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Incidence
• 1.5-2 per 100.000 /year
• all ages
Acute inflammatory polyneuropathies
• Subgroups Typically
• AIDP Acute infl demyelin. pnp Motor/sensory, relatively good progrnosis
• AMSAN Acute motor-sensory axonal pnp Motor/sensory, severe nerve damage
• AMAN Acute motor axonal pnp Weakness, normal sens, severe nerve
damage
• MFS Miller Fisher´s syndrom Ataxia (sens) and diplopia, often not weak
GBS; Symptoms
Predominantly weakness
– few or many muscle groups
– usually symmetic, but not always
– 1/6 need respirator
– 50% have cranical nerve involvement
•Sensory symptoms may dominate
– distal parestesia
– Deep sensibily most involved
– pain may be prominent
Findings
• reduced reflexes
– if refl are normal but no weakness, diagnosis ??
• ataxia (peripheral or brain stem)
• unclear papilledema
• autonomic dysfunction common
• sympatetic-orthostatic hypotension
• parasympatetic-tachycardia, bladder paresis, bowel
paresis
• can give mors subita
Time course
• The symptoms develop during days-weeks
• Progress < 2 weeks at 50%
• If progress > 8 weeks -> CIDP
• Recovery varies from weeks to months
– the slowest at axonal injury
• Mortality 5%
– autonomic dysfunction
– complications to immobilization
– 10% sequel
– 3% reoccur within weeks, months, years
Etiology och pathophysiology
• Autoimmune disease
– 2/3 comes after infection or surgery
– vaccinations
– malignant lymphoma
– aids
• Pathophysiology
– perivenulär patchy endoneural infiltration of mononuclear cells
– conduction block
– segmental demyelination
– axonal degeneration in severe cases
Impulse conduction block
MUSCLE
Two stimulation points;
Amplitude reduction = conduction block
F-response
MUSCLE
F-response is a recurrent response travelling from
stimulation to spinal cord and then to the muscle.
CV in entire nerve, also proximal part.
At conduction block, no transmission
in blocked nerve.
stim
A-waves
MUSCLE
A wave is usually generated in an abnormal
hyperexcitable axon
EDX vid GBS
Conduction block amplitude decay prox-dist, few F
Demyelination low CV, particularly MCV
Axonal degeneration low CMAP + denervation (EMG)
Motor hyperexcitability A-waves during 1-14 days
Sensory hyperexcitability not seen with routine EDX
May be due to
ion-channel interference
demyelination
Localization
mainly motor nerves
proximal or distal
symmetrical sometimes
Proximal, general or distal slowing
Prox slowing
MUSCLE
Generalized slowing
MUSCLE
Distal slowing
MUSCLE
Acute polyradiculitis
(Guillain-Barré Syndrome, GBS, AIDP)
Strategy
• demonstrate acute motor and sensory neuropathy
• demonstrate conduction block
• assess: severity, pathology, distribution
Acute polyradiculitis, GBS
-expected findings
Expected abnormal findings
Neurography, MCS
• conduction block
• F waves delayed and few
• DL prolonged
• reduced MCV, sometimes normal initially
• distal amplitude normal/low
Neurography, SCS
• reduced amplitude
EMG
• reduced IP, later acute neurogenic EMG findings
Autonomic tests
• often abnormal
Acute polyradiculitis (GBS, AIDP)
- procedure
Neurography
• MCS: n.med, n.uln, n.tib, n.per unilaterally
• SCS: n.radialis, n.suralis unilaterally
EMG
• < 10 days: not indicated (earlier onset ?)
• > 10 days: degree of axonal involvement
Autonomic tests
• RR-interval, SSR, pletysmography
Sensory thresholds (optional)
• temperature and vibration
Motor conduction block in GBS
-distal and prox CB
wrist
elbow
MCV F-waves
Median nerve
691104
Motor conduction block in GBS-
distal,proximal CB and general demyelination
ankle
knee
MCV F-waves
Fibular nerve
691104
Guillain Barré
Tibial nerve showing cond block below knee
340420
F-waves in GBS.
Proximal cond block
Normal GBS
A-waves
N Medianus
A-waves
N Medianus
