Cidp ajay
- 1. Chronic Inflammatory Demyelinating Polyradiculoneuropathy Dr. Ajay Kumar Agarwalla Resident, Phase- B Neurology 11/30/2017 1
- 2. Epidemiology Incidence 1-2/lac All ages(peak 5th -6th decades) Males > Females 13-20% of all initially undiagnosed neuropathy 11/30/2017 2
- 3. GBS CIDPSpecificforGBS • Preceding infection obvious • Monophasic or Relapsing • Doesn’t respond to Steroid • No nerve thickening • Cranial nerve usual Similarities • CSF- AC dissociation • Histopathology- Multifocal inflammatory demyelination • NCS SpecificforCIDP • Preceding infection less than 10% • Chronic progressive & Relapse- Remitting • Good response to steroid • Nerve thickening may found • Cranial nerve rarely 11/30/2017 3
- 4. Temporal pattern Common in DM Relapse during pregnancy and postpartum •Elderly onset •About 60% •Fatal Chronic progressive •Young onset •About 30% •Better prognosis Relaping 11/30/2017 4
- 5. Presentation • Weakness • Gait abnormalities • Some sensory complaint 11/30/2017 5
- 6. Clinical finding • Diminished power • Sensory impairment • Areflexia/ Hyporeflexia • Postural tremor in hands • Nerve thickening • Papilledema • Facial/ Bulbar weakness • Diplopia 11/30/2017 6
- 7. Variants • Pure sensory • Pure motor • MADSAM (Lewis-Sumner Syndrome) • DADS 11/30/2017 7
- 8. Complications • Respiratory failure/Aspiration • Autonomic dysfunction (Cardiac, Bowel- Bladder, GIT) • Myelopathy • Vision loss • CNS demyelination resembling MS 11/30/2017 8
- 9. Diagnostic criteria Mandatory inclusion criteria: • Progressive or relapsing muscle weakness for 2 months or longer • Symmetrical proximal and distal weakness in upper and lower extremities • Hyporeflexia or areflexia 11/30/2017 9
- 10. Mandatory exclusion criteria: • Evidence of relevant systemic disease or toxic exposure • Family history of neuropathy • Nerve biopsy findings incompatible with diagnosis Diagnostic criteria……cont. 11/30/2017 10
- 11. Diagnostic criteria……cont. Mandatory laboratory criteria: • Nerve conduction studies with features of demyelination (motor nerve conduction <70% of lower limit of normal) • Cerebrospinal fluid protein level>45 mg/dl, cell count<10/µl • Sural nerve biopsy with features of demyelination and remyelination including myelinated fiber loss and perivascular inflammation11/30/2017 11
- 12. Diagnostic categories Definite: Mandatory inclusion and exclusion criteria and laboratory criteria Probable: Mandatory inclusion and exclusion criteria and two of the three laboratory criteria Possible: Mandatory inclusion and exclusion criteria and one of three laboratory criteria 11/30/2017 12
- 13. Associations CIDP is a syndrome presented in many diseases HIV infections SLE DM MGUS Plasma cell dyscrasia Chronic active hepatitis IBD Hodgkin lymphoma Hashimoto’s thyroiditis Drugs 11/30/2017 13
- 14. Differentials • AIDP • Sub-acute GBS • Multifocal motor neuropathy • Amyotrophic lateral sclerosis • Hereditary motor and sensory neuropathy 11/30/2017 14
- 15. Features CIDP MMN ALS Weakness Proximal=distal Usually Symmetrical Distal>proximal Asymmetrical Distal>Proximal UML sign --- --- +++ Sensory loss +++ --- ---- Motor conduction block Frequent Frequent Absent Sensory Low SNAPs Normal Normal CSF protein Elevated Normal >70% Normal 11/30/2017 15
- 16. Investigations Nerve conduction study: a) Reduction in motor conduction velocities in at least two motor nerves b) Partial conduction block or abnormal temporal dispersion in at least one motor nerve at non entrapment sites c) Prolong distal latencies in at least two motor nerves d) Absent F waves or prolonged F wave latencies in at least two motor nerves 11/30/2017 16
- 17. Investigation…..cont. CSF: CSF protein >45mg/dl in 95% of cases 11/30/2017 17
- 18. Figure. Sagittal (A through C) and parasagittal MR slices of the lumbar spine in a patient with chronic inflammatory demyelinating polyradiculoneuropathy. Gerd Diederichs et al. Neurology 2007;68:701 11/30/2017 18 MRI:
- 19. Nerve biopsy: 1) Moderate reduction in myelinated fibres 2) Endoneural and subperineural edema 3) Segmental demyelination and remyelination demyeli 48% axonal 21% mixed 13% Normal 18% 11/30/2017 19
- 20. Others investigations CBC with ESR FBS,HbA1C HIV antibody CXR Hepatitis profiles Thyroid function test Serum and urine immunoelectrophoresis and immunofixation Skeletal bone survey ACE ANA LFT RFT Vasculitic screening 11/30/2017 20
- 21. Treatment When to initiate treatment? Progression is rapid or walking is compromised (significant functional deficit) Treatment options: 1) IV Ig 2) Plasma exchange 3) Corticosteroid 4) Other Immunosuppressive agents 11/30/2017 21
- 22. IVIg: • 2gm/kg body wt given in divided doses over 2-5 days; • three monthly courses are recommended • Gradually reduce dose and increase gap between the doses………….. interval according to response Plasma exchange: Initiated two to three treatments per week for 6 weeks 11/30/2017 22 Treatment….cont.
