Demyelinating disease

Introduction
• Myelin sheaths cover many nerve fibers in the central
and peripheral nervous system; they accelerate axonal
transmission of neural impulses.
• Myelin formed by oligodendroglia in the CNS and by
Schwann cells peripherally.
 Demyelinating disease
• A demyelinating disease is any condition that results
in damage to the myelin sheath.
• When the myelin sheath is damaged, nerve impulses
slow or even stop, causing neurological problems.
• The reduction in conduction ability causes deficiency
in sensation, movement, cognition, or other functions
depending on which nerves are involved.
• De myelination tends to be segmental or patchy,
affecting multiple areas simultaneously or
sequentially.
• Re myelination often occurs, with repair, regeneration,
and complete recovery of neural function.
• However, extensive myelin loss is usually followed by
axonal degeneration and often cell body
degeneration; both may be irreversible.
 Classification
• Can be divided on basis of the cause
1.Demyelinating myelinoclastic diseases –
Secondary: myelin is destroyed by a toxic (eg, alcohol,
ethambutol), infectious agents, chemical or autoimmune
substance.
2. Demyelinating leukodystrophic diseases –
Primary: myelin is abnormal and degenerates; caused by
genetics, and some by unknown factors. •

Can be divided by other criteria in inflammatory and

noninflammatory, according to the presence or lack of
inflammation.
• Can be divided into 2 groups of diseases presenting
different demyelination conditions .
1. Affecting the CNS
2. Affecting the peripheral nerves.
 Demyelinating disorders of the CNS
Myelinoclastic disorders, in which myelin is attacked
by external substances
• Standard Multiple sclerosis,
• Devic's disease
• Other disorders with immune system involvement
called inflammatory demyelinating diseases.
Leukodystrophic disorders, in which myelin is not
properly produced:
CNS Neuropathies like Vitamin B12 def
Central pontine myelinolysis.
Tabes dorsalis (syphilitic Myelopathy)
Progressive multifocal leukoencephalopathy
• 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 idiopathic and for some others the
cause has been found, like some cases of
neuromyelitis optica.
 Demyelinating diseases of the PNS
• Guillain-Barré syndrome and its chronic counterpart,
chronic inflammatory demyelinating polyneuropathy
• Anti-MAG peripheral neuropathy
• Charcot–Marie–Tooth disease
• Copper deficiency associated conditions (peripheral
neuropathy, myelopathy, and rarely optic neuropathy)

• Progressive inflammatory neuropathy

 Multiple sclerosis
• Multiple sclerosis (MS) is the most common
demyelinating and autoimmune disease of the central
nervous system.
• In this disorder, underlying mechanism is thought to
be either destruction by the immune system or failure
of the myelin-producing cells.
• The cause of MS is unknown; however, it is believed
to occur as a result of some combination of genetic
and environmental factors such as infectious agents.
• This causes inflammation and injury to the sheath
and ultimately to the nerve fibers that it surrounds
and may result in multiple areas of scarring
(sclerosis).
• The three main characteristics of MS are the
formation of lesions in the CNS (plaques),
inflammation, and the destruction of myelin sheaths
of neurons.
• These lesions most commonly affect the white matter
in the optic nerve, brain stem, basal ganglia, and
spinal cord, or white matter tracts close to the lateral
ventricles.
• This damage disrupts the ability of parts of the
nervous system to communicate, resulting in a range
of signs and symptoms, including physical, mental,
and sometimes psychiatric problems.
 Guillain–Barré Syndrome(GBS)
• Is a rapid-onset muscle weakness caused by the
immune system damaging the peripheral nervous
system.
• Two thirds of people with Guillain–Barré syndrome
have experienced an infection before the onset of the
condition.
• Most commonly these are episodes of gastroenteritis
or a respiratory tract infection.
• Approximately 30% of cases are provoked by
Campylobacter jejuni bacteria, which cause diarrhea.
• A further 10% are attributable to cytomegalovirus
 Other types of demyelinating disease
• Optic neuritis — inflammation of the optic nerve in
one or both eyes, occurs preferentially in females
typically between the ages of 30 and 35.
• Neuromyelitis optica (Devic’s disease) — inflammation
and demyelination of the central nervous system,
especially of the optic nerve and spinal cord
• Transverse myelitis — inflammation of the spinal cord
• Acute disseminated encephalomyelitis —
inflammation of the brain and spinal cord
• Adrenoleukodystrophy and adrenomyeloneuropathy
— rare, inherited metabolic disorders.
• MS and others most commonly result in vision loss,
muscle weakness, muscle stiffness, loss of
coordination, loss of sensation, pain, and changes in
bladder and bowel function.