thesnowyday
New member
- Joined
- Apr 30, 2014
- Messages
- 7
- Reason
- Other
- Country
- US
- State
- wa
- City
- vancouver
40 year old male physician, previously healthy
In February 2014, sudden onset over 48 hours of widespread sensation that limbs are falling asleep, which is positionally worse.
Within 1-2 weeks, increasing weakness in R hand, then L hand. Buttoning, typing, writing, cooking.
Over next month, occasional cramping pain both forearms esp in the brachioradialis, weakness progressing. Now pots are heavy, I cannot whip scrambled eggs without muscle failure.
A month later (two months since initial symptoms), occasional cramping pain both quads, esp in the vastus medialis, no clear weakness in legs but subjectively they feel heavy.
Recently, general sensation of internal tremulousness, wakes me at night. No visible fasiculations.
Weight - stable.
No trouble speaking, swallowing, eating, or thinking.
Unusual symptoms: paresthesia like flushing, numbness, mild, over brachial plexus distribution (shoulder, arm, anterior chest, face), R worse than L.
Symptoms are dramatically worse if hand gets cold, almost to the point of being unable to hold a mug.
Neuro exam by excellent general neurologist: normal in all respects, 3 times over 2 months, including normal strength and normal sensation, normal gait, no objective atrophy, no signs of UMN disease such as hyperreflexia/clonus.
functionality: cannot lift weights anymore due to strength/grip weakness, especially pulling exercises. Can still run without any serious/obvious impairment but legs feel heavy.
Labs: normal (CK, chemistries, CBC, TSH, B12)
MRI neck with and without contrast: negative
MRI brain with and without contrast: negative
nerve conduction studies: no abnormalities
EMG: not done yet
CSF studies: not done
anti GM1, other antibody studies: not done
Arguing against ALS/MND: rapid onset; clinically normal exam; notable non-motor symptoms including brachial plexopathy and sensation of limbs falling asleep.
Arguing for ALS/MND: progressive weakness in at least 2 extremities with normal MRI neck and brain and normal NCV studies; tremulousness throughout body (fasiculation equivalent?)
Differential rule out:
1. GBS/CIDP - weakness has continued to progress beyond the usual time frame, and NCV is normal.
2. MS - normal MRI brain and neck with no evidence of demyelinating lesions.
3. MMN with conduction block - no conduction block on NCV, but MMN can exist w/o conduction block.
4. MMA/Hirayama syndrome - weakness has progressed to second extremity.
So.
I've read a lot. Scholarly articles, published journal articles, textbooks, and on the internet. So far, I have no clear evidence of what I have, nor any convincing evidence that I do or don't have ALS/MND.
The symptoms are quite disrupting, though not yet disabling. But of course I'm petrified.
Two questions:
1. ALS/MND is classically described as purely motor. A textbook on the subject goes so far as to say, "Pain and sensory symptoms are impermissible with the diagnosis." However, we know this is not true from other published reports. Question: Has anyone with confirmed ALS/MND had, as their first or one of their first symptoms, paresthesias or the sensation that their limbs are falling asleep?
2. I know I need an EMG. However, given all of the above, can anyone think of any diagnosis which explains these symptoms which is NOT MND/ALS? Progressive weakness in 2 distal extremities with normal MRI brain/neck (ruling out radiculopathy and CNS demyelinating disease such as MS) and normal NCV rules out a lot of things.
Your insights and knowledge much appreciated,
In February 2014, sudden onset over 48 hours of widespread sensation that limbs are falling asleep, which is positionally worse.
Within 1-2 weeks, increasing weakness in R hand, then L hand. Buttoning, typing, writing, cooking.
Over next month, occasional cramping pain both forearms esp in the brachioradialis, weakness progressing. Now pots are heavy, I cannot whip scrambled eggs without muscle failure.
A month later (two months since initial symptoms), occasional cramping pain both quads, esp in the vastus medialis, no clear weakness in legs but subjectively they feel heavy.
Recently, general sensation of internal tremulousness, wakes me at night. No visible fasiculations.
Weight - stable.
No trouble speaking, swallowing, eating, or thinking.
Unusual symptoms: paresthesia like flushing, numbness, mild, over brachial plexus distribution (shoulder, arm, anterior chest, face), R worse than L.
Symptoms are dramatically worse if hand gets cold, almost to the point of being unable to hold a mug.
Neuro exam by excellent general neurologist: normal in all respects, 3 times over 2 months, including normal strength and normal sensation, normal gait, no objective atrophy, no signs of UMN disease such as hyperreflexia/clonus.
functionality: cannot lift weights anymore due to strength/grip weakness, especially pulling exercises. Can still run without any serious/obvious impairment but legs feel heavy.
Labs: normal (CK, chemistries, CBC, TSH, B12)
MRI neck with and without contrast: negative
MRI brain with and without contrast: negative
nerve conduction studies: no abnormalities
EMG: not done yet
CSF studies: not done
anti GM1, other antibody studies: not done
Arguing against ALS/MND: rapid onset; clinically normal exam; notable non-motor symptoms including brachial plexopathy and sensation of limbs falling asleep.
Arguing for ALS/MND: progressive weakness in at least 2 extremities with normal MRI neck and brain and normal NCV studies; tremulousness throughout body (fasiculation equivalent?)
Differential rule out:
1. GBS/CIDP - weakness has continued to progress beyond the usual time frame, and NCV is normal.
2. MS - normal MRI brain and neck with no evidence of demyelinating lesions.
3. MMN with conduction block - no conduction block on NCV, but MMN can exist w/o conduction block.
4. MMA/Hirayama syndrome - weakness has progressed to second extremity.
So.
I've read a lot. Scholarly articles, published journal articles, textbooks, and on the internet. So far, I have no clear evidence of what I have, nor any convincing evidence that I do or don't have ALS/MND.
The symptoms are quite disrupting, though not yet disabling. But of course I'm petrified.
Two questions:
1. ALS/MND is classically described as purely motor. A textbook on the subject goes so far as to say, "Pain and sensory symptoms are impermissible with the diagnosis." However, we know this is not true from other published reports. Question: Has anyone with confirmed ALS/MND had, as their first or one of their first symptoms, paresthesias or the sensation that their limbs are falling asleep?
2. I know I need an EMG. However, given all of the above, can anyone think of any diagnosis which explains these symptoms which is NOT MND/ALS? Progressive weakness in 2 distal extremities with normal MRI brain/neck (ruling out radiculopathy and CNS demyelinating disease such as MS) and normal NCV rules out a lot of things.
Your insights and knowledge much appreciated,