A-waves
axon reflex extra discharge
(IDD)
ephaps M satellite
A waves
N Tibialis
A waves
N Tibialis
A- waves and GBSA- waves and GBS
15 year old girl
Uppsala: The final diagnosis if referral asks
GBS and EDX is normal
EDX normal Clinical EDX day
15/113 GBS?, myelopati, TIA 16d
GBS?? 43 d
CVL, vomiting 2d
Myosit, GBS, Ciprofloxacin (pain) 10d
(abnormal day3, normal day 7).
Transient GBS 3d
ADEM 7d
Diarrea after trip abroad 22d
Parestesia, 168d
increase refl, pain 14d
Vaccination 31d
lumbar pain MR uneg. 1d
Meningitis 14d
Inflammation in disc 22 d
GBS 6 y ago, now numbness, exclude GBS 20d
GBS för 8 y ago, now relaps? Prob not 8 år
Uppsala: The final diagnosis (referral, or a
clinical final diagnosis) if EDX at the first
occasion was interpreted as GBS
62 patients with EDX interpreted as GBS
Clinical final diagnosis GBS 60
Weakness, became GBS 1
Numbness – IVIG (GBS 2 years ago) became GBS
1
Referral GBS?
1:a EDX ”patol EDX” (unspec)
Which was the final diagnosis
GBS/GBS trol/GBS? 8
"non-GBS" 8
Encephalitis/myelitis 5
Unclear cases (weakness) 5
Root/Spinal Stenosis 4
Sensory loss 3
Lymphoma, antiHU 2
Central bleeding 2
Miller Fisher 1
Lyme disease 1
Critical illness myopathy 1
40/113 patienter
CIDP, clinical
• Signs of cranial nerve (CN) involvement (eg,
facial muscle paralysis or diplopia)
• Gait abnormalities
• Motor deficits (eg, symmetric weakness of both
proximal and distal muscles in upper and lower
extremities)
• Diminished or absent deep tendon reflexes
• Sensory deficits (typically in stocking-glove
distribution)
• Impaired coordination
CIDP, EDX
Electrodiagnostics - electromyography (EMG) and nerve
conduction study (NCS). In usual CIDP, the nerve
conduction studies show demyelination. These findings
include:
•a reduction in nerve conduction velocities;
•the presence of conduction block or abnormal temporal
dispersion in at least one motor nerve;
•prolonged distal latencies in at least two nerves;
•absent F waves or prolonged minimum F wave latencies in
at least two motor nerves. (In some case EMG/NCV can be
normal).
Chronic polyradiculitis
CIDP
Etiology
• probably prolonged autoimmune reaction
against peripheral nerves.
Strategy
• demonstrate subacute motor and sensory
neuropathy
• demonstrate conduction block
• assess: severity, pathology, distribution
CDPCDP
Distribution of conduction slowing
proximal even distal
AIDP/CIDP ++
CMT1 ++
anti MAG ++
modified from Attrian et al. Clin neurophys March 2001
length dependent dispersion
wrist
below elbow
above elbow
Multifocal Motor Neuropathy; MMNMultifocal Motor Neuropathy; MMN
Clinical featuresClinical features
•debut young adults, slow progression
•progressive weakness, even in non-atrophic muscles
•nerve distribution
•atrophy
•muscle cramps, fasciculations
•distal predominance
•no sensory disturbance
•reflexes diminished Stålberg
MMNMMN
ElectrodiagnosisElectrodiagnosis
•EMG shows denervation
•Nerve conduction:
reduced velocity
conduction block!
sensory normal
Stålberg
MMNMMN
wrist
elbow
Stålberg
MMNMMN
General commentsGeneral comments
•Symptoms similar to ALS!
In diagnosis of ALS, exclude MMN
by means of nerve conduction studies
Check for GM1 antibodies
•Is the disease a motor variant of pure motor CIDP?
Sometimes sensory nerves are involved
Nerve distribution
Conduction block
Effect of immunosuppresive treatment
Weakness also in non-atrophic muscles Stålberg
MMN/CIDP a comparison, Kimura textbook
MMN CIDP
pure motor frequent rare
mononeuritis multiplex yes no
remission/exacerbation no yes
generalized areflexia no yes
CSF protein often normal elevated
site with cond block forearm, brachial plexus entrapment sites
elevated anti-GM1 antibody frequent rare
choice of therapy immunosupp,IVIG steroids.plasmaex
IVIG

