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INTRODUCTION
Demyelinating diseases, though well-known phenomena in the
medical milieu are still subject to a lot of research. Due to their
varied prevalence among various units of a populace, and
within sub-units of any given population, with sub-types
affecting people of different ages, races, and both genders,
demyelinating diseases remain a relevant topic of research and
study.
This paper shall attempt to discuss the medical imaging of
such disorders, with eventual emphasis on multiple sclerosis, it
being the most common autoimmune disorder of the central
nervous system.
Emphasis will obviously be put upon the incidence and
imaging thereof, among patients in Constanta, Romania.
CHAPTER 1
1.1 Definition
A demyelinating disease is any disease affecting the
central nervous system, which results in damage to the myelin
sheath of neurons. This damage impairs the conduction of
signals in the affected nerves. In turn, the reduction in
conduction ability causes deficiency in sensation, movement,
cognition, or other functions depending on which nerves are
involved (1).
Some demyelinating diseases are caused by genetics, some
by infectious agents, others by autoimmune reactions, and a
few by unknown factors and agents. Organophosphates, a class
of chemicals which are the active ingredients in commercial
insecticides such as sheep dip, weed-killers, and flea treatment
preparations for pets, etc., will also demyelinate nerves.
Neuroleptics can also cause demyelination(2).
Lysophosphatidylcholine causes demyelination and is in
unnaturally high amounts in foods with lecithin treated with the
enzyme phospholipase (enzyme-modified foods) and as
lysolecithin in products such as make up and personal care
products.
1.2 Classification
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1.3 Pathophysiology
In the most known example, Multiple Sclerosis (MS), there
is good evidence that the body's own immune system is at
least partially responsible. Acquired immune system cells called
T-cells are known to be present at the site of lesions. Other
immune system cells called Macrophages (and possibly Mast
cells as well) also contribute to the damage.
Some demyelinating diseases are caused by genetics, some
by infectious agents, some by autoimmune reactions, some by
exposure to chemical agents, and some by unknown factors.
The role of prolonged cortical myelination in human evolution
has been implicated as a contributing factor in some cases of
demyelinating disease. Unlike other primates, humans exhibit a
unique pattern of post pubertal myelination, which may
contribute to the development of psychiatric disorders and
neurodegenerative diseases that present in early adulthood
and beyond. The extended period of cortical myelination in
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1.5 Symptoms
That present in demyelinating diseases are different for each
condition. Below is a list of symptoms that can present in a
person with a demyelinating disease:
Ataxia
Clonus
Dysarthria
Fatigue
Clumsiness
Hand paralysis
Hemiparesis
Genital anaesthesia
Incoordination
Paraesthesia
Ocular paralysis
Weakness (muscle)
Loss of sensation
Impaired vision
Neurological symptoms
Unsteady gait
Spasticity
Incontinence
Hearing problems
Speech problems
gadolinium contrast
Position abutting ventricles (often perpendicular)
Juxtacortical position (gray-white junction)
Involvement of brainstem, cerebellum, or corpus callosum
Spinal cord lesions
One or two vertebral segments in length
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Artificial joints
Staples
Many cardiac valve replacements (check with physician)
Disconnected medication pumps
Vena cava filters
Brain shunt tubes for hydrocephalus
Projectile risk
The extremely high strength of the magnetic field can
cause projectile effect (or "missile-effect") accidents. This
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Pregnancy
No effects of MRI on the foetus have been proven. MRI
avoids the use of ionizing radiation, to which the foetus is
particularly sensitive. However, as a precaution, current
guidelines recommend that pregnant women undergo MRI
only when essential. This is particularly the case during
the first trimester of pregnancy, as organogenesis takes
place during this period. The concerns in pregnancy are
the same as for MRI in general, but the foetus may be
more sensitive to the effectsparticularly to heating and
to noise.
Despite these concerns, MRI is rapidly growing in
importance as a way of diagnosing and monitoring
congenital defects of the foetus because it can provide
more diagnostic information than ultrasound and it lacks
the ionizing radiation of CT (7).
