Multiple Sclerosis

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Multiple Sclerosis ICD-9M Code: 340: Multiple Sclerosis 1. Definition A. A Chronic Autoimmune Demyelinating Disease Of The CNS. B. Characterized By Clinical Attacks Correlated With Lesions Of The CNS Separated In Time & Space. C. Subtypes: 1. Relapsing Remitting MS (RRMS) 2. Secondary Progressive MS (SPMS) (Progression With Disability With Few Or No Relapses) 3. Rare Variants: A. Balo’s Concentric Sclerosis 1. Neuroimaging & Pathology : Alternating Rings of Myelination & Demyelination B. Marburg’s Disease: 1. Neuroimaging: Tumorlike Lesions With Significant Edema 2. Pathology: Alternating Rings Of Myelination & Demyelination C. Schilder’s Diffuse Sclerosis: 1. Childhood Onset 2. 1-2 Large Assymmetric Lesions D. Neuromyelitis Optica: Devic’s Disease: 1. Recurrent Relapses Involving Only the Optic Nerve & Spinal Cord

2. Epidemiology: A. Is The Most Common Demyelinating Disease Of The CNS B. Prevalence: 1. Higher In Northern Latitudes 2. Prevalence Per 10 Varies From: 30-70: Parts of Italy &Spain 100-200: Scandinavia 100: USA 16-30: Middle Eastern Arabs < 5-10: Asia, Central America, Africa C. Sex & Age: Female/Male Ratio: 1.5/1 Young Adults Mean Age Of Onset: 1. 30 Years Of Age: RRMS 2. 40 Years Of Age: SPMS 3. 37 Years Of Age: PPMS D. Subtypes: RRMS: 58% SPMS: 27% PPMS: 15% E. Genetics: 1. 3-5% Frequency In Dizygotic Twins & Siblings 3-5% 2. 20-40% Frequency In Monozygotic Twins 3. HLA Clusters: Classes I &II: ( DRB1-1501, DQA1-0102,DQB1-0602, T Cell Receptor Beta, CTLA4, ICAMA-1, & SH2D2A)

3. Etiology: A. Thought To Be An Interaction Between: 1. Multiple Genes Influencing The Immune System 2. Enviornmental Factors A. Possible Viral Vectors B. Vitamin D C. Sun Exposure 4. Pathogenesis: A. There Is A Significant Body of Indirect Evidence That It Is An Autoimmune Disease Involving T Cell Injury To The Myelin Sheaths And/Or Oligodendroglial Cells B. Both CD4 & CD8 Cells Are Present Within The Lesions & Many Are Reactive Against Myelin Basic Protein. C. A Disorder Bearing Some Resemblance To Human MS Termed Chronic Experimental Allergic Encephalomyelitis Can Be Induced In Mice By Sensitizing Them To the Components Of Myelin & Can Be Transfered Into Naïve Animals By Sensitized T Cells. D. Antibodies Against Myelin Oligodendrocyte Protein & Myelin Basic Protein Along With Activated Complement May Be Present In Lesions Undergoing Demyelination. E. Current Evidence Suggests Both Environmental (Infectious ?) & Genetic Factors May Play A Role In The Disease F. A Genetic Component Is Suggested By The Increased Risk Of MS In Association With HLA-DR2 Genes & A Higher Rate Of Concordance In Identical Twins Than Dizygotic Twins. 4. Diagnosis: A. MS: Clinical Diagnosis Primarily With Evidence Of CNS Demyelinating Lesions Disseminated In Time & Space. 1. RRMS: A. History Of 2 Relapses B. Confirmation On Neurologic Examination C. At Least 2 Demyelinating Lesions With Separation In Time & Space

2. PPMS: A. Insidious Progression Of Disability With Positive CSF Findings B. MRI: 1. 9 T2 Lesions In Brain Or 2. 2 Lesions In Spinal Cord Or 3. 4-8 Brain Lesions + 1 Spinal Cord Lesion Or 4. Visual Evoked Potentials & 4-8 Brain Lesions + 1 Spinal Cord Lesion 5. MRI Dissemination In Time As Continued Progression For 1 Year 5. Clinical Features: A. Common Manifestations Include: 1. Fatigue 2. Diplopia 3. Hemiparesis 4. Paraparesis 5. Numbness 6. Paresthesias 7. Ataxia 8. Cognitive Dysfunction 2. Visual Disturbances (Blurred Vision) A. Diplopia B. Scotomata C. Paresthesias D. Marcus Gunn Pupil E. Internuclear Opthalmoplegia: ( Paresis Of Adducting Eye On Conjugate Lateral Gaze With Hiorizontal Nystagmus Of The Abducting Eye)

3. UMN Signs: A. Spasticity B. Increased DTRs C. Extensor Plantar Response D. UMN Pattern of Weakness: 1. Shoulder Abduction 2. Elbow, Hand & Finger Extension 3. HipAnd Knee Flexion 4. Foot Dorsiflexion 4. Sensory Loss: A. Pain/Temperature B. Vibration/ Position Sense 7. Ataxia: A. Intentionr Tremor B. Heel To Shin Ataxia C. Difficulty With Tandem Gait 5. Bladder Dysfunction: A. Detrusor Hyperreflexia (Urge Incontinence) B. Flaccidity (Neurogenic Bladder) C. Dyssynergia (Bladder Contracts Against A Closed Sphincter) 6. Lhermitte’s Sign: Flexion of the Neck Elicits An Electrical Sensation Down The Spine) 7. Uthoff’s Phenomenon: Exercise Or Heat Induced Deterioration of Function 8. Emotional Disturbances: A. Depression, B. Mood Lability.

