Motor neuron disease

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Motor neuron disease
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Multiple sclerosis
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Motor neuron disease

• Motor neuron disease is degenerative disease
  which selectively affect motor tract fibers
• (corticospinal tract anterior horn cell)
• UMN signs LMN signs

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Motor pathway

• cortex motor area
• Corticospinal fiber corticobulber
• AHC motor neuron
  disease
• Peripheral nerves
• NMJ
• muscle

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pathology

• Degeneration of the neurons

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path physiology

• Sporadic90 unclear
• Inherted10 familial ALS,25 mutation in gene
  encoding copper zinc super oxide dismutase (SOD1)

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course

• Is progressive median survival is approximately
  3y

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classification

• Classic ALS (amyotrophic lateral
  sclerosis)..UMNLMN signs
• others
• Progressive muscular atrophy
• Primary lateral sclerosis
• Progressive bulbar palsy
• Progressive pseudo bulbar palsy

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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

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• Nooooooooooo
• Cognitive
• Sensory
• Ocular
• Autonomic Sphincter dysfunction

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diagnosis

• El Escorial criteria for dx
• Definitive
• Probable
• possible

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Electrophysiological

• NCS sensory..N
• motornormal or dec amp
• EMG denervation

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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..

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Diagram weakness approach
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Multiple sclerosis
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• MS is the most disabling neurological condition
  of young adults

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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

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pathophysiogy

• Inflamatory rxn causes variable tissue damage
• Destruction of myelin producing cells
  (oligodendrocytes
• Some cells damaged without remyelination but
  oligodendrocytes precursors ..remyelinate..plaque
  

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Risk factors

• Genetic
• Infection viral
• autoimmune

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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 twins30

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Clinical presentation

• Relapsing remitting the commonest
• (gtone attack in gtone site (multifocal)
• Progressive relapsing
• Primary progressive
• Secondary progressive

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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

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• 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
  movementusually 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

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• 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

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• Late stage deficit include dementia, inability
  to stand or walk, slurred speech, ataxic,
  incontinence ,and marked sensory loss in hands
  legs

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• Lehrmit sign
• Athoufs phenomena

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Diagnostic workup

• MRI

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• 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
• Sizegt5mm
• Site adjacent to the lateral ventricles, corpus
  callosum, cerebellum

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• LP modest no of lymphocytes lt50/mm,total protein
  lt.8g/L,elevated immunoglobulin G(IgG), level
  oligoclonal banding on electrophoresis(80)
• Evoked potentials VER,BAR,somatosensory evoked
  potential

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diagnosis

• McDonald criteria
• Confirm lesion gtone site gt one attack

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Diffrential diagnosis

• Clinically
• Multiple infarctions
• Autoimmune diseases
• Vascuilities behcets
• Sarcidosis
• Infection chronic meningitis

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Diseases that cause similar MRI pictures

• Vascular vascuilities,small vesseles
  disease,migraine
• InfectionHIV.Lyme disease
• Granulomtous sarcidosis
• ADEM

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Treatment

• Definitive supportive

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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 tt ltfew years little benefit

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• 4- primary progressive patients can not be
  expected to response to any tt
• 5-multiple sclerosis is a life long disease ,no
  specific time when to discontinue tt once it
  started,if one modality of tt fail or not
  tolerated ,another medication shouled be tried
• 6-patients need to be watched for signs of
  disease activity by clinical or magnetic
  resonance monitring or bothor both.

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Drug used for long term management

• Interferon B(avonex,betaseron,rebif..dec the
  risk of the attacks by 30(sc.IM)
• Side effects
• Depression,flu like,hepatitis
• Copaxon
• Widespread articaria

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Other immunsuppretion
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Drug for acute phase

• Methylpredinsolone 1g iv for 5d
• Side effects

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Supportive care symptomatic)

• Spasticity
• Depression
• Fatigue
• Urinary urgency
• pain

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thanks