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DE MYELIN ATING D I SORDERS

DE MYELIN ATING D I SORDERS. Burcu Ormeci , MD Assistant Professor Department of Neurology. Myelin. It is essential for fast conduction ( saltatory conduction) It is generated by Oligodendrocytes  in the central nervous system Schwann cells  in the p eripheral nervous system.

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DE MYELIN ATING D I SORDERS

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  1. DEMYELINATING DISORDERS BurcuOrmeci, MD AssistantProfessor Department of Neurology

  2. Myelin • It is essential for fast conduction (saltatory conduction) • It is generated by • Oligodendrocytes in the central nervous system • Schwann cells  in the peripheral nervous system

  3. Demyelinating Disorders • Central Nervous System • Multiple sclerosis, progressive multifocal leukoencephalopathy, acute disemminated encephalomyelitis, adrenoleukodystrophy • Peripheral Nervous System • GuillainBarre, CIDP

  4. Myelin Related Disorders • Autoimmune • Multiple sclerosis • Acute disseminated encephalomyelitis (ADEM) • GuillainBarreSyndrome(AIDP) • Chronicinflammatory demyelinating polyneuropathy(CIDP) • Infectious • Progressive multifocal leukoencephalopathy • Metabolic / toxic • CO poisoning • Vitamin B12 deficiency • Mercury poisoning • Miyelinolizis central pontine • Hypoxia • Radiation toxicity • Alcohol / tobacco • Inherited disorders of myelin metabolism • Metachromatic leukodystrophy (MLD) • Adrenoleukodystrophy (ALD) • Phenylketonuria • Vascular • Binswanger's disease

  5. DEMYELINATING DISORDERS • Multipl Sclerosis • Optic Neuritis • NeuromyelitisOptica (Devic’s Disease) • Transverse Myelitis • Schilder'smyelinoclastic diffuse sclerosis • Acute disseminated encephalomyelitis (ADEM)

  6. DEMYELINATING DISORDERS The differential diagnosis of demyelinating disorders • Systemic lupus erythematosus • Sjogren's syndrome • Primary central nervous system vasculitis • Behcet's disease (Neuro-Behcet’s) • Neurosarcoidosis

  7. MULTIPLE SCLEROSIS Definition MS is an auto-immune disease. It is characterized by multifocal demyelination in the white matter of the central nervous system (brainandspinalcord)

  8. MS ClinicalFeatures • About 350,000 peoplehavemultiplesclerosis in the U.S. • Usually, diagnosebetween 20 and 50 years of age • but it mayoccurs in childrenand in theelderly • Most common cause of nontraumatic disability in young adults • Reduction in life expectancy <5-7 years

  9. MS ClinicalFeatures • 10-15% have “Benign” disease • Patients fully functional at 15 years after disease onset • Less than 10% have “malignant” disease • Rapid progression to significant disability or death in a short time

  10. MS GeneticalFeatures • Womenaretwice as likely as men to be affected • Somepopulationsdon’tdevelopmultiplesclerosis • Europeangypsies, EskimosandAfricanBantu • Somepopulationshavea lowincidence • NativeIndians of North and South America, JapaneseandotherAsiangroups • Chance of developingmultiplesclerosis; • Inthe general population has lessthan 1% • If a first-degreerelative has thedisease 1-3 % • In a non-identicaltwin 4% • In an identicaltwin 30%

  11. MS Etiology • Thecause of multiplesclerosis is stillunknown 1.Viral infection and auto-immune reactions 2.Genetic factors: inherited predisposition 3.Environmental factors

  12. Symptomsof MultipleSclerosis • Symptomsof multiplesclerosismay be singleormultiple • Symptomsmayrangefrom • Mildto severe in intensity • Shorttolong in duration • Typicallylastsmorethan 24 hours • Generallymorethan a fewweeks (rarelymorethanfourweeks) • Complete orpartialremissionfromsymptomsoccursearly in about 70%

  13. Symptoms of MultipleSclerosis Visual disturbances (optik nerve) • Opticneuritis • May be thefirstsymptoms of multiplesclerosis, but theyusuallysubside • A personmaynotice • blurredvision, colordesaturation • monocularvisualloss • Visual symptomsduetoopticnerveinflammationusuallyareaccompaniedorprecededbyeyepain

  14. Symptoms of MultipleSclerosis Visual disturbances(brainstem) • Ophthalmoplegia, diplopia, nystagmus • oculomotor nuclei, PPRF, MLF, cerebellum, vestibuler nuclei Sensory dysfunctions • Numbness, prickling, pain • One or more limb, face, trunk • Lermitte sign • an electric shock-like sensation on flexion of the neck

  15. Symptoms of MultipleSclerosis Motor disturbances • Limbweakness • Oneormorelimb • Balance disturbances • Ataxia • Dizziness • Tremors • Muscle spasm, fatigue

  16. Symptoms of MultipleSclerosis Speech andswallowingimpediment • Dysarthria • typicallya problem articulatingwords • Dysphagia

  17. Symptoms of MultipleSclerosis MentalChanges • 50% of peopleexperiencementalchanges • Depression • Decreasedconcentration • Attentiondeficits • Somedegree of memoryloss • İnabilitytoperformsequentialtasks • İmpairment in judgment

