CIDP + MMN - how to diagnose and treat. Dr Hadi Manji

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2 CIDP + MMN - how to diagnose and treat Dr Hadi Manji

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5 Outline Introduction CIDP Diagnosis Clinical features MRI Nerve conduction tests Lumbar puncture Nerve biopsy Treatment IV Ig Steroids Plasma Exchnage

6 CIDP (1) Incidence 0.15 per 100,000 Prevalence per Mean age of onset 52 years (range 10-82) 20% of referrals to a specialist clinic

7 CIDP (2)- Clinical Progressive weakness (> 2 months) Weakness Distal and proximal Usually symmetrical Sensory loss Large fibre involvement Areflexia

8 CIDPclinical (other features) Cranial nerve lesions Repiratory involvement Autonomic features papilloedema.

9 CIDP - clues to diagnosis Upper limb onset Postural tremor Pseudoathetosis Weakness but no wasting Thickened nerves

10 Neuropathic tremor

11 CIDP - clinical course Progressive Relapsing and remitting monophasic RR then progressive GBS - like onset * Axonal loss correlated with prognosis

12 Outline Introduction CIDP Diagnosis Clinical features MRI Nerve conduction tests Lumbar puncture Nerve biopsy Treatment IV Ig Steroids Plasma Exchnage

13 PT MRI

14 Outline Introduction CIDP Diagnosis Clinical features MRI Nerve conduction tests Lumbar puncture Nerve biopsy Treatment IV Ig Steroids Plasma Exchnage

15 CIDP - investigations NCT and EMG MCV slowing (variable) Prolongation of DML Prolongation of f wave Conduction block Temporal dispersion

16 Outline Introduction CIDP Diagnosis Clinical features MRI Nerve conduction tests Lumbar puncture Nerve biopsy Treatment IV Ig Steroids Plasma Exchnage

17 CIDP - investigations Cell count < 10 cells CSF Protein > 1 gram (80%) Oligoclonal bands - maybe +

18 Outline Introduction CIDP Diagnosis Clinical features MRI Nerve conduction tests Lumbar puncture Nerve biopsy Treatment IV Ig Steroids Plasma Exchnage

19 CIDP - investigations nerve biopsy Demyelinated fibres Remyelinated nerve fibres Endoneurial inflammatory infltrates Schwann cell proliferation (onion bulbs) Macrophage stripping of myelin (EM) Endoneurial oedema

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21 Indications for nerve biopsy in CIDP Discrepency between clinical, neurophysiology and CSF Evaluation of axonal loss as an indicator of prognosis in CIDP Bouchard C et al. Neurology;1999:

22 Nerve biopsy in CIDP Barohn et al. Arch Neurol 1989;46: % 21% 21% 18% demyelination axonal mixed normal

23 CIDP variants Monomelic CIDP Sensory ataxic variant CIDP + CNS involvement CIDP + MGUS (IgA & IgG) Anti-MAG IgM kappa paraproteinaemic demyelinating neuropathy Multifocal demyelinating motor and sensory neuropathy (Lewis Sumner syndrome) Multifocal motor neuropathy with conduction block

24 CIDP - differential diagnosis (1) Hereditary Toxic HMSN 1a,1b, X linked HNPP Refsum s disease MLD Globoid cell leucodystrophy Amiodarone perhexilene

25 CIDP - differential diagnosis (2) Paraproteinaemic Myeloma Waldenstrom s macroglobulinaemia POEMS Castlemans disease

26 Treatment of CIDP Corticosteroids PE ivig 65-95% response 80% 60-70%? Cheap rapid rapid Long term comp invasive relapses Gradual benefit expensive, expensive specialist centre blood product

27 Other immunosuppressive treatments Azathioprine Cycloposphamide Cyclosporine Mycophenalate Interferons

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29 Case study NS 64 year old man Staggering gait Progressive over 2 years Falls (referred from A and E)

30 O/E Postural tremor Gait normal, Romberg s positive Normal power Absent knee and ankle jerks JP absent to ankles, vibration absent to knees, pp reduced to ankles

31 NCT (NS) SAP Upper limbs: reduced Lower limbs: absent (sural and SPN) Motor studies CPN: DML - 8.7ms, CV- 34 m/s, f-wave 69.8ms

32 CSF (NS) 1 WBC Protein 0.42 g/l Oligoclonal bands negative

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38 PT (1) 34 years old Never good at running Age 19 partial L 3rd nerve palsy Age 21 walking difficulty Gradual progression until rapid deterioration April 1999 wheelchair bound

39 PT (2) o/e Cranial nerves - normal Wasting hand and forearm muscles Ulnar nerve thickened Weakness 4+ proximally 1 distally Areflexia JP, Vibration shoulders PP to mid forearm

40 PT (3) Wasting below knees, pes cavus Weakness 2 proximally 0 distally Areflexia JP, vibration to hips PP T 4 (anteriorly)

41 PT (4) Investigations: 1986 : Routine bloods N : CSF 1 WBC 1.35 g/l protein IgG oligoclonal band positive 1999 : CSF 22 WBC >3.0 g/l protein IgG oligclonal bands positive serum negative

42 PT (6) NCT/EMG 1986 : Sensory median (F 2 - wrist) 3.5 mv sural absent : Motor median DML MCV MAP (wrist) MAP (elbow) 3.6 msec 4.3 msec 15 m/sec 4 mv 1 mv (dispersed)

43 PT MRI

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49 Mulltifocal Motor Neuropathy with conduction block (1) Clinical features Progressive, asymmetric weakness Atrophy Fasiculations Multifocal peripheral nerve involvement NCT and EMG Motor conduction block Normal sensory conduction Anti GM -1 antibody + 70% Drug induced TNF blockers

50 MMN with CB Treatment Good response to iv IG (95%) Some deteriorate with steroids Anecdotal benefit with cyclophosphamide

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52 Subjective assesment of strength Better 3.5 Effect of IVIG dose and frequency Patient R Male 52 yrs - 80kg - Subjective assessment of relative strength. IVIG 2.7mg/hr Worse 150mg in 5 days 49 day cycle Assessed on: 150mg in 5 days 42 day cycle 120mg in 4 days 42 day cycle 90mg in 3 days 35 day cycle Rh leg & Lh finger strength, Stair climbing, bladder & ankle control.

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