relapsing polyneuropathy electrodiagnosis
TRANSCRIPT
RELAPSING NEUROPATHY ELECTRODIAGNOSIS
Dr Mohamed Ibrahim KhalilPhysical Medicine Rheumatology and Rehab ,
Alexandria University , [email protected]
46 years old male called Sobhy Ahmed , married , From Kafr El Dawwar Presented with:
1. Flail left upper limb 2. Weakness ,wasting and Numbness of both
upper and lower limbs 3. Gait instability , Heaviness of both Lower
Limbs
Condition started 1 year ago by incidious oncet and Progressive course of Weakness of the right upper limb
**The patient neither can comb his hair nor button his shirt **No Diurnal variation **Associated with Dull aching Pain around the am radiating
distally relived by analgesics , (no ppt factors) ** Associated with numbness in his right hand and
forearm The Patient Sought medical advice . Xray , CT and MRI
Cervical spine was done Revealing : C5-6 Posterolateral Disc Bulge
The weakness and wasting of his right upper limb run through a • Progressive course over a period of 3 month duration and his right upper limb become flail with inability to flex his elbow and associated with severe wasting and
weakness in his right hand and forearm muscles • Stationary course for 2 months • Then regressive course upon neurotonics and Physical
therapy (the patient attains Partial recovery , regaining some ability to flex his right elbow )
5 months ago he suffered from the same complaint with the same pattern on his Left Upper Limb
and Presented with :
1. Flail left Upper limb Inability to flex his left elbow 2. Wasting and weakness of hand and forearm muscles3. Gait instability associated with Heaviness of both lower
limbs 4. Loss of weight5. Numbness of the Both upper and lower limbs 6. Pain at his both shoulder , elbow and ankle
The patient was admited to El Hadara University Hospital
Flail left Upper limb Inability to flex his left elbow
Loss of weight
Severe finger clubbing
Wasting and weakness of forearm and hand muscles
Hand Tremors
Wasting of Thenar eminence
Wasting of hypothenar eminence
Wasting of the 1st Dorsal interosseous
CLINICAL EXAMINATION
• The patient is conscious cooperative oriented with normal intellectual • Speech and articulation : Normal receptive expressing, naming ,
writing and reading • Cranial Nerve examination showed ?Tongue fasciculation• Motor examination: Tone : Hypotonia all over Reflexes : Areflexia all over Extensor planter : unequivocal bilaterally Coordination : cannot be assessed due to weakness Gait : instability , inability to walk on heel or tip of the toes
2+
1
33
5
4
4+ 4+
3+ 3
3+ 3
Muscle Power examination
ASYMMETRICAL WEAKNESS
• The patient is Heavy smoker • Patient is not DM nor HTN nor cardiac / renal / hepatic
• NO Bulbar Manifestation • No sphincteric complaint • No Cognitive impairment • NO specific drug or sensitivity • No Relevant Cutaneous Manifestation• No history of previous operation • NO family history of similar condition(-ve consanguinity )
NEUROLOGICAL DIFFERENTIAL DIAGNOSIS
1. Paraneoplastic neuropathy2. Vasculitic neuropathy3. Infectious neuropathy4. Immune mediated neuropathy5. Motor neuron disease6. Multifocal Motor neuropathy 7. Inclusion body Myositis8. Lewis-sumner syndrome
ELECTROPHYSIOLOGICSTUDY
LEFT AXILLARY NERVE
LEFT MUSCULOCUTANEOUS NERVE
RIGHT MEDIAN LEFT MEDIAN
RIGHT ULNAR LEFT ULNAR
RIGHT AXILLARY NERVE
RIGHT MUSCULOCUTANEOUS
LEFT ULNAR NERVE
Poor F wave
RIGHT ULNAR NERVE
Marked delay F wave
Giant waves ???
Poor F wave
RIGHT MEDIAN NERVE
Marked delay F wave
LEFT MEDIAN NERVE
AFCL = ( F + DL ) - (2 х Proximal latency)
F wave Latency Distal Latency
2 х Proximal latency ----AFCL-----
AXILLARY F CENTRAL LOOP LATENCY
13.6 ms
DistalProximal
AFCL = ( F + DL ) - (2 х Proximal latency)
AXILLARY F CENTRAL LOOP LATENCY
Ulnar Nerve : ( 38.6 + 3.6 ) – ( 2 х 11.8 ) = 42.2 – 23.6 = 18.6 ms
Median nerve : ( 42.4 + 4.7 ) – (2 х 12.7) = 47.1 – 25.4 = 21.7 ms
NORMAL F WAVE
5.AXONAL NEUROPATHY AND DEMYLINATING POLYNEURORADICULOPATHY
WHAT ARE THE CASES WITH ABNORMAL MARKED DELAY IN F WAVE ?
