1 / 37

Motor neuron disease

Motor neuron disease. Multiple sclerosis. Motor neuron disease is degenerative disease which selectively affect motor tract fibers (corticospinal tract+ anterior horn cell) UMN signs LMN signs. Motor neuron disease. Motor pathway. cortex motor area

idola
Download Presentation

Motor neuron disease

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  1. Motor neuron disease

  2. Multiple sclerosis

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

  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. 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 decreased amplitude • 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…..

  14. Multiple sclerosis • MS is the most disabling neurological condition of young adults

  15. 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

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

  17. Risk factors • Genetic • Infection :viral • autoimmune

  18. 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%

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

  20. 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

  21. 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

  22. 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

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

  24. Lehrmit sign • Athoufs phenomena

  25. Diagnostic workup • MRI

  26. 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

  27. 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

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

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

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

  31. Treatment: • Definitive supportive

  32. 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

  33. 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.

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

  35. 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

  36. Supportive care symptomatic • Spasticity • Depression • Fatigue • Urinary urgency • pain

  37. thanks

More Related