This document describes the case of a 46-year-old male patient presenting with weakness, wasting, and numbness in both upper and lower limbs over the past year. Electrodiagnostic testing revealed abnormal findings consistent with a multifocal demyelinating sensorimotor neuropathy. Based on the asymmetric progression and electrodiagnostic results, the patient was diagnosed with Lewis-Sumner syndrome, a chronic relapsing demyelinating polyneuropathy.
2. 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
3. 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
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7. 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 )
8. 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 muscles
3. Gait instability associated with Heaviness of both
lower limbs
4. Loss of weight
5. 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
9. Flail left Upper limb Inability to flex his left elbow
Loss of weight
14. • 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
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17. • The patient is Heavy smoker
• Patient is not DM nor HTN nor cardiac / renal / hepatic
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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 )
36. AFCL = ( F + DL ) - (2 х Proximal latency)
F wave Latency
Distal Latency
----AFCL----13.6 ms
2 х Proximal latency
37. AFCL = ( F + DL ) - (2 х Proximal latency)
Ulnar Nerve :
( 38.6 + 3.6 ) – ( 2 х 11.8 ) = 42.2 – 23.6 =
Median nerve :
( 42.4 + 4.7 ) – (2 х 12.7) = 47.1 – 25.4 =
18.6 ms
21.7 ms
38.
39.
40. 1.Diabetic Poly-Radiculoneuropathy
2.Chronic imf demylinating Polyradiculoneurop.
3.Early Guillian Barre ( Axillary loop block)
4.Severe demylinating peripheral neuropathy
5.Demylinating Brachial Plexopathy(Br. Neuritis)
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
86. •
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.
87. • 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.
88. • 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.