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QUIZ RNS
 
QUIZ SFEMG
QUIZ SFEMGQUIZ SFEMG
QUIZ SFEMG
 
NCS special
NCS specialNCS special
NCS special
 
NCS routine
NCS routineNCS routine
NCS routine
 
EMG special
EMG specialEMG special
EMG special
 
EMG routine
EMG routineEMG routine
EMG routine
 
EMG NCS Scoring
EMG NCS ScoringEMG NCS Scoring
EMG NCS Scoring
 
20. Late resp F H
20. Late resp F H20. Late resp F H
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11. Myopati
11. Myopati11. Myopati
11. Myopati
 
TM
TMTM
TM
 
Macro EMG
Macro EMGMacro EMG
Macro EMG
 
History SFEMG
History SFEMGHistory SFEMG
History SFEMG
 
EDX Facialis
EDX FacialisEDX Facialis
EDX Facialis
 
Decrement 171026
Decrement 171026Decrement 171026
Decrement 171026
 
CNE Jitter method
CNE Jitter methodCNE Jitter method
CNE Jitter method
 
ALS m Polio MMN
ALS m Polio MMNALS m Polio MMN
ALS m Polio MMN
 
33. SFEMG in diseases
33. SFEMG in diseases33. SFEMG in diseases
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25. CTS due to median compression at the wrist
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25. CTS due to median compression at the wrist
 
19. Neurography principles
19. Neurography principles19. Neurography principles
19. Neurography principles
 
6. Basic and Gemg
6. Basic and Gemg6. Basic and Gemg
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AIDPCIDP MMN Stålberg

