3._Vallat_JM_-_Diagnostic_work-up__therapeutic_management_and_electrophysiological__examples.pdf

Peripheral neuropathy

1. Jean-Michel VALLAT
[email protected]
www.unilim.fr/neurolim
Departmentof Neurology
National referralcenter: «rare peripheralneuropathies»
Universityhospital
LIMOGES –France
DIAGNOSTIC WORK UP AND THERAPEUTIC MANAGEMENT OF PERIPHERAL
NEUROPATHIES

2. ASSESSMENT OF A POLYNEUROPATHY
INTRODUCTION
The diagnosisof peripheralneuropathyisessentiallybasedon the
clinicaldata
The electrophysiologicalfindingsare usefulbut not indispensable
Etiologies are numerous: acquiredand genetic(the wholemedicine…)
A generalclinicalexam and a few biologicaltests are mandatory

3. IGA

5. TEASING: NORMAL HUMAN NERVE
RANVIER NODE

6. MULTIFOCAL RANDOMLY DISTRIBUTED DEMYELINATING LESIONS: ACQUIRED

7. DIFFUSE ACUTE AXONAL LESIONS

8. STEPS TO DIAGNOSE A NEUROPATHY
To obtainan history
An accuratephysicalexamination
Electrophysiologictests
Laboratoryevaluation

9. DIAGNOSIS OF A NEUROPATHY
HISTORY
Pastmedicalhistory(underlyingdisease, treatments...?)
Social history(occupations, behaviour...)
Origin: country?
Familyhistory: familytree(consanguinity?)
Course of the disease:
acute, subacute, chronic, long standing
monophasic, progressive, relapsing

10. ASSESSMENT OF A POLYNEUROPATHY
CLINICAL PRESENTATION
Main symptoms:
weakness
sensorydisturbances
walkingdifficulties
Others:
cramps, fasciculations, myotonia, tremor
autonomicsymptoms

11. DIAGNOSIS OF A NEUROPATHY
CLINICAL SYMPTOMS AND SIGNS
(2)
Sensory-motor
Pure motor ganglionopathyor neuronopathy
Pure sensory:
«smallfiberneuropathy»
Predominantinvolvementof the autonomicnervoussystem

12. DIAGNOSIS OF A NEUROPATHY
PATTERN OF DISTRIBUTION
OF NERVE INVOLVEMENT (3)
Mononeuropathy
Multiple mononeuropathy
(or mutiplexmononeuropathy, mononeuriticmultiplex)
Polyneuropathy(distal, proximal, diffuse)
Polyradiculopathy, polyradiculoneuropathy
Plexopathy
Radiculopathy( )

13. CLINICAL CLASSIFICATION OF NEUROPATHIES
(traumatic and entrapment N excluded)
Sensori-motoror motor:
-acute : GBS, AMAN, AMSAN
-subacute:
symmetrical: nutritional, dysimmune(subacuteGBS)
asymmetrical:
multiplex mononeuritis: polyarteritisnodosa, leprosy
-chronic:
symmetrical: proximal and/or distal :
toxic, diabetes, hemopathies, CIDP, nutritional
distal : CMT, DADS
asymmetrical(mono, multiplex neuritis) : leprosy, diabetes
Sensory:
-ataxicand/or sensory(large fibers) : ganglionopathy, neuornopathy
symmetrical: toxic, dysimmune, HSAN
asymmetrical: diabetes, paraneo
-smallfibersneuropathies : diabetes, Sjögren...??
Autonomicsystem involvement:
-latent
-severe(or pure) : rarelyacute : GBS
chronic: diabetes, amyloidosis(smallfibers)

14. STEPS TO DIAGNOSE A NEUROPATHY
To obtainan history
An accuratephysicalexamination
Electrophysiologictests
Laboratoryevaluation

15. ASSESSMENT OF A POLYNEUROPATHY
ENMG
Not mandatory
Helpful
Motor nerve : velocities, distal latencies, F waves, action potentials
Sensory nerve: velocities, action potentials
Electromyogram
AXONAL LOSS-DEMYELINATION

16. CIDP

17. TANKISI Clinicalneurophysio2005

18. LD (ms) Amp (mV) VC (m/s) F (ms)
PERONIER P D NO NO NO NO
TIBIAL D NO NO NO NO
MEDIAN D 4.0 2 44 NO
ULNAIRE D 2.8 5.7 16 35.9
Amp (µV) VC (m/s)
SURAL D NO NO
MEDIAN D NO NO
RADIAL D 1.5 42.5
FasciculationsFibrillationsTracé effort
1er IOD D 0 0 Neurogène
Jambier ant D 0 0 Neurogène
Jambier ant G 0 0 Neurogène
CONDUCTION M OTRICE
CONDUCTION SENSITIVE
EM G NO: not obtained

