demyelinating d i sorders burcu ormeci, md assistant professor department of neurology
TRANSCRIPT
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DEMYELINATING DISORDERS
Burcu Ormeci, MDAssistant Professor
Department of Neurology
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Myelin
• It is essential for fast conduction (saltatory conduction)
• It is generated by
Oligodendrocytes in the central nervous system
Schwann cells in the peripheral nervous system
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Demyelinating Disorders
• Central Nervous System• Multiple sclerosis, progressive multifocal
leukoencephalopathy, acute disemminated encephalomyelitis, adrenoleukodystrophy
• Peripheral Nervous System• Guillain Barre, CIDP
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Myelin Related Disorders• Autoimmune• Multiple sclerosis• Acute disseminated encephalomyelitis (ADEM)• Guillain Barre Syndrome(AIDP)• Chronic inflammatory demyelinating
polyneuropathy(CIDP)
• Infectious• Progressive multifocal leukoencephalopathy
• Metabolic / toxic• CO poisoning• Vitamin B12 deficiency• Mercury poisoning• Miyelinolizis central pontine• Hypoxia• Radiation toxicity• Alcohol / tobacco
• Inherited disorders of myelin metabolism• Metachromatic leukodystrophy (MLD)• Adrenoleukodystrophy (ALD)• Phenylketonuria
• Vascular• Binswanger's disease
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DEMYELINATING DISORDERS
• Multipl Sclerosis
• Optic Neuritis
• Neuromyelitis Optica (Devic’s Disease)
• Transverse Myelitis
• Schilder's myelinoclastic diffuse sclerosis
• Acute disseminated encephalomyelitis (ADEM)
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DEMYELINATING DISORDERS
The differential diagnosis of demyelinating disorders• Systemic lupus erythematosus• Sjogren's syndrome• Primary central nervous system vasculitis• Behcet's disease (Neuro-Behcet’s)• Neurosarcoidosis
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MULTIPLE SCLEROSIS
Definition
MS is an auto-immune disease. It is characterized by multifocal demyelination in the white matter of the central nervous system (brain and spinal cord )
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MS Clinical Features
• About 350,000 people have multiple sclerosis in the U.S.
• Usually, diagnose between 20 and 50 years of age• but it may occurs in children and in the elderly
• Most common cause of nontraumatic disability in young adults
• Reduction in life expectancy <5-7 years
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MS Clinical Features
• 10-15% have “Benign” disease• Patients fully functional at 15 years after
disease onset
• Less than 10% have “malignant” disease• Rapid progression to significant disability
or death in a short time
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MS Genetical Features
• Women are twice as likely as men to be affected
• Some populations don’t develop multiple sclerosis• European gypsies, Eskimos and African Bantu
• Some populations have a low incidence• Native Indians of North and South America, Japanese
and other Asian groups
• Chance of developing multiple sclerosis;• In the general population has less than 1% • If a first-degree relative has the disease 1-3 %• In a non-identical twin 4% • In an identical twin 30%
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MS Etiology
• The cause of multiple sclerosis is still unknown
1.Viral infection and auto-immune reactions
2.Genetic factors: inherited predisposition3.Environmental factors
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Symptoms of Multiple Sclerosis
• Symptoms of multiple sclerosis may be single or multiple
• Symptoms may range from • Mild to severe in intensity• Short to long in duration
• Typically lasts more than 24 hours• Generally more than a few weeks (rarely more
than four weeks)
• Complete or partial remission from symptoms occurs early in about 70%
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Symptoms of Multiple Sclerosis
Visual disturbances (optik nerve)
• Optic neuritis
• May be the first symptoms of multiple sclerosis, but they usually subside
• A person may notice• blurred vision, color desaturation• monocular visual loss • Visual symptoms due to optic nerve
inflammation usually are accompanied or preceded by eye pain
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Visual disturbances (brainstem)• Ophthalmoplegia, diplopia, nystagmus • oculomotor nuclei, PPRF, MLF, cerebellum,
vestibuler nuclei
Sensory dysfunctions• Numbness, prickling, pain • One or more limb, face, trunk
• Lermitte sign• an electric shock-like sensation on flexion of
the neck
Symptoms of Multiple Sclerosis
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Symptoms of Multiple Sclerosis
Motor disturbances
• Limb weakness • One or more limb
• Balance disturbances• Ataxia• Dizziness• Tremors
• Muscle spasm, fatigue
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Symptoms of Multiple Sclerosis
Speech and swallowing impediment• Dysarthria• typically a problem articulating words
• Dysphagia
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Symptoms of Multiple Sclerosis
Mental Changes
• 50% of people experience mental changes • Depression• Decreased concentration• Attention deficits• Some degree of memory loss• İnability to perform sequential tasks• İmpairment in judgment
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Symptoms of Multiple Sclerosis