MRI vs CT
MRI and computed tomography (CT) are complementary
imaging technologies and each has advantages and
limitations for particular applications. A concern is the
potential for CT to contribute to radiation-induced cancer
and in 2007 it was estimated that 0.4% of current cancers
in the United States were due to CTs performed in the
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past, and that in the future this figure may rise to 1.52%
based on historical rates of CT usage.
An advantage of MRI is that no ionizing radiation is used
and so it is recommended over CT when either approach
could yield the same diagnostic information.
However, though MRI cost has fallen, thus making it
more competitive with CT, there are not many common
imaging scenarios in which MRI can simply replace CT,
although this substitution has been suggested for the
imaging of hepatic disorders.
The effect of low doses of radiation on carcinogenesis is
also disputed. Although MRI is associated with certain
biological effects, these have not been proven to cause
significant and measurable harm (8).
Regardless of the benefts of CT, let us note that it has no
bearing upon the diagnosis of MS.
MRI remains the absolute imaging method for the positive
diagnosis of the disease.
Practical Guidelines
Simple practical guidelines/applications for the MRI procedure
include:
- Allowing two hours for the MRI exam. In most cases, the
procedure takes 40 to 80 minutes; during that time,
several dozen images may be taken.
- Personal items such as watches, wallets (including credit
cards with magnetic strips that can be erased by the
magnet), and jewelery should be removed prior to the MRI
scan.
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1.9 Treatment
Typically involves improving the patient's quality of life. This is
accomplished through the management of symptoms or
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1.9.1 Prognosis
This depends on the condition itself. Some conditions such as
multiple sclerosis depend on the subtype of the disease and
various attributes of the patient such as age, sex, initial
symptoms and the degree of disability the patient experiences.
Life expectancy in Multiple sclerosis patients is 5 to 10 years
lower than unaffected people. MS is an inflammatory
demyelinating disease of the central nervous system (CNS) that
develops in genetically susceptible individuals after exposure to
unknown environmental trigger(s).The bases for MS are
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CHAPTER 2
2.1 Various Demyelinating disorders of the
Central Nervous System
The demyelinating disorders of the CNS include:
- Myelinoclastic disorders, in which myelin is attacked by
external substances.
- Standard Multiple sclerosis, Devic's disease and other
disorders with immune system involvement called
inflammatory demyelinating diseases.
- Leukodystrophic disorders, in which myelin is not properly
produced:
- CNS Neuropathies like those produced by Vitamin B12
deficiency.
- Central pontine myelinolysis.
- Myelopathies like Tabes dorsalis (syphilitic Myelopathy).
- Leukoencephalopathies like Progressive multifocal
leukoencephalopathy.
- Leukodystrophies.
These disorders are normally associated also with the
conditions Optic neuritis and Transverse myelitis, which are
inflammatory conditions, because inflammation and
demyelination are frequently associated. Some of them are
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idiopatic and for some others the cause has been found, like
some cases of neuromyelitis optica.
CHAPTER 3
3.1 Definition of Multiple Sclerosis
Multiple sclerosis (MS), also known as disseminated
sclerosis or encephalomyelitis disseminata, is an
inflammatory disease in which the insulating covers of nerve
cells in the brain and spinal cord are damaged. This damage
disrupts the ability of parts of the nervous system to
communicate, resulting in a wide range of signs and symptoms,
including physical, mental, and sometimes psychiatric
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3.3 Pathophysiology
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3.4 Epidemiology
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b) Pathogenic
Many pathogens, infectious agents and microbes have been
proposed as triggers of MS, but none have been confirmed.
Evidence for a virus as a cause include: the presence of
oligoclonal bands in the brain and cerebrospinal fluid of most
people with MS, the association of several viruses with human
demyelination encephalomyelitis, and the occurrence of
demyelination in animals caused by some viral infection.
Human herpes viruses are a candidate group of viruses.
Individuals having never been infected by the Epstein-Barr virus
are at a reduced risk of getting MS, whereas those infected as
young adults are at a greater risk than those having had it at a
younger age. Although some consider that this goes against the
hygiene hypothesis, since the non-infected have probably
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From the
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Periventricular
juxtacortical
infratentorial
spinal cord
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18,000 deaths that year. In Africa rates are less than 0.5 per
100,000, while they are 2.8 per 100,000 in South East Asia, 8.3
per 100,000 in the Americas, and 80 per 100,000 in Europe.
