1. Motor neuron disease

2. Multiple sclerosis

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

4. Motor pathway cortex motor area Corticospinal fiber & corticobulber
AHC motor neuron disease
Peripheral nerves
NMJ
muscle

5. pathology
Degeneration of the neurons

6. path physiology Sporadic:90% unclear
Inherted:10% familial ALS,25% mutation in gene encoding copper zinc super oxide dismutase (SOD1)

7. course
Is progressive : median survival is approximately 3y

8. Classic ALS (amyotrophic lateral sclerosis)..UMN+LMN signs
classification
Classic ALS (amyotrophic lateral sclerosis)..UMN+LMN signs
others
Progressive muscular atrophy
Primary lateral sclerosis
Progressive bulbar palsy
Progressive pseudo bulbar palsy

9. 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

10. Nooooooooooo
Cognitive
Sensory
Ocular
Autonomic Sphincter dysfunction

11. diagnosis
El Escorial criteria for dx
Definitive
Probable
possible

12. Electrophysiological
NCS: sensory..N
motor:normal or dec amp
EMG: denervation

13. 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…..

15. Diagram weakness approach

16. Multiple sclerosis

17. MS is the most disabling neurological condition of young adults

18. 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

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

20. Risk factors
Genetic
Infection :viral
autoimmune

21. 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%

22. Clinical presentation
Relapsing remitting: the commonest
(>one attack in >one site (multifocal)
Progressive relapsing
Primary progressive
Secondary progressive

23. 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

24. 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

25. 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

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

27. Lehrmit sign
Athoufs phenomena

28. Diagnostic workup
MRI

29. 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

30. LP: modest no of lymphocytes <50/mm,total protein <
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

31. diagnosis McDonald criteria:
Confirm lesion >one site +> one attack

32. Diffrential diagnosis
Clinically:
Multiple infarctions
Autoimmune diseases
Vascuilities: behcets
Sarcidosis
Infection: chronic meningitis

33. Diseases that cause similar MRI pictures
Vascular: vascuilities,small vesseles disease,migraine
Infection:HIV.Lyme disease
Granulomtous :sarcidosis
ADEM

34. Treatment:
Definitive supportive

35. 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 <few years little benefit

36. 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.

37. 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

38. Other immunsuppretion

39. Drug for acute phase
Methylpredinsolone 1g iv for 5d
Side effects:

40. Supportive care symptomatic)
Spasticity
Depression
Fatigue
Urinary urgency
pain

41. thanks