4. WorkUp: A. Lumbar Puncture: 1. CSF Abnormalities A. Increased Protein B. Increased WBCs C. Increased IgG Index: 70% D. Oligoclonal IgG Bands: 90% E. False Positive Rarely In Oligoclonal Bands 2. Serum Work Up: A. CBC B. ESR C. Metabolic Profile D. LFTs E. ANA F. B12 3. Consider: A. Lyme Titer B. TFTs C. B12 Levels D. Collagen Vascular Serologies E. ANA F. Very Long Chain Fatty Acids 4. Consider Evoked Potentials: A. VEP, SSEP, BAER B. Demyelination Will Show Slow Conduction Velocities 5. Imaging Techniques: A. MRI Has Been Found To Be An Effective Tool When Used In Conjunction With Clinical & Ancillary CSF Studies. 6. Course of the Disease: A. Some Patients Present With Rapid Fulminating, Progressive Disease & Die Within Weeks To Months Of Onset. B. Others Experience A Normal Life Span C. Others Have Multiple Exacerbations & Remissions With Cumulative Neurologic Deficits Over The Course Of Several Years To Decades

6 Differential Diagnosis: A. Autoimmune: 1. Acute Disseminated Encephalomyelitis 2. Postvaccination Encephalomyelitis B. Degenerative: 1. Subacute Combined Degeneration Of The Spinal Cord (Vitamin B12 Deficiency) 2. Inheritied Spastic Paraparesis C. Infections: 1. PML 2. Lyme Disease 3. Syphilis 4. HIV 5. HTLV-1 6. Whipple Disease D. Inflammatory: 1. SLE 2. Sjogren’s Syndrome 3. Behcet’s Vascultis 4. Sarcoidosis 5. Celiac Disease E. Inheritied Metabolic Disorders 1. Leukodystrophies F. Mitochondrial Diseases 1. Leber’s Herdiary Optic Neuropathy 2. Mitochondrial Encephalopathy 3. Lactic Acidosis 4. Strokes

7. Treatment: For: Relapsing Remitting Form: A. Methylprednisolone B. Interferon Beta 1a (Avonex) C. Interferon Beta 1b (Betaseron) D. Glatiramer Acetate (Copaxone): Conflicting Evidence E. Mitoxantrone & Methotrexate: Potential Toxicities May Outweigh Clinical Benefit For Secondary Progressive From: Consider: A. Cyclophosphamide B. Mitoxantrone Chronic Treatment: 1. Fatigue: A. Aspirin Alternatives For Fatigue: A. Amantadine B. Methylphenidate C. Mixed Amphetamine Salts D. Modafinil E. Energizing Antidepressants: 1. Bupropion 2. Fluoxetine 3. Venlafaxine F. Speculative: 1. Acetyl- Carnitine 2. 4 Aminopyridine Extended Release 2. Spasticity: A. Baclofen B. Diazepam C. Tizanidine D. Lorazepam E. Intrathecal Baclofen

3. Pain: A. Carbamezapine B. Gabapentin C. Amitriptyline 4. Depression: A. Anti-Depressants 5. Spastic Bladder: A. Oxybutynin B. Propantheline C. Tolterodine 6. Tremor: A. Clonazepam B. Carbamazapine C. Propanolol

Rx: Investigational Agents: 1. Tysabri: Natalizumab: SE: Cases of PML 2. Leukocyte Depletion: Campath: Alemtuzumab A. CD 52 Antibody Against The CD52 Antigen On Lymphocyte Cladribine: Purine Nucleoside Derivative A. Against CD4 & CD8 Cells B. Influences Rate Of Clinical Relapse C. Role: May Minimize Disability Resulting From Acute Inflammation D. Role: May Play Future Role As Agent Targeting Inflammatory Component Of MS 3. HMG-CoA Reductase Inhibitors : Statins A. Atorvastatin: Enhances Th-2 Cell 4. Teriflunomide (Novartis Pharmaceutical) A. Broad Effect On Lymphocyte Biology 5. Orencia: Abatacept A. Clinical Phase 2 Trials In RR-MS 6. Rapamycin : Sirolimus A. Blocks Interleukin 2 Activation 7. Acetylcholinesterase Inhibitors: A. May Have Modest Effect On Neurodegeneration 8. Riluzole: A. Inhibits Glutamate and Aspartate B. Affects Glutamate Receptors & Sodium Channels C. Phase 2 Trials

9. Lymphocyte Sequestration: FTY720: A. Agonist Of Sphingosine-1-Phosphate Receptors B. Reduced Symptoms In Experimental Autoimmune Encephalitis : Murine Model Of Inflammatory Demyelination C. Shows Promise As Part Of Immunomodulatory Cocktail 10. Fingolimod: For Relapsing MS: ClinicalTrials 11. BG000012 ( Fumarate, Furmaric Acid Ester) 12. Laquinimod (Teva ) 13. Zenapax (Biopharma) 14. Neurovax (Orchestra Therapeutics) 15. Tovaxin (Opexa) 16. Fampridine (Acorda) 17. MBP8298 (BioMS) 18. Estriol 19. Rituxan (Genentech & Biogen Pharmaceuticals)

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