Zach London Profile picture
Feb 11, 2022 11 tweets 6 min read Read on X
1/
We often check SPEP and/or immunofixation in patients with neuropathy. Why bother, and what should I do with the results?
Welcome to Paraproteinemic Neuropathy: A #tweetorial for neurologists who order tests they have no business interpreting.
2/
For starters, let's clear the air about terminology. These are all synonyms:
- Paraproteinemia
- Monoclonal gammopathy
- M-protein
- M component
They all mean identical plasma cells are overproducing identical copies of the same immunoglobulin or immunoglobulin fragments. From AAN Neurobytes: Paraproteinemia and Neuropathy
3/
Paraproteinemia is common as we age. It's usually benign (MGUS) and asymptomatic, but can be associated with almost ANY neuropathy syndrome.

(PSA: If you do a Google image search for Stocking and Glove looking for neuropathy pics, be prepared to find a bunch of S&M sites.)
4/
How do you test for it? Serum protein electrophoresis AND immunofixation. Getting both will help find low level gammopathies, which can still be associated with neuropathy!
UPEP, BJS, and free light chain ratio might help a little, but I don't include them as screening tests.
5/
Paraproteins will be reported with three pieces of information:
A heavy chain (IgG, IgA, or IgM)
A light chain (kappa or lambda)
A concentration (i.e. 0.6 g/dL)

Almost any other info they report (polyclonal gammopathy, etc.) is probably not useful to neurologists. Fight me.
6/
Different heavy and light chains are associated with different malignancies. Get to know which with this handy chart.
As for me, my heart beats a little faster when I find an IgM heavy chain or a lambda light chain paraproteinemia.
7/
The concentration (or level) correlates with the risk of malignancy.
Low levels, like 0.1 g/dL, are almost always MGUS.
High levels, like 3.0 g/dL = DANGER.

(Is it any wonder that the immunoglobulin fragments can so easily be rearranged to make the #Decepticon symbol?)
8/
Neuropathy may be the first symptom of a heme malignancy, so neurologists may be the first to realize something serious is going on.

Are we heroes? Well, I wouldn't necessarily use that term. People do call us that, though.

So when should we worry and refer to heme-onc?
9/
Consult heme-onc for:
1. IgA heavy chain (IgA probably has the highest risk of malignant transformation.)
2. Paraprotein level >1.5 g/dL. (If lower, just check annually.)
3. Anemia, renal failure, hypercalcemia.
4. Lytic or sclerotic lesions on bone scan or skeletal survey Adapted from https://wchcmr.org/2015/11/11/a-crabby-diagnosi
10/
Take home:
- Neuropathy ▶️check SPEP and IFIX
- Paraproteinemia▶️check CBC, Cr, Ca, bone scan and refer to heme-onc for abnormalities
- For bonus life points, learn which heavy and light chains are associated with different neuropathy syndromes and malignancies!
11/
For a little more information, but a little less fun, see my @AANMember Neurobyte on this topic. learning.aan.com/diweb/catalog/… #MedEd #neurotwitter #neurotwitternetwork #futureneurologist @AANEMorg

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More from @zach_london

May 2
Ulnar neuropathy "at the wrist" is really in the hand. Guyon's canal is between the pisiform and hamate bones. It's close to the wrist, though.

The numbers in this image are the Zones where injuries can take place, and they each create a unique clinical picture. #tweetorial
1/6 Image
Let's count down from Zone 4. The superficial terminal branch, for practical purposes, is purely sensory, supplying the palmar aspect of the digits 4-5.

FYI: The dorsal ulnar cutaneous nerve (not shown) comes off in the forearm, supplies the backs of these fingers.
2/6 Image
Zone 3 is a motor branch that supplies the first dorsal interosseous. A lesion here could mimic the Split Hand in ALS, where the muscles on the side of the thumb are disproportionately weak.

I never met someone who was crucified, but I suppose that's one way to get this.
3/6 Image
Read 6 tweets
Oct 23, 2023
Saw another patient with sensory neuronopathy who had been misdiagnosed with functional neurological disorder.

What is sensory neuronopathy, and why do we keep missing it? A #tweetorial.
1/ Image
First order of business. Neuronopathy is not the same as neuropathy. There’s a NO right in the middle of it.

That helps you remember the answer to the question: Is this just numb feet in a patient with diabetes?
2/ Image
Where's the lesion?
Sensory neuronopathy is a syndrome of damage to the dorsal root ganglia. These little campers park up and down the spinal cord, so when they aren't working, the sensory loss or pain can be in the distribution of multiple roots.
3/ Image
Read 13 tweets
Jul 27, 2023
🧷🔥The Sensory Exam.🧊👈

Some neurologists just skip it. Compared to other parts of the exam, it's mired in subjectivity.

And slow! you could spend 10 minutes doing it and learn nothing.

But it can still be valuable, especially when done EFFICIENTLY. #tweetorial #neurology Image
In this🧵, we're talking about examining a patient who does NOT have a sensory deficit as their chief complaint.

The screening sensory exam in this situation should focus on things people might not bring up in the history. What on earth could that be? Image
The most common would be neuropathy.

Patients with numb feet may only complain of imbalance. Or pain. Or nothing.

So my screening sensory exam focuses on finding NUMB FEET. 👣

And here's a hot take: Neuropathy is almost always symmetric, so...
Read 15 tweets
Jul 16, 2023
I administered the Montreal Cognitive Assessment (MoCA) to the image-generating AI program DALL-E this morning. Thing really went off the rails.

Let's see how it did. 🧵 Image
"Do trails starting with 1-A-2-B etc."

Okay, that's not what we meant, but I see the confusion. 0 points. Image
"Copy a three dimensional cube."

Impressive, but that wasn't the assignment. 0 points. Image
Read 22 tweets
May 16, 2023
What do inkblots and puppies hugging teach us about the way the long tracts are organized in the spinal cord? Everything.

Behold, our favorite tracts in the cervical cord.

A = fibers to/from the arm
T = fibers to/from the trunk
L = fibers to/from the leg

#tweetorial
1/7 Image
Now let's just look at the arm fibers. For all three tracts, they are right up against the grey matter.

Want a #mnemonic to help you remember that?
2/7 Image
Well, some say the grey matter looks like a butterfly.

You love butterflies, so what do you do when we see one? You hug it.

That's why your arm fibers are right up in there. For butterfly hugs.
3/7 Image
Read 7 tweets
Mar 22, 2023
Not pointing fingers, but I think some of us are misusing the word "bulbar."

The Bulb is the archaic term for the medulla. So bulbar weakness refers only to muscles supplied by cranial nerves 9-12.

Let's play a game called "Is THIS bulbar weakness?"
1/🧵
Is THIS Bulbar Weakness?

Yes.

(The picture's not upside down... you're upside down.)

A lateral medullary stroke damages the nucleus ambiguus of CN X, leading to dysphagia, dysarthria, and dysphonia.
2/🧵
Is THIS Bulbar Weakness?

Yes!

This bulb is completely exhausted. (It needs to stop resting on its florals.)
3/🧵
Read 7 tweets

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