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Understanding Demyelinating Disorders: Multiple Sclerosis Overview

Explore the complexities of demyelinating disorders, focusing on Multiple Sclerosis (MS). Learn about the clinical features, genetic factors, symptoms, and types of MS, along with related disorders and differential diagnoses.

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Understanding Demyelinating Disorders: Multiple Sclerosis Overview

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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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