1.Diabetic Poly-Radiculoneuropathy 2.Chronic imf demylinating Polyradiculoneurop.3.Early Guillian Barre ( Axillary loop block)4.Severe demylinating peripheral neuropathy5.Demylinating Brachial Plexopathy(Br. Neuritis)
Demylinating Polyneuroradiculopathy
NB: Axonal Lesion never lead to abnormal Delay in Fwave mylinating In severe axonal neuropathy or plexopathy F wave is Lost Single motor Radiculopathy never cause abnormal f wave except if the lesion is severest
RIGHT DEEP PERONEAL
LEFT DEEP PERONEAL
Poor F wave
LEFT POSTERIOR TIBIAL
Poor F wave
• POOR H REFLEX BILATERALLY
RIGHT FEMORAL NERVE
Amplitude of Tested nerves in mV
Asymmetric Pattern
No MultipleMononeuritis Multiplex Pattern
WHAT ARE THE ASYMMETRICAL AXONAL NEUROPATHIES RELEVANT TO THIS CASE ?
1.Non Systemic vasculitic Neuropathy2.Paraneoplastic neuropathy
NOT VASCULITIC
VASCULITIC NEUROPATHY PRESENTED ALWAYS BY
MONONEURITIS MULTIPLEX PATTERN
VERY IMPORTANT TO CHECK FOR IN SUSPECTED PARANEOPLASTIC NEUROPATHY :
1. Neuromyotonia2. Lambert Eaton syndrome3. Polymyositis4. Motor neuron disease5. Vasculitic neuropathy6. Myasthenia Gravis
PATHOPHYSIOLOGY OF PARANEOPLASTIC NEUROPATHY :
IS THE ELECTROPHYSIOLOGICAL FINDING ALONE DEFINITE IN DIAGNOSING PARANEOPLASTIC NEUROPATHY ?
NO
IS ELECTROPHYSIOLOGY DEFINITE IN DIAGNOSING PARANEOPLASTIC NEUROPATHY ?
BACK TO OWR CASE
COMPARATIVE FOLLOW UP ELECTROPHYSIOLOGIC
STUDY
LEFT MUSCULOCUTANEOUS NERVE N
EW
OLD
LEFT ULNAR NERVE
Poor F wave
Markedly delayed F wave
NEW
NEW
OLD
OLD
Poor F wave
LEFT MEDIAN NERVE
Markedly delayed F wave
NEW
NEW
OLD
OLD
Poor F wave
RIGHT MEDIAN NERVE
NEW
OLD
OLD
RIGHT DEEP PERONEAL N
EW
OLD
RIGHT POSTERIOR TIBIAL
OLD
NEW
NEW
RIGHT FEMORAL NERVE
NEW
NEW
OLD
RIGHT ACCESSORY NERVE
NEW
LEFT MEDIAN N
EW
OLD
NEW
NEW
NEW
NEW
NEW
Amplitude of Tested nerves in mV
4
NEWOLD
4 MONTHS
WHAT ARE THE RELAPSING ASYMETRICAL VARIANTS OF CIDP ?
1.Lewis-Sumner Syndrome 2.Chronic relapsing Dysimmune Polyneuropathy
(CRDP )
• The Lewis- Sumner syndrome (LSS) is a dysimmune multifocal demyelinating sensorimotor neuropathy. It should be considered as a clinical asymmetrical variant (CIDP).
• Patients with LSS usually present with an asymmetrical involvement of the upper limb with distal sensorimotor deficit in median or ulnar territories.
1. A purely sensory onset with numbness and paresthesia or pain in median or ulnar territory is observed 30% of cases.
2. A lower limb onset is present in 30% of patients with a distal and asymmetrical sensorimotor deficit.
3. Amyotrophy and cranial nerve involvement may be observed in 50% and 20% of patients,
• LSS could mimick a nerve entrapment or a vasculitis. The course is progressive or remitting.
WHAT IS LEWIS SUMNER SYNDROME?
• Electrophysiological pattern associates a multifocal motor demyelination with conduction blocks mostly situated in the forearm.
• Contrarily to CIDP, other conduction anomalies (reduction
of truncal motor nerve velocities, prolonged distal latencies or prolonged F waves) occur rarely outside the blocked nerve territory.
• Sensory conduction shows a multifocal sensory involvement.
WHAT IS LEWIS SUMNER SYNDROME?
• Sural nerve biopsy in LSS show elements consistent with a primary demyelination, indistinguishable from that seen in typical CIDP. However nervous biopsy is not necessary to establish the diagnosis ( unlike vasculitis ) .
• Serum anti-GM1 antibodies are negative and CSF protein content is usually normal or mildly elevated with a mean value of 0.7 g/l. LSS is characterized by a responsiveness to IVIg and steroids.
• For LSS patients, a treatment similar to that of CIDP,
Plasma exchanges are not recommended in LSS.
WHAT IS LEWIS SUMNER SYNDROME?
WHAT IS CRDP ( CHRONIC RELAPSING DYSIMMUNE POLYNEUROPATHY )?
WHAT IS CRDP ( CHRONIC RELAPSING DYSIMMUNE POLYNEUROPATHY )?
WHAT IS CRDP ( CHRONIC RELAPSING DYSIMMUNE POLYNEUROPATHY )?
