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Motor neuron disease
Multiple sclerosis
Motor neuron disease
Motor neuron disease is
degenerative disease which
selectively affect motor tract fibers
(corticospinal tract+ anterior horn cell)
UMN signs
LMN signs
Motor pathway
cortex motor area
Corticospinal fiber & corticobulber
AHC
Peripheral nerves
NMJ
muscle
motor neuron disease
pathology
Degeneration of the neurons
path physiology
Sporadic:90% unclear
Inherted:10% familial ALS,25%
mutation in gene encoding copper
zinc super oxide dismutase (SOD1)
course
Is progressive : median survival is
approximately 3y
classification
Classic ALS (amyotrophic
lateral sclerosis)..UMN+LMN
signs
others
Progressive muscular atrophy
Primary lateral sclerosis
Progressive bulbar palsy
Progressive pseudo bulbar palsy
Classic ALS
Mixed upper motor neuron + upper
motor neuron signs
Early patient may exhibit only LMN
signs or upper LMN signs
Weakness begin a symmetrical and
distally then spread to involve
contiguous group of motor neurons
Bulbar &pesudobulber palsy
involvement ..dysphagea & dysarthria
Nooooooooooo
Cognitive
Sensory
Ocular
Autonomic Sphincter dysfunction
diagnosis
El Escorial criteria for dx
Definitive
Probable
possible
Electrophysiological
NCS: sensory..N
motor:normal or decreased
amplitude
EMG: denervation
treatment
Riluzole :50 mg bid ( extend
tracheotomy free survival by 2-3
months, not improving the survival or
muscle strength
Supportive care physiotherapy,
respiratory, swallowing…..
Multiple sclerosis
MS is the most disabling neurological
condition of young adults
Epidemiology
Onset is typically in the mid 20s,although
the dx may be delayed for several years
The ratio of f to m 1.77 to 1
The incidence of MS in blacks residing in
the united states is about 25% that of
whites
High incidence includes all of
Europe,North america,New
Zealand,southern austeralia but the
incidence also increasing in middle east
pathophysiogy
Inflamatory rxn causes variable
tissue damage
Destruction of myelin producing cells
(oligodendrocytes)
Some cells damaged without
remyelination but oligodendrocytes
precursors ..remyelinate..plaque
Risk factors
Genetic
Infection :viral
autoimmune
genetic
In general in the united states, the
prevalence of MS is about ,1%
If a mother has MS,, her children's
have a chance 3-5% .
If father has MS, his son has a1%
chance & his daughter a 2% chance
Non identical twins has 3-4%
Identical twins:30%
Clinical presentation
Relapsing remitting: the commonest
(>one attack in >one site (multifocal)
Progressive relapsing
Primary progressive
Secondary progressive
diagnosis
Clinical :typical relapses come on over a
few days, lasts for weeks or months ,and
then clear, over 80% of patients begin with
relapses
All central nervous system can be affected
Typical relapses
A-optic neuritis
B-myelopathy(spinal cord)
C-brain stem &cerebellar
Optic neuritis: clouding or blurring of
central vision in one eye
loss of measured activity, impair pupillary
light reflex, some local pain made worse by
eye movement…usually full recovery
Myelopathy: often sensory only; numbness
&tingling from a certain level on the trunk
on down through the rest of the body. if
marked ..weakness
Brain stem
Each of these relapses may leave some
residual
After several attacks of various types, a
patient may present common deficit
Mild reduction in vision in one eye
No conjugate eye movements
Extensor planter responses &inability to
walk heel and toe
Reduced vibration sense in the legs
Urgency of bladder function
Late stage deficit include: dementia,
inability to stand or walk, slurred
speech, ataxic, incontinence ,and
marked sensory loss in hands &legs
Lehrmit sign
Athoufs phenomena
Diagnostic workup
MRI
Mri is now the dominant laboratory method
of diagnosis in MS
MS lesions are usually easily detected and
often characteristic…
Multiple bright lesion in T2
Contrast enhanced lesion
Shape :ovoid
Size:>5mm
Site: adjacent to the lateral ventricles,
corpus callosum, cerebellum
LP: modest no of lymphocytes
<50/mm,total protein <.8g/L,elevated
immunoglobulin G(IgG), level
oligoclonal banding on
electrophoresis(80%)
Evoked potentials:
VER,BAR,somatosensory evoked
potential
diagnosis
McDonald criteria:
Confirm lesion >one site +> one
attack
Diffrential diagnosis
Clinically:
Multiple infarctions
Autoimmune diseases
Vascuilities: behcets
Sarcidosis
Infection: chronic meningitis
Diseases that cause similar MRI
pictures
Vascular: vascuilities,small vesseles
disease,migraine
Infection:HIV.Lyme disease
Granulomtous :sarcidosis
ADEM
Treatment:
Definitive
supportive
definitive
Six principles of management in multiple
sclerosis
1-relapses with significant impairment of
function should be treated with high dose
IV corticosteroid
2-All relapsing remitting patients should be
receiving long term immunomodulatory
treatments
3-Secondary progressive need aggressive
tt early,late treatment <few years little
benefit
4- primary progressive patients can not be
expected to response to any treatment
5-multiple sclerosis is a life long disease
,no specific time when to discontinue
treatment once it started, if one modality of
treatment fail or not tolerated ,another
medication should be tried
6-patients need to be watched for signs of
disease activity by clinical or magnetic
resonance monitoring or both.
Drug for acute phase
Methylpredinsolone 1g iv for 5d
Side effects:
Drug used for long term
management
Interferon –B(avonex,betaseron,rebif..
decrease the risk of the attacks by
30%(sc.IM)
Side effects:
Depression, flu like, hepatitis
Copaxon: Widespread articaria
Supportive care symptomatic
Spasticity
Depression
Fatigue
Urinary urgency
pain