  18. Symptoms of MultipleSclerosis UrogenitalDisturbans • Sexualdysfunctionorreducedbowelandbladdercontrol Utoffphenemenon • Heatappearstointensifymultiplesclerosissymptomsforabout 60%

  19. Symptoms of MultipleSclerosis

  20. Types of Multiple Sclerosis • Relapsing-remitting MS (RR-MS) • Primary-progressive MS (PP-MS) • Secondary-progressive MS (SP-MS) • Progressive-relapsing MS (PR-MS)

  21. Relapsing-RemittingRR-MS • About65%-80% of individualsbeginwithRR-MS • Thisis themostcommontype of MS • Itis characterizedbyunpredictableacuteattacks • Theseseries of attacksarefollowedbycompleteorpartialdisappearance of thesymptoms (remission) untilanotherattackoccurs (relapse) • Itmay be weekstodecadesbetweenrelapses

  22. Primary-ProgressivePP-MS • PP-MS is a type of MS characterizedby a gradual but steadyprogression of disability • Therearenoobviousrelapsesandremissions • Thisform of diseaseoccurs in just 15% of allpeoplewithMS • Itis themostcommontype of MS in peoplewhodevelopthediseaseaftertheage of 40

  23. Secondary-ProgressiveSP-MS • Initiallybeginswith a relapsing-remittingcourse, but laterevolvesintoprogressivedisease • Theprogressivepart of thediseasemaybeginshortlyaftertheonset • or • Itmayoccuryearsordecadeslater • About50% of RR-MS individualswilldevelop SP-MS within 10 years

  24. Progressive-RelapsingPR-MS • PR-MS is theleastcommon form of thedisease • It is characterizedby a steadyprogression in disabilitywithacuteattacks • Course of diseasemayormay not be followedbysomerecovery • People withPR-MS initiallyappeartohaveprimaryprogressiveMS

  25. Diagnosis of multiplesclerosis • Multiplesclerosismay not be diagnosedformonthstoyearsaftertheonset of symptoms • Physicians, particularlyneurologists, shouldtakedetailedhistoriesandperformcompletephysicalandneurologicalexaminations

  26. Diagnosis of multiplesclerosis • The demonstration of abnormal physical signs indicating the presence of lesions at two separate sites in the CNS • In an individual with a history of at least two episodes of neurological disturbance of the kind seen in MS • There is no better explanation for the clinical picture These criteria can be fulfilled by clinical assessment alone

  27. Diagnosis of multiplesclerosis • MRI with contrast • Cerebrospinal fluid (CSF) analysis can identify immunoglobulin synthesis • Evoked potentials can demonstrate clinically and even MRI silent lesions

  28. Diagnosisof multiplesclerosis • MRI scanswithintravenousgadoliniumhelpstoidentifyanddescribetheplaques • Plaquesareusuallyroundor oval in shapeand >3mm • Perpendiculartothecorpuscallosum • Sites of involvementarecubcorticalperiventriclewhitematter, corpuscallosum, barainstem, cerebellum, spinalcord

  29. Diagnosisof multiplesclerosis • Collectively, thesetestshelpthephysician in confirmingthediagnosis of multiplesclerosis • Fora definitediagnosis of multiplesclerosis; • Disseminationin time • at leasttwoseparatesymptomaticeventsorchanges on MRI overtime • Dissemination in anatomicalspace • at leasttwoseparatelocationswithinthecentralnervoussystem, which can be demonstratedby MRI orneurologicalexam mustbe demonstrated

  30. CSF • Routine and biological examination cell • normal or slightly high, <15 • Protein • slightly high • IgGindex • >0.7 • Oligoclonalbands of IgG (OB) • Different from blood serum bands

  31. Evoked Potentials • VEP (visual evoked potential) • Prolonged P100 latency • 115 msec < • SEP (somatosensory evoked potential) • Prolonged P40 (lower extremity) and N20 (upper extremity) latencies

  32. Treatment Of Multipl Sclerosis • Improvingthespeed of recoveryfromattacks • treatmentwithsteroiddrugs • Methylprednisolone 1000mg/per days x 5/7/10 days • Oral high dose steroids • Plasma exchange • Intravenous immunoglobulin

  33. Treatment Of Multipl Sclerosis • ReducingTheNumber Of AttacksOrTheNumber Of MRI Lesions • TreatmentWithDiseaseModifyingDrugs • Interferons • Beta Interferon-1a: Avonex, Rebif • Beta Interferon-1b: Betaferon • Glatiramer Acetate • Immunosuppressives -mitoxantrone -cyclophosphamide -azathioprine -methotrexate

  34. Treatment Of Multipl Sclerosis • Relieffromcomplicationsduetotheloss of function of affectedorgans • Treatmentwithdrugsaimed at specificsymptoms • Musclespasticitymusclerelaxant • Fatigue modafinil • Emotionalproblems  antidepressant, neuroleptic • Pain  pain killer, anti-convulsants • Bladderdysfunction  antibiotics, Anticholinergic agents • Sexual dysfunction  sildenafil, papaverin, vaginal gels

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