Rates surpass 200 per 100,000 in certain populations of
Northern European descent. The number of new cases that
develop per year is about 2.5 per 100,000 (19).
Rates of MS appear to be increasing; this, however, may be
explained simply by better diagnosis. Studies on populational
and geographical patterns have been common and have led to
a number of theories about the cause.
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SPECIAL PART
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CHAPTER 4
An understanding of what is seen in medical imaging of
demyelination, with emphasis on Multiple Sclerosis, is
necessary to be understood, before embarking upon specific
patient files.
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How it works:
Unlike a computed tomography (CT) scan or conventional Xray, MRI does not use radiation. MRI measures the water
content in tissues both normal tissue and abnormal. MRI
uses a powerful magnetic field that:
- Makes the hydrogen protons in water molecules line up in
the direction of the magnetic field.
- Once the hydrogen protons have been lined up, radio
waves are used to knock them out of line.
- When the radio waves are stopped, the protons relax back
into line. As they relax, the protons release resonance
signals that are transmitted to a computer.
Types:
The various types of MRI scans that are used (most commonly
the T1 and T2) measure this relaxation time in different ways.
Computer programs translate these data into cross-sectional
pictures of the water in human tissue.
Because the layer of myelin that protects nerve cell fibers is
fatty, it repels water. In the areas where the myelin has been
damaged by MS, the fat is stripped away. With the fat gone, the
area holds more water, and shows up on an MRI scan as either
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to identify or rule out any other potential causes for the attack
or symptom(s).
The type and number of assessments that may be used in the
diagnosis of a clinically isolated syndrome may vary but the
main test that is used is an MRI scan. An MRI scan will show any
areas of scarring or demyelination in the central nervous
system.
The sites of scarred tissue (areas often referred to as lesions)
vary in clinically isolated syndrome and may determine the
type of symptoms experienced. The areas where this damage is
most frequently seen include:
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CHAPTER 5
5.1 Research Study Subjects
Five (5) patients in the Constanta area, in Romania, were
examined. Two male, ages 35 and 26 (Born 1980 and 1989,
respectively), and three female, ages 47, 43, and 38. (Born
1968, 1972, and 1977, respectively). They were diagnosed with
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multiple sclerosis within the past year and a half, from the time
of this study.
The age range of the patients was on point with the general
age range of multiple sclerosis affected patients (20-50) given
that the youngest of the 5 was 26 (male), and the oldest was
47 (female).
The gender distribution was also in accordance with general
statistics, with slightly more women being affected than men.
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the lesions are not in the aforementioned areas. Thus the MRI
images would look quite different.
All patients examined showed juxtacortical lesions, meaning
lesions adjacent to and touching the cortex.
All 5 patients also showed manifestations of multiple lesions,
adjacent to the ventricles, as well as ovoid lesions,
perpendicular to the ventricles. These ovoid lesions, known as
Dawsons Fingers, represent areas of demyelination along the
small cerebral veins that run perpendicular to the ventricles
(see Fig.9).
There were also multiple lesions on the spinal cords. This is
another typical feature of MS. Typical spinal cord lesions in MS
are relatively small and peripherally located. They are most
often found in the cervical cord and are usually less than 2
vertebral segments in length.
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In the 26 year old male and the 38 year old female, especially
on the T2 axial plane, we could readily observe signs of
multiple enhancing lesions.
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Periventricular
Juxtacortical
Infratentorial
Spinal cord
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CHAPTER 6
CONCLUSION
Getting an MS diagnosis can be a lengthy process. When some
patients finally learn they have the condition after months or
even years of symptoms, for some it might be something of a
relief. For others, it can be shocking. Either way, it is lifechanging. It is important to remember that MS affects
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REFERENCES
1 "Demyelinating Disease" at Dorland's Medical Dictionary
2 Jump up ^ Konopaske GT; Dorph-Petersen KA; Sweet RA
et al. (April 2008). "Effect of chronic antipsychotic exposure
on astrocyte and oligodendrocyte numbers in macaque
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