass.
The CSF and EEG and brain scan are as before.
A mass is seen on CT scan or MRI.
Brain Abscess
Treatment with antibiotics alone may not be effective
because the abscess is walled off; surgical drainage
may be necessary.
Untreated, the brain abscess may cause cerebral
herniation or rupture into the ventricles, causing severe
(and often fatal) meningitis. The WBC count in the CSF
in the latter instance is often more than 10,000/cu mm.
Prion disease
This fascinating group of uncommon diseases is pathologically
similar and has the following characteristics:
They are transmissible experimentally.
They have a long latent period (up to many years).
Pathologically they resemble degenerative diseases; there is
neuronal degeneration with astrocytic reaction but no evidence
of inflammation.
Clinically they produce a chronic or subacute illness, which is
steadily progressive.
The nature of the infectious agent is an abnormally folded
protein.
Prion disease
Prions infectious proteins :
Kuru- cannibalistic rituals in new guinea
CJ Creutzfeldt-Jakob of humans
Mad cow disease in cattle bovine
spongiform encephalopathy