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Demylinating Diseases - Multiple Sclerosis.pdf
- 1. Demyelinating Diseases By Mohammed GamalAbdellatif Lecturer of Neurology Sohag Faculty of Medicine
- 2. Diseases of Myelin Autoimmune •Multiple sclerosis Acute disseminated encephalomyelitis Toxic/Metabolic • Central pontine myelinolysis • Radiation Hereditary Disorders of Myelin Metabolism Dysmelination • Adrenoleukodystrophy • Metachromatic leukodystrophy
- 8. 1.Multiple Sclerosis • Definition: Inflammatoryimmune-mediated demyelinating disease of CNS, destroying myelin and axon in variable degrees has relapses and remissions.
- 9. Epidemiology • Age ofonset : 29–32. • Sex Distribution women more than men. • Geographical Distribution High-frequency areas of the world include all of Europe the United States. • Worldwide, approximately 2.1 million people are affected by MS. • Mortality MS shortens life span, on average, by a period of 7–14 years
- 10. •Etiology: 1.Autoimmunity : Antigenicsimilarity 2.Genetic susceptibility : HLADRB1*1501 allele 3.Infection : Epstein-Barr virus (EBV) 4. Vitamin D deficiency 5.Smoking
- 11. Pathology Changes in AxonalConduction with Demyelination Pathological hallmark is formation of MS Plaque.
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- 19. Clinical phenotypes 1.Relapsing remittingMS: Clearly defined relapses with full recovery or with sequelae and residual deficit on recovery. 2. Secondary progressive MS: Initial relapsing-remitting disease course followed by progression with or without occasional relapses 3. Primary progressive MS: Disease progression from onset
- 20. Diagnosis and Investigations •The Modified McDonald Criteria 2017 for dissemination in time and space •
- 22. Differential Diagnosis • Systemiclupus erythematosus • Sjögren disease • Behçet disease • Acute disseminated encephalomyelitis • Neuromyelitis optica spectrum disorder • CNS HIV infection • Sarcoidosis • Vitamin B12 deficiency
- 23. FACTORS INFLUENCING CLINICALCOURSE • Sex : Men • Age at onset : Late • Initial disease course : progressive • Initial manifestations : pyramidal brainstem cerebellar symptoms
- 24. Treatment 1. Acute attacktreatment High dose corticosteroids Methyl prednisolone 1gm intravenous infusion per day for 3-5 days 2. Disease Modifying therapy prevention of attacks and disability progression Interferon Beta, Fingolimod, Terflonamide, Natalizumab, Ocreluzumab 3.Symptomatic treatment fatigue bladder motor and pain symptoms 4.Physiotherapy spasticity
- 25. 2.ADEM Acute disseminated Encephalomyelitis •immune-mediated inflammatory demyelinating condition affects white matter of the brain and spinal cord. • acute-onset encephalopathy associated with polyfocal neurologic deficits • febrile prodromal illness or immunizatioassociated encephalopathy of varied degrees.
- 26. Pathophysiology • Autoimmune responsemediated by stimulated clones of T-helper cells sensitized to auto antigens such as myelin proteins ➢ANTIGINIC SIMILARITY ➢Disturbance of the blood-brain barrier •Epidemiology • incidence rate of 0.07 per 100,000 • Risks Genetic factors, prevalence of infectious pathogens, immunization status • 10% mortality rate • Morbidity visual, motor, autonomic, and behavioral/intellectual deficits and epilepsy.
- 27. Clinical presentation • Historyof preceding infectious illness (respiratory or gastrointestinal) or immunization. • Association with constitutional symptoms and signs, such as fever • Cortical signs such as encephalopathy and seizures • Long tract signs (clonus, increased muscle stretch reflexes, upgoing toes) • MRI diffuse, poorly demarcated large lesions involving the cerebral white matter
- 28. Clinical presentation • Irritabilityand lethargy Fever Meningismus • Multifocal neurologic abnormalities • Acute hemiparesis, cranial nerve abnormalities (including visual loss), ataxia • Mental status disturbances include lethargy, fatigue, confusion, irritability and coma. • Focal or generalized seizures occur as an early sign in a minority of cases.
- 29. Differential Diagnosis • MultipleSclerosis • Bacterial meningitis • Brain Metastasis • Primary CNS Lymphoma • Cardioembolic Stroke • CNS Complications in HIV • Cerebral Venous Thrombosis • Herpes Simplex Encephalitis
- 31. Investigations • CSF Modest-to-moderateelevation white and red blood cell counts • Lesions multiple, large sized bilateral asymmetric widespread areas od demyelination enhance with gadolinium
- 32. Treatment • high-dose intravenouscorticosteroids, methylprednisolone of 1 g/day for 3-5 days. • Alternative therapy is intravenous immune globulin (IVIG)
- 33. 3.Neurmyelitis Optica Spectrum Disorders •Autoimmune diseases characterized by acute inflammation of the optic nerve (optic neuritis) and the spinal cord (myelitis). • Episodes of optic neuritis and myelitis can be simultaneous or successive. • NMO is caused by immunoglobulin G autoantibodies to aquaporin 4 (anti-AQP4), the most abundant water channel protein in the CNS. • Prevalence 0.5 to 10 cases per 100,000 people
- 34. Clinical picture • Myelitis,result in muscle limbs weakness, lost or reduced sensation, loss of bladder and bowel control. • Myelitis can be transverse, affecting entire cross-section of the spinal cord. • ON results in severe visual loss at onset, with bilateral involvement, and permanent visual deficits • Lesions in the area postrema of the medulla oblongata can cause vomiting or hiccups • Positive plasma autoantibodies to aquaporin 4 (anti-AQP4)
- 35. Differential Diagnosis • MS •ADEM • SLE, Sjogren, Behcet diseases
- 36. Treatment • Acute attacks 1.Shortcourses (3–5 days) of high dosage intravenous corticosteroids, methylprednisolone IV 2.Plasmapheresis can be an effective treatment when attacks progress • Secondary prevention 1.Eculizumab Monoclonal antibody against complement protein C5 2.Satralizumab Monoclonal antibody against IL-6
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