Lambert Eaton

syndrome

Lambert-Eaton
Myasthenic Syndrome (LEMS)
Disorder of the neuromuscular junction in which
antibodies are made against presynaptic voltagegated calcium channels
Symptoms include proximal muscle weakness, fatigue
and autonomic dysfunction
Annual Incidence = 0.48 per million population
There is a high association with malignancy

 Lambert-Eaton myasthenic syndrome (LEMS)

is an autoimmune disease a disease in

which the immune system attacks the body's
own tissues.
The attack occurs at the connection between
nerve and muscle (the neuromuscular
junction) and interferes with the ability of
nerve cells to send signals to muscle cells.

 Occurs sporadically or as paraneoplastic

syndrome chiefly small cell lung carcinoma

May be mistaken with myaesthenia gravis

Pathophysiology

Signs and Symptoms

Symptoms

Signs

Proximal limb weakness

Legs > arms
Fatigue or fluctuating weakness
Difficulty rising from sitting;
climbing stairs
Metallic taste in mouth
Autonomic dysfunction
Dry mouth
Constipation
Blurred vision
Impaired sweating

Proximal limb weakness

Legs > arms
Weakness on exam is less
demonstrable than pts level
of disability
Hypoactive or absent muscle
stretch reflexes
Lamberts sign (grip becomes
more powerful over several
seconds)
Sluggish pupillary reflexes

 Weakness or loss of movement that can be

more or less severe, including difficulty

chewing, difficulty climbing stairs, difficulty
lifting objects, difficulty talking, drooping of the
head, and the need to use the hands to get up
from a sitting or lying position
Swallowing difficulty, gagging, or choking
Vision changes, such as blurry vision, double
vision, and problems keeping a steady gaze
Generally occurs over the age of 40

Physical examination
A physical examination shows:
Decreased reflexes
Possible loss of muscle tissue
Weakness or paralysis that gets slightly better

with activity
Autonomic system

Diagnosis
Blood tests to look for the antibodies that

attack the nerves

Electromyography (EMG) to test the health of
the muscle fibers
Nerve conduction velocity (NCV) to test the
speed of electrical activity along nerves

Electrodiagnostic Evaluation with

Repetitive Nerve Stimulation:
moderately severe disorder of presynaptic
neurotransmission with findings supportive of
an endplate myopathy
Compound Muscle Action Potentials (CMAP) Post-Exercise Facilitation

Exercise testing in
LEMS with median
nerve stimulation and
abductor pollicis brevis
muscle recorded

Baseline

Immediately after 10
seconds of maximal
voluntary exercise

Malignancy diagnosis
Tumor markers
CT chest/abdomen/pelvis
PET scan

Myastenia Gravis vs. LEMS

Both are acquired autoimmune disorders characterized
by defective neuromuscular transmission
LEMS

MG

Antibodies against voltage-gated

Ca channels

Antibodies about acetylcholine

receptors

Usually starts at extremities and

moves up

Usually starts at eyes and moves

down

Autonomic dysfunction present

No autonomic dysfunction

Diplopia and dysphagia uncommon Diplopia and dysphagia common

Weakness improves with activity

Weakness worsens with activity

Associated with SCLC

Associated with thymoma

PT