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Disorder of The Neuromuscular Junction: Courtesy . DR - Syeda Afsheen Hasnain DPT/MSPT Neuroloical

The key points are: - Myasthenia gravis is a postsynaptic disorder of the neuromuscular junction where antibodies are produced against acetylcholine receptors on muscles, blocking and destroying these receptors. - Symptoms include fluctuating muscle weakness that worsens with use and improves with rest, most commonly affecting eye muscles causing drooping eyelids or double vision, or face/throat muscles causing speech/swallowing problems. - Tests like repetitive nerve stimulation can help diagnose by showing fatigue at neuromuscular junction, and antibodies to acetylcholine receptors are present in many cases.

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CHANGEZ KHAN SARDAR
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98 views16 pages

Disorder of The Neuromuscular Junction: Courtesy . DR - Syeda Afsheen Hasnain DPT/MSPT Neuroloical

The key points are: - Myasthenia gravis is a postsynaptic disorder of the neuromuscular junction where antibodies are produced against acetylcholine receptors on muscles, blocking and destroying these receptors. - Symptoms include fluctuating muscle weakness that worsens with use and improves with rest, most commonly affecting eye muscles causing drooping eyelids or double vision, or face/throat muscles causing speech/swallowing problems. - Tests like repetitive nerve stimulation can help diagnose by showing fatigue at neuromuscular junction, and antibodies to acetylcholine receptors are present in many cases.

Uploaded by

CHANGEZ KHAN SARDAR
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PPTX, PDF, TXT or read online on Scribd
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Disorder of the Neuromuscular Junction

Courtesy …. Dr.SYEDA AFSHEEN HASNAIN


DPT/MSPT NEUROLOICAL
MYSTHENIA GRAVIS
• At the neuromuscular junction motor neurons release acetylcholine that binds to
acetylcholine receptors on the postsynaptic muscle fibers, causing them to contract.
Diseases of the neuromuscular junction can be classified as either presynaptic (e.g.,
Lambert-Eaton syndrome, botulism) or postsynaptic (e.g., myasthenia gravis)
• Myasthenia gravis (MG) is a neuromuscular disorder that causes weakness in the
skeletal muscles, which are the muscles your body uses for movement. It occurs
when communication between nerve cells and muscles becomes impaired. This
impairment prevents crucial muscle contractions from occurring, resulting in muscle
weaknes
• Myasthenia gravis is a postsynaptic disorder of the neuromuscular junction in which
antibodies are produced against components of the acetylcholine receptors on
muscle. These antibodies are most commonly against the acetylcholine receptor
itself, leading to blocking and destruction of these receptors
• Weakness in myasthenia fluctuates and is fatigable: It emerges or worsens with
exertion and late in the day, and resolves with rest. Imagine the acetylcholine rushing
across the neuromuscular junction to synapse on its receptors on the muscles. If
these receptors are blocked or diminished in number due to antibody-mediated
destruction, the available receptors will quickly be saturated, and no further
response to acetylcholine can be elicited. This leads to initial muscular force but
subsequent fatigue.
Symptoms

• Muscle weakness caused by myasthenia gravis worsens as the affected muscle is used
repeatedly. Because symptoms usually improve with rest, your muscle weakness may
come and go.
• Ocular (ptosis, diplopia) 45-50%
• Bulbar (dysarthria, dysphagia) 20%
• Extremity weakness (usually proximal) 30-35%
• Distal extremity -rare
• Respiratory -rare
• Eye muscles
• In more than half the people who develop myasthenia gravis, their first signs and
symptoms involve eye problems, such as:
• Drooping of one or both eyelids (ptosis).
• Double vision (diplopia), which may be horizontal or vertical, and improves or
resolves when one eye is closed.
Face and throat muscles

• In about 15 percent of people with myasthenia gravis, the first symptoms involve
face and throat muscles, which can cause:
• Altered speaking. Your speech may sound very soft or nasal, depending upon which
muscles have been affected.
• Difficulty swallowing. You may choke very easily, which makes it difficult to eat, drink
or take pills. In some cases, liquids you're trying to swallow may come out your nose.
• Problems chewing. The muscles used for chewing may wear out halfway through a
meal, particularly if you've been eating something hard to chew, such as steak.
• Limited facial expressions. Your family members may comment that you've "lost
your smile" if the muscles that control your facial expressions have been affected.
• Neck and limb muscles
• Myasthenia gravis can cause weakness in your neck, arms and legs, but this usually
happens along with muscle weakness in other parts of your body, such as your eyes,
face or throat.
• The disorder usually affects arms more often than legs. However, if it affects your
legs, you may waddle when you walk. If your neck is weak, it may be hard to hold up
your head.
Investigations
• What tests would you order?
• Antibodies
• Tensilon & Ice Pack test
• EMG/ NCS: what should you ask for?
• Repetitive stimulation
• Single fiber EMG (SFEMG)
• CT chest
AchR Abs in MG?
• Anti-AChR antibody is positive in what percentage of generalized & ocular MG?
• 85-90% of generalized adult MG patients
• 50% of childhood MG
• 50-70% of Ocular MG
• MUSK
• In >40% of patients with no Ach Abs
• “Seronegative”
Diagnosis

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