  • 1.
  • 2. Incidence • 1.5-2 per 100.000 /year • all ages
  • 3. Acute inflammatory polyneuropathies • Subgroups Typically • AIDP Acute infl demyelin. pnp Motor/sensory, relatively good progrnosis • AMSAN Acute motor-sensory axonal pnp Motor/sensory, severe nerve damage • AMAN Acute motor axonal pnp Weakness, normal sens, severe nerve damage • MFS Miller Fisher´s syndrom Ataxia (sens) and diplopia, often not weak
  • 4. GBS; Symptoms Predominantly weakness – few or many muscle groups – usually symmetic, but not always – 1/6 need respirator – 50% have cranical nerve involvement •Sensory symptoms may dominate – distal parestesia – Deep sensibily most involved – pain may be prominent
  • 5. Findings • reduced reflexes – if refl are normal but no weakness, diagnosis ?? • ataxia (peripheral or brain stem) • unclear papilledema • autonomic dysfunction common • sympatetic-orthostatic hypotension • parasympatetic-tachycardia, bladder paresis, bowel paresis • can give mors subita
  • 6. Time course • The symptoms develop during days-weeks • Progress < 2 weeks at 50% • If progress > 8 weeks -> CIDP • Recovery varies from weeks to months – the slowest at axonal injury • Mortality 5% – autonomic dysfunction – complications to immobilization – 10% sequel – 3% reoccur within weeks, months, years
  • 7. Etiology och pathophysiology • Autoimmune disease – 2/3 comes after infection or surgery – vaccinations – malignant lymphoma – aids • Pathophysiology – perivenulär patchy endoneural infiltration of mononuclear cells – conduction block – segmental demyelination – axonal degeneration in severe cases
  • 8. Impulse conduction block MUSCLE Two stimulation points; Amplitude reduction = conduction block
  • 9. F-response MUSCLE F-response is a recurrent response travelling from stimulation to spinal cord and then to the muscle. CV in entire nerve, also proximal part. At conduction block, no transmission in blocked nerve. stim
  • 10. A-waves MUSCLE A wave is usually generated in an abnormal hyperexcitable axon
  • 11. EDX vid GBS Conduction block amplitude decay prox-dist, few F Demyelination low CV, particularly MCV Axonal degeneration low CMAP + denervation (EMG) Motor hyperexcitability A-waves during 1-14 days Sensory hyperexcitability not seen with routine EDX
  • 12. May be due to ion-channel interference demyelination Localization mainly motor nerves proximal or distal symmetrical sometimes
  • 13. Proximal, general or distal slowing Prox slowing MUSCLE Generalized slowing MUSCLE Distal slowing MUSCLE
  • 14. Acute polyradiculitis (Guillain-Barré Syndrome, GBS, AIDP) Strategy • demonstrate acute motor and sensory neuropathy • demonstrate conduction block • assess: severity, pathology, distribution
  • 15. Acute polyradiculitis, GBS -expected findings Expected abnormal findings Neurography, MCS • conduction block • F waves delayed and few • DL prolonged • reduced MCV, sometimes normal initially • distal amplitude normal/low Neurography, SCS • reduced amplitude EMG • reduced IP, later acute neurogenic EMG findings Autonomic tests • often abnormal
  • 16. Acute polyradiculitis (GBS, AIDP) - procedure Neurography • MCS: n.med, n.uln, n.tib, n.per unilaterally • SCS: n.radialis, n.suralis unilaterally EMG • < 10 days: not indicated (earlier onset ?) • > 10 days: degree of axonal involvement Autonomic tests • RR-interval, SSR, pletysmography Sensory thresholds (optional) • temperature and vibration
  • 17. Motor conduction block in GBS -distal and prox CB wrist elbow MCV F-waves Median nerve 691104
  • 18. Motor conduction block in GBS- distal,proximal CB and general demyelination ankle knee MCV F-waves Fibular nerve 691104
  • 19. Guillain Barré Tibial nerve showing cond block below knee 340420
  • 20. F-waves in GBS. Proximal cond block Normal GBS
  • 23. A-waves axon reflex extra discharge (IDD) ephaps M satellite
  • 26. A- waves and GBSA- waves and GBS 15 year old girl
  • 27.
  • 28. Uppsala: The final diagnosis if referral asks GBS and EDX is normal EDX normal Clinical EDX day 15/113 GBS?, myelopati, TIA 16d GBS?? 43 d CVL, vomiting 2d Myosit, GBS, Ciprofloxacin (pain) 10d (abnormal day3, normal day 7). Transient GBS 3d ADEM 7d Diarrea after trip abroad 22d Parestesia, 168d increase refl, pain 14d Vaccination 31d lumbar pain MR uneg. 1d Meningitis 14d Inflammation in disc 22 d GBS 6 y ago, now numbness, exclude GBS 20d GBS för 8 y ago, now relaps? Prob not 8 år
  • 29. Uppsala: The final diagnosis (referral, or a clinical final diagnosis) if EDX at the first occasion was interpreted as GBS 62 patients with EDX interpreted as GBS Clinical final diagnosis GBS 60 Weakness, became GBS 1 Numbness – IVIG (GBS 2 years ago) became GBS 1
  • 30. Referral GBS? 1:a EDX ”patol EDX” (unspec) Which was the final diagnosis GBS/GBS trol/GBS? 8 "non-GBS" 8 Encephalitis/myelitis 5 Unclear cases (weakness) 5 Root/Spinal Stenosis 4 Sensory loss 3 Lymphoma, antiHU 2 Central bleeding 2 Miller Fisher 1 Lyme disease 1 Critical illness myopathy 1 40/113 patienter
  • 31. CIDP, clinical • Signs of cranial nerve (CN) involvement (eg, facial muscle paralysis or diplopia) • Gait abnormalities • Motor deficits (eg, symmetric weakness of both proximal and distal muscles in upper and lower extremities) • Diminished or absent deep tendon reflexes • Sensory deficits (typically in stocking-glove distribution) • Impaired coordination
  • 32. CIDP, EDX Electrodiagnostics - electromyography (EMG) and nerve conduction study (NCS). In usual CIDP, the nerve conduction studies show demyelination. These findings include: •a reduction in nerve conduction velocities; •the presence of conduction block or abnormal temporal dispersion in at least one motor nerve; •prolonged distal latencies in at least two nerves; •absent F waves or prolonged minimum F wave latencies in at least two motor nerves. (In some case EMG/NCV can be normal).
  • 33. Chronic polyradiculitis CIDP Etiology • probably prolonged autoimmune reaction against peripheral nerves. Strategy • demonstrate subacute motor and sensory neuropathy • demonstrate conduction block • assess: severity, pathology, distribution
  • 34. CDPCDP Distribution of conduction slowing proximal even distal AIDP/CIDP ++ CMT1 ++ anti MAG ++ modified from Attrian et al. Clin neurophys March 2001
  • 36. Multifocal Motor Neuropathy; MMNMultifocal Motor Neuropathy; MMN Clinical featuresClinical features •debut young adults, slow progression •progressive weakness, even in non-atrophic muscles •nerve distribution •atrophy •muscle cramps, fasciculations •distal predominance •no sensory disturbance •reflexes diminished Stålberg
  • 37. MMNMMN ElectrodiagnosisElectrodiagnosis •EMG shows denervation •Nerve conduction: reduced velocity conduction block! sensory normal Stålberg
  • 39. MMNMMN General commentsGeneral comments •Symptoms similar to ALS! In diagnosis of ALS, exclude MMN by means of nerve conduction studies Check for GM1 antibodies •Is the disease a motor variant of pure motor CIDP? Sometimes sensory nerves are involved Nerve distribution Conduction block Effect of immunosuppresive treatment Weakness also in non-atrophic muscles Stålberg
  • 40. MMN/CIDP a comparison, Kimura textbook MMN CIDP pure motor frequent rare mononeuritis multiplex yes no remission/exacerbation no yes generalized areflexia no yes CSF protein often normal elevated site with cond block forearm, brachial plexus entrapment sites elevated anti-GM1 antibody frequent rare choice of therapy immunosupp,IVIG steroids.plasmaex IVIG