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Neurology: Myasthenia Gravis Upper Limbs

The document discusses the clinical evaluation and management of a patient with suspected myasthenia gravis (MG), highlighting key examination findings such as ptosis and fatigable weakness. It outlines the differentiation between neuromuscular junction disorders and myopathies through neurophysiological tests and antibody testing. Initial management steps include resuscitation in acute cases, monitoring, and treatment options like pyridostigmine and immunosuppressive therapies, along with a CT scan to check for thymoma.

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Naseeb Ullah
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17 views3 pages

Neurology: Myasthenia Gravis Upper Limbs

The document discusses the clinical evaluation and management of a patient with suspected myasthenia gravis (MG), highlighting key examination findings such as ptosis and fatigable weakness. It outlines the differentiation between neuromuscular junction disorders and myopathies through neurophysiological tests and antibody testing. Initial management steps include resuscitation in acute cases, monitoring, and treatment options like pyridostigmine and immunosuppressive therapies, along with a CT scan to check for thymoma.

Uploaded by

Naseeb Ullah
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd
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Neurology

Myasthenia gravis upper limbs:

Follow Up Questions/Answers

Could you please present the case to me?


o On general inspection, there is evidence of:
• left-sided partial ptosis
• normal tone on examination
• reduced power proximally
• initially brisk looking reflexes, however, on examining for Hoffman’s reflex
and checking for finger jerks, I can conclude that there is no evidence of
pathologically brisk reflexes
• intact coordination and sensation.
o When we consider patients presenting with weakness in the context of normal
sensation and normal reflexes, we can localise the pathology to either:
• the neuromuscular junction in terms of myasthenia gravis (MG)
• the muscles themselves - for example, a myopathy.
o We can differentiate between these two broad categories with neurophysiological
tests:
• In patients with neuromuscular junction conditions, I would expect repetitive
stimulation to show a decrement in the action potentials associated with
stimulation.
• In patients who present with myopathy, I would expect to find characteristic
changes on the electromyography (EMG).
o We can also help to differentiate between these two with antibodies:
• for example, I would expect acetylcholine receptor antibodies to be positive
in patients with MG.
What clinical tests could you do at the bed side to help differentiate between these
two possibilities?
o There are further examination findings that I could look for to help to conclude a
diagnosis of MG, so for example:
• If I examine the eyes, I would be looking for ptosis - fatigable ptosisgets -
worse after looking up for a prolonged period.
• In MG as well, you can get a complex ophthalmoplegia, so its restricted eye
movements but without reflecting any of the particular cranial nerves.
o The limbs can show fatigable weakness, so for example I can check shoulder
abduction strength, then ask her to exercise this muscle group and then I would
expect it to be weaker the second time I check this strength.
• There are some other clinical tests that can be performed, for example the
use of an ice bag, carefully placed on the ptosis could provide some resolution
of the symptoms in patients with myasthenia.
Neurology

What points in the history would be helpful in concluding this is myasthenia?


o It would be important to take a thorough history of patients presenting with these
symptoms we should look at the onset of the symptoms and the progression and
the amount of time it has taken to reach their current symptoms.
o Other symptoms that should be specifically enquired about would include things
such as the symptoms getting worse towards the end of the day, which I would
expect in patients with myasthenia or after a period of sustained exercise getting
worse as well.
o It will be particularly important to ask about features, so do they notice diplopia,
do they notice any change in their voice, or in their swallowing.
In newly diagnosed myasthenia, what are some important initial steps to take?
o In patients who are presenting with a myasthenia crisis, so for
example if they presented acutely, it would be important to approach the patient
in an ABCDE manner and resuscitate them, as necessary.
o With monitoring of forced vital capacity, I would want to discuss this patient with
my senior colleagues early, and with other colleagues in intensive care to see
whether they would need any ventilatory support.
o In patients who present more sub-acutely it would be important to make the
diagnosis by way of history, examination, neurophysiological studies and by
testing for antibodies.
o Once the diagnosis has been made, it would be important to arrange for a
computed tomography (CT) chest, to look for any evidence of a thymoma, which, if
present, might need to be resected Treatments for this condition would include:
• Medical treatments, so for example pyridostigmine can be used to help
with the symptoms, however, would not change the natural history of
the condition.
• Immunosuppressive therapies, such as steroids, can be used to help
dampen down the immune system; titration of steroid sparing agents can
also be used.
Neurology

Myasthenia gravis upper limbs:

Key Words and Phrases

This candidate was asked to examine the upper limbs in a patient who presented with
difficulty in dressing. The neurological examination was performed in a confident, fluent,
and well-structured manner. As is customary in upper limb exams, the candidate inspected
the patient in general and then checked tone, power, reflexes, coordination, and
sensation. All the key elements were covered, and the pertinent signs were elicited.
The main finding was weakness, and the candidate did well in the assessment of this. He
remembered to put the muscles into activation first, checking power against gravity and
then testing against resistance. Other aspects of the examination were normal, which is a
key point and helps in the differential diagnosis. The candidate clearly felt that the reflexes
might have been initially brisk, so sensibly checked for pathological reflexes by testing for
finger jerks and Hoffman’s sign.
The fact that tone was not increased, and the reflexes were not brisk means that this
weakness is unlikely to be due to an upper motor neurone lesion. The fact the reflexes
were normal rather than depressed or absent means that the weakness is unlikely to be
neurogenic. So, this leaves us with only a couple of options – the weakness could be either
myopathic or due to a neuromuscular junction disorder. Both problems tend to result in
proximal and symmetrical weakness, so the pattern will not help us differentiate these
disorders.
So, what other signs can we use? Well, looking for eye involvement is crucial as
neuromuscular disorders such as myasthenia characteristically involve the eyes, although
it is less common in Lambert Eaton myasthenic syndrome. If you can demonstrate
fatigable ptosis and/or oculoparesis then myasthenia is highly likely. In this case the
candidate noted on observation that there was a left-sided partial ptosis, and this is a
reasonable clue to the fact that this patient does indeed have MG. Another useful clinical
sign is the demonstration of fatigable proximal upper limb weakness. This is conducted by
asking the patient to repeatedly abduct their arm at the shoulder and then comparing the
strength there with the opposite non-exercised side.
Investigating Myasthenia is straightforward. Most patients will be seropositive, so the first
line investigation is acetylcholine receptor antibodies. In patients who are antibody
negative, neurophysiology can be helpful; repetitive stimulation or single fiber EMG can
demonstrate characteristic changes in support of a neuromuscular junction disorder.
Having confirmed a new diagnosis of MG, there are several important next steps. Firstly,
there are some decisions to make about treatment – this will be guided by the severity of
the presentation. In acute cases - sometimes referred to as myasthenic crises - inpatient
care, intensive monitoring, and treatment with IVIG or plasma exchange may be
required. At the other end of the spectrum, there are some very mildly affected patients
who only need pyridostigmine. In generalised myasthenia, however, most patients will
need immunosuppression.
In all patients diagnosed with myasthenia we get a CT thorax to check for the presence of a
thymoma.

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