Suggest Treatment For Weakness In Muscles
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Detailed Answer:
Hello Doctor.
I am sorry that you are not feeling well. From reading your question I imagine you must be in great distress.
I went back and reread our last exchange and to be honest your symptoms don't look typical for myasthenia gravis. For one thing you don't mention ptosis or any other ocular symptoms during those 2-3 years you have had your symptoms. Ocular symptoms are present in up to 90% of patients with myasthenis gravis during the course of the disease. Also your symptoms had a distal predominance while in myasthenia a proximal involvement is more characteristic. CK elevation isn't characteristic either. So while it can not be excluded myasthenia gravis wasn't the first alternative to be considered.
As for lipoprotein receptor-related protein-4 (LRP4) test, that is a new test which only in these last few years has entered into practice (not done at all in my country). LRP4 and muscle-specific kinase (MuSK) forms together with acetylcholine receptor (AChR) clusters in the neuromuscular junction. However LRP antibodies are not the routine exam, they are present in only around 18% of myasthenia patients and are tested only in specialized centers, when other tests come back negative.
So I would like to know what other findings made the doctors reach towards that conclusion. Do you have ocular signs now?
Did you have an edrofonium test (improvement of symptoms after edrophonium application)?
What were the findings of the EMG?
What about antibodies towards AChR and MuSK what were the results of their testing?
Were any other alternative diagnoses mentioned like Lambert Eaton myasthenic syndrome?
I remain at your disposal for further questions possibly answering the questions above.
i dont particuarly have ocular symptoms but i feel weakness in my lower facial muscles and i want to droop my neck
the nerve conduction studies showed a decrement in contraction after repetetive stimuli the neurologist said
he tested my eye muscles and lip muscles and found them to be weak
he could easily open them with his hands
I donno if it is age related tired XXXXXXX or weakness proper
the nerve conduction on other bigger muscles of the hand and lower leg were normal and the sensory aspect is normal
i have a family hisory of hashimotos and my mother had polymtositis as i mentioned before
he did not do an edrophonium test
iam doing a ct chest some time, and Ach and MUSK antibodies were oredered and the results are awaited
My muscle weaknees is more of cetral and proximal type now
shoulder, upper arms, thighs and legs, neck and back muscles
iam able to funtion near normally but i can feel the weakness while I work
Iam a pathologist and have some diffculty putting my neck erect and I am trying to adjust my microscope posture
this is my update
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Detailed Answer:
Well, now that you have added that info I must say that MG could well be the case, apparently there is facial and ocular involvement which has been mild and not been noted by you. Decrement after repetitive stimuli also indicate MG.
Edrophonium test is usually performed. I am sure your neurologist knows that himself though. One explanation why it hasn't been done might be that it would be difficult to assess if there's improvement or not, usually it's done by evaluating an objective signs like a change in ptosis, but since you have none, tough to quantify a change in your other symptoms.
Another possible cause for a myasthenic syndrome might be Lambert Eaton. If you're sure that symptoms have been present for 2-3 years though it's not likely as being a paraneoplastic syndrome the primary tumor would have manifested itself.
I don't think you should worry much about LRP antibodies for now since the Ach and MUSK ones aren't back yet, LRP is done only if those are negative which is rare in MG. If they come back positive the combination of clinical picture, EMG and antibodies, with a tendency for autoimmune conditions, would confirm the diagnosis.
CT scan is always done for thymoma. Actually even if it comes back negative thymectomy often improves symptoms and is done anyway in generalized myasthenia. It is done routinely for patients 10-55, often in older age as well, and since you are only 56 it should be considered.
As for medical therapy as you might know yourself as a physician, it consists mainly in AChE inhibitors and immune suppressing therapy.
I hope to have been of help.
just pl.let me know
1. the chances of progression into a more severe disease in percentage and the duration taken for it
2. and are there chances of it being stable at my stage? has it reached its peak yet?
3.and percentage of myasthenic crisis in my case
XXXXXXX
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Detailed Answer:
I like the way in which you put your questions which makes perfect sense.
However it is a little hard to answer when it is the case of one of the most variable and unpredictable conditions. By variable I don't mean only between different patients but also in the same patient in time.
Most cases do progress gradually in the first few years, but the peak is reached at 2-3 years, so since you report symptoms for a couple of years chances are this could be its peak. However the disease does vary a lot in the same patient, it can be influenced by many factors such as infections, certain drugs, surgery or trauma, endocrine imbalance. So even in a stable patient any of those factors can precipitate a myasthenic crisis. The therapy itself, the start of the corticosteroid treatment can initially aggravate the clinical picture.
Also the results of your tests are necessary as there is different response to treatment, patients anti AChR positive have a better prognosis and response to treatment than anti MuSK positive ones. Presence of thymoma also is a negative prognostic factor.
The percentage of a myasthenic crisis during the course of the disease is around 13%.
So as you can see there are many variables at play. It's a treatable disease though, with most patients leading normal lives if regularly followed by a neurologist and considering the healthcare system in your country you will certainly get the best possible care.
This morning I found out I DONT have any mediastinal/ thymic lesions
Iam waiting for my blood test results
And will take your advise after I get them
Thank you again XXXXXXX
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Detailed Answer:
That is good news.
If antibodies come back positive though and diagnosis is confirmed I would still consider a thymectomy (for possible hyperplasia which is not visible) which as I said leads to improvements in many patients.
I will willingly answer any more queries you might have.