19. DIAGNOSIS OF A NEUROPATHY
LESIONS
Fibers:
motor
sensory: large and small, large, small(«smallfiberneuropathies»)
Lesions: demyelinating
axonal (walleriandegeneration, dyingback)
mixed
nodo-, para-nodopathy

20. 1
2Walleriandegeneration(axonalprocess)
Dyingback axonopathy3
1 demyelination

21. Modifié, d’après YukiN et Hartung HP, N EnglJ Med, 2012, 366(24):2294-304
mac: abaxonal
mac: adaxonal
Demyelinatingprocess
Axonal subtype: «nodopathy»
ACUTE INFLAMMATORY DEMYELINATINGPOLYRADICULONEUROPATHY
CIDP: mostof cases
MAN
AMAN: acute motoraxonalneuropathy
AMSAN
CIDP: a few cases
NODOPATHIES

22. NODO-, PARANODOPATHIES (Uncini)
May induce: «AXONAL CONDUCTION BLOCK » (CB)
(in conditions whichaffect the excitable axolemmaat the
nodal region)
Arrestotnerve conduction
No dispersion
May promptlyreverse : «reversibleconduction failure»
NC maybeslow and improvein parallelwiththe
resolutionof CB
necessityof severalrecordings

23. STEPS TO DIAGNOSE A NEUROPATHY
To obtainan history
An accuratephysicalexamination
Electrophysiologictests
Laboratoryevaluation

24. DIAGNOSIS OF A NEUROPATHY
SCREENING LABORATORY TESTS
Complete bloodcount
Erythrocyte sedimentationrate
Blood glucose test (impairedglucose tolerancetests)
(Vitamins?)
Liver, renal, thyroidfunctiontests
Serumproteinelectrophoresis(immunofixation?)
Genetictesting: DNA
STORE SERUM IN A FREEZER
CSF STUDY IS NOT MANDATORY

25. EnglandJD 2009 AAN, AANEM, AAPMR

26. ACUTE «PRIMITIVE» DYSIMMUNE NEUROPATHIES
YukiN and Hartung HP NEJM 2012
(AIDP)
(AMAN)
(AMSAN)

27. ANTIBODIES IN PERIPHERAL NEUROPATHY
MAG(myelinassociatedglycoprotein) Monoclonal IgM
MGUS
Waldenström
GANGLIOSIDES:
-GM1 : mutifocalmotorneuropathy(MMN)
AMAN
AMSAN
GBS
-GQ1b:Miller Fisher syndrome (MFS)
CANOMAD (ChronicAtaxicNeuropathy,
Ophthalmoplegia, M protein, cold Agglutinins, Disialosys)
-Multi : GD1b, GT1b, GT1a, GD2, GD3
CANOMAD
PARANEOPLASIC : Hu ; CV2/CRMP5
ANTI PARANODAL –NODAL proteins: CNT1, Caspr1, NF155, NF186
CIDP (nodoparanodopathy)

28. DIAGNOSIS OF A NEUROPATHY
Salivaryaccessoryglands (amyloidosis; Sjogren?)
Skin :
-classicaltechniques (amyloidosis?)
-to count intra-epidermousnerve fibers(smallfiberneurop)
Sensorynerve
Muscle
BIOPSIES

29. Salivarygland biopsy
Sjogren

30. Amyloiddeposits
dermis
epidermis
Skin biopsy
Congo red

31. Normal skin: intra-epidermicnerve fibers(IENF)
epidermis
epidermis
IENF have completelydisappeared
dermis
dermis

32. POLYNEUROPATHIES
CAUSES
SYMPTOMS
MANAGEMENT

33. POLYNEUROPATHIES
MANAGEMENT
Accordingto the causes
Somepatients do not needto be
treatedimmediately followup :
-non malignantdysglobulinemia
-CIDP

34. POLYNEUROPATHIES
Neuropathic pain
Rehabilitation
Outcome measures
Symptomatic management

35. Ouedraogo et coll. Bull. de l’ALLF n°32, Juin 2017, 11-14
(Moneuropathiesand multiplex mononeuropathies)

36. ASSESSMENT OF A POLYNEUROPATHY
CONCLUSION
The diagnosisof peripheralneuropathyisessentiallybasedon the
clinicaldata
The electrophysiologicalfindingsare usefulbut not indispensable
Thinkof CIDP
The understandingof lesionmechanismsmaybemandatoryto decidea
specifictreatment