Urogenital Disturbans• Sexual dysfunction or reduced bowel and
bladder control
Utoff phenemenon• Heat appears to intensify multiple
sclerosis symptoms for about 60%
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Symptoms of Multiple Sclerosis
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Types of Multiple Sclerosis
• Relapsing-remitting MS (RR-MS)
• Primary-progressive MS (PP-MS)
• Secondary-progressive MS (SP-MS)
• Progressive-relapsing MS (PR-MS)
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Relapsing-RemittingRR-MS
• About 65%-80% of individuals begin with RR-MS
• This is the most common type of MS
• It is characterized by unpredictable acute attacks
• These series of attacks are followed by complete or partial disappearance of the symptoms (remission) until another attack occurs (relapse)
• It may be weeks to decades between relapses
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Primary-Progressive PP-MS
• PP-MS is a type of MS characterized by a gradual but steady progression of disability
• There are no obvious relapses and remissions
• This form of disease occurs in just 15% of all people with MS
• It is the most common type of MS in people who develop the disease after the age of 40
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Secondary-Progressive
SP-MS• Initially begins with a relapsing-remitting
course, but later evolves into progressive disease• The progressive part of the disease may begin
shortly after the onset• or
• It may occur years or decades later
• About 50% of RR-MS individuals will develop SP-MS within 10 years
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Progressive-Relapsing
PR-MS• PR-MS is the least common form of the
disease
• It is characterized by a steady progression in disability with acute attacks
• Course of disease may or may not be followed by some recovery
• People with PR-MS initially appear to have primary progressive MS
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Diagnosis of multiple sclerosis
• Multiple sclerosis may not be diagnosed for months to years after the onset of symptoms
• Physicians, particularly neurologists, should take detailed histories and perform complete physical and neurological examinations
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Diagnosis of multiple sclerosis
• The demonstration of abnormal physical signs indicating the presence of lesions at two separate sites in the CNS
• In an individual with a history of at least two episodes of neurological disturbance of the kind seen in MS
• There is no better explanation for the clinical picture
These criteria can be fulfilled by clinical assessment alone
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Diagnosis of multiple sclerosis
• MRI with contrast
• Cerebrospinal fluid (CSF) analysis can identify immunoglobulin synthesis
• Evoked potentials can demonstrate clinically and even MRI silent lesions
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Diagnosis of multiple sclerosis
• MRI scans with intravenous gadolinium helps to identify and describe the plaques
• Plaques are usually round or oval in shape and >3mm
• Perpendicular to the corpus callosum
• Sites of involvement are cubcortical periventricle white matter, corpus callosum, barainstem, cerebellum, spinal cord
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Diagnosis of multiple sclerosis
• Collectively, these tests help the physician in confirming the diagnosis of multiple sclerosis
• For a definite diagnosis of multiple sclerosis;• Dissemination in time
• at least two separate symptomatic events or changes on MRI over time
• Dissemination in anatomical space• at least two separate locations within the
central nervous system, which can be demonstrated by MRI or neurological exam
must be demonstrated
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CSF
• Routine and biological examination cell• normal or slightly high, <15
• Protein• slightly high
• IgG index• >0.7
• Oligoclonal bands of IgG (OB)• Different from blood serum bands
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Evoked Potentials
• VEP (visual evoked potential)• Prolonged P100 latency• 115 msec <
• SEP (somatosensory evoked potential)• Prolonged P40 (lower extremity) and N20
(upper extremity) latencies
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Treatment Of Multipl Sclerosis
• Improving the speed of recovery from attacks• treatment with steroid drugs• Methylprednisolone 1000mg/per days x 5/7/10
days• Oral high dose steroids• Plasma exchange• Intravenous immunoglobulin
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Treatment Of Multipl Sclerosis
• Reducing The Number Of Attacks Or The Number Of MRI Lesions• Treatment With Disease Modifying Drugs• Interferons• Beta Interferon-1a: Avonex, Rebif• Beta Interferon-1b: Betaferon
• Glatiramer Acetate• Immunosuppressives
-mitoxantrone
-cyclophosphamide
-azathioprine
-methotrexate
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Treatment Of Multipl Sclerosis
• Relief from complications due to the loss of function of affected organs• Treatment with drugs aimed at specific
symptoms• Muscle spasticity muscle relaxant
• Fatigue modafinil
• Emotional problems antidepressant, neuroleptic
• Pain pain killer, anti-convulsants
• Bladder dysfunction antibiotics, Anticholinergic agents
• Sexual dysfunction sildenafil, papaverin, vaginal gels