- 23. Treatment….cont. Corticosteroid: 60 -80 mg Prednisolone PO daily for 1 -2 months Continue high dose until a remission or plateau gradual reduction of 10 mg per month …patient may need 10 to 30 mg alternate day steroid for 2 to 3 years to prevent relapse Pulse steroid few side effect but equally effective (40dexa 40mg/day for 4 days…….then repeat 4 weekly 11/30/2017 23
- 24. IV Ig PE Steroid Response rate 70 80 65 Response speed ++++ +++ ++ Relapse +++ +++ _ Cost Complication (immediate and late) 11/30/2017 24
- 26. Mechanism of action: Uncertain and probably multi- factorial. • Neutralize circulating antibody • Blocks complement cascade IVIg 11/30/2017 26
- 27. IVIg Side effects • MI • CCF • Stroke DVT, retinal vein thrombosis • Anaphylaxis in IgA deficiency • Renal failure • Migraine attacks • Aseptic meningitis • Acute renal tubular necrosis Contraindication •Low IgA •Advanced renal failure •Uncontrolled HTN •Hyperosmolar state 11/30/2017 27
- 28. Fig: Plasmapheresis/ apheresis machine at BSMMU 11/30/2017 28
- 29. Plasma exchange 40-50 ml/kg three times over a week Adverse effects Need for large bore venous access Pneumothorax Hypotension Arrhythmia Electrolyte imbalance Potential exposure to blood products Suppression of the patient's immune system Autonomic instability Hypocalcemia Bleeding due to depletion of clotting factors 11/30/2017 29
- 30. Prognosis 50% severely disabled at some point of disease 10% patients remain disabled in spite of treatment 95% respond with improvement after immunosuppressive therapy 40% remained in partial or complete remission without any medication 62% 26% 12% Good Failed to respond Died Six years after treatment 11/30/2017 30
- 31. References • Harrison’s Neurology in clinical medicine – 4th edition • Bradley’s Neurology in clinical practice – 6th edition • Adams and Victor’s Principles of Neurology – 10th edition • Davidson’s principles and practice of medicine – 22nd edition • Medscape • www.aan.com • Google Image 11/30/2017 31
Editor's Notes
- #3: Incidence less than GBS but prevalence more than GBS………ten times more in DM both insu dep and non insu dep…….
- #5: Monophasic and progressive all….,,,steo wise and progressive…….33%
- #6: Manifestation extremely variable, proximal equal distal. Not length dependent, both upper and lower limb involved. Sensory…tingling and numbnesss most prominent but sometimes pain may occur…. Preceding infection (infrequent) Initial limb weakness, both proximal and distal Sensory symptoms (eg, tingling and numbness of hands and feet) Motor symptoms (usually predominant) In about 16% of patients, a relatively acute or subacute onset of symptoms
- #7: Symmetri, proxi equal distal….stocking glove
- #8: Multifocal acquired Demyelinating sensory and motor ……………..asymmetricall Distal acquired symmetrical Demyelinating neuropathy…………..Ataxia may present in sensory
- #9: Massive nerve root enlargement, symptomatic lumbar stenosis…..progressive pseudotumor cerebri
- #14: Associateion found about 25% cases………………………tacrolimus, macrolides, etaneercept, infiximab, adlimumab………. ………drug after 2 weeks to 16 weeks
- #15: Family history, bony abonormalities, pes cavas, hammer toe, proximal more than distal muscle weakness……MGUS….older, more protracted course, less response to immunotherapy…
- #17: Evidence of axonal loss due to prolong demyelination can be found in 50% case…..have a poor prognosis……….. At least three criteria are necessary to diagnosis CIDP
- #18: 45---250…1000 papilloedema………….elevated gamma globulin and lymphocytic pleocytisis in 10% cases …………. and level above 100mg/dl are common Pleocytosis if hiv
- #19: Figure. Sagittal (A through C) and parasagittal MR slices of the lumbar spine in a patient with chronic inflammatory demyelinating polyradiculoneuropathy. (A) T2-weighted image, showing lack of regular fluid-isointense signal, due to swollen cauda equina fibers (arrows). (B) Corresponding T1-weighted, fat-saturated image. Diffuse cauda equina enhancement (arrows) is depicted, indicating inflammation. (C) Parasagittal T1-weighted, fat-saturated image. Enlarged and enhancing root fibers are shown, exiting the neuroforamen (arrows).
- #20: Semithin section
- #22: Mild Disorder : Awating spontaneous remission
- #24: 50% achieve plateau in 6 months….steroid side effect sodium not more2 gm/day, ppi , ca, vit d, cat, bisphosphonates
- #25: One third become resistant to ivig andd pe after repeated use in 1 or more years
- #26: For resistant cases, no RCT evidence………..high dose cyclo following GCSF shown promising result in resistant cases