Interesting case

[MitchLevi]

Thought this was an interesting case, and figured I'd post it.

Zero pain complaints, just weakness.

Early 60s F professor, husband is a surgeon. I got an email from him a few weeks ago asking if I'd see his wife who has lower extremity weakness. Severe food poisoning (2009) with weakness, myalgias, rhabdo, progressive LE wasting and lower extremity weakness. Saw a local PMR guy who has a great reputation maybe months after this incident or possibly a year later or so, and diagnosed with a Trendelenburg gait, lower extremity weakness and atrophy which was treated with PT (helpful).

Over the next several years, she spent a lot of timetime on a treadmill (4 miles per day), but due to LE weakness and falls she began using the handrails to the extent she developed a Dupuytren's contracture. In 02/2021 she began blood flow restriction + leg press therapy (some degree of proximal strength benefit - I'll call it modest at best, but they feel it has been great). Currently using BFR without leg press. Tried/failed e-stim (increased fall rate). She feels she supinates on the right while walking.

Cranial nerve IV palsy in the 1980s - Only real PMH.

I walked her down the hallway - Trendelenburg with near hip hiking bilaterally. She barely clears her toes walking, and step up onto a stool she is clearly hiking the hip for toe clearance. Strength is 1/5 dorsiflexion bilaterally, 5/5 plantarflexion, 5/5 knee extension, and hip flexion 4/5. Absent DTR. Tibialis anterior atrophy relative to the gastroc. SLR negative. Tone is diminished in the legs globally, including the pelvic muscles and glutes.

I'm getting an EMG.

Guillain-Barre missed Dx in '09? Severe food poisoning with rhabdo.

With a weird cranial nerve palsy in the 80s I thought briefly about MS, but this sounds like GBS no?

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[lobelsteve]

Add mitochondrial disease to DD.

 

[willabeast]

After effects of Botulism?

 

[SSdoc33]

hope you know the EMGer. this could go a lot of ways, and if they dont know what they are doing, you may not get a good answer.

this does look like an old GBS, but even it is was, you are way out of the treatment window. she may have had a run of the mill peripheral neuropathy along with her incident in 2009.

it may not hurt to image the L-spine if not already done.

AFOs? lofstrand crutches?

 

[runfastnow]

Need a good EMGer for sure.

First guess would be CIDP or similar process. If patient open to it would offer carbon AFO on most symptomatic side. Unfortunately probably not much to do outside of current PT/HEP. Maybe some sort of immunomodulation/therapy.

 

[drrosenrosen]

Need a good EMGer for sure.

First guess would be CIDP or similar process. If patient open to it would offer carbon AFO on most symptomatic side. Unfortunately probably not much to do outside of current PT/HEP. Maybe some sort of immunomodulation/therapy

If CIDP, can respond pretty well to IVIG and/or steroids. I just saw a pt yesterday with this diagnosis, proximal and distal weakness with gait disturbance

 

[Ducttape]

neurology should have seen this patient.

what does the MRI show? what has neurology thought? sounds like transverse myelitis.

 

[SSdoc33]

neurology should have seen this patient.

what does the MRI show? what has neurology thought? sounds like transverse myelitis.

you would see increased reflexes and spasticity with TM

 

[ErrantWhatever]

Poor gal. Agree w much of the above. Sounds peripheral>>central given your exam. Has ESR ever been checked? Would also consider myopathies like PMR or statin myopathy if applicable. Could also consider one of the Charcot Marie Tooth types (CMT2?).

EMG should be informative if in the right hands. Good luck!

 

[MitchLevi]

neurology should have seen this patient.

what does the MRI show? what has neurology thought? sounds like transverse myelitis.

No MRI ever done.

 

[Dr. Ice]

Neurology will do their own emg and muscle biopsy likely. This might be a myopathy

 

[SSdoc33]

Neurology will do their own emg and muscle biopsy likely. This might be a myopathy

Myopathies are proximal weakness, neuropathies are distal. You can rule pit a spinal problem and make a better diagnosis, but with the given info, i dont see this getting much better

 

[cameroncarter]

I agree with above re: peripheral > central and possible GBS / CIDP. That being said, I’d still get an MRI brain and pan-spine in addition to the EMG.

 

[clubdeac]

Myopathies are proximal weakness, neuropathies are distal. You can rule pit a spinal problem and make a better diagnosis, but with the given info, i dont see this getting much better

not always... IBM has more distal weakness as well

 

[epidural man]

Thank goodness for PM&R docs.

Just tell me where I should put the needle.

 

[MitchLevi]

EMG tomorrow. I'm pretty sure it's GBS and not CIDP. Abrupt onset.

Can't believe this passed through the hands of so many other guys, especially given the fact her husband is a surgeon.

 

[lobelsteve]

Painless weakness.

ALS over GBS. 85% reported back pain in GBS. Also known as “pervasive”.

 

[GoBeers]

EMG tomorrow. I'm pretty sure it's GBS and not CIDP. Abrupt onset.

Can't believe this passed through the hands of so many other guys, especially given the fact her husband is a surgeon.

do you have many GBS patients?
pain is essentially neuropathic in etiology so do you typically run the list on neuropathics ? really sad cases as they seem pretty miserable when recovery doesn't seem likely

 

[specepic]

I would send this to academic/tertiary care Neurology dept

 

[MitchLevi]

Painless weakness.

ALS over GBS. 85% reported back pain in GBS. Also known as “pervasive”.

Sx began 2009.

 

[MitchLevi]

do you have many GBS patients?
pain is essentially neuropathic in etiology so do you typically run the list on neuropathics ? really sad cases as they seem pretty miserable when recovery doesn't seem likely

We'll see in the next week or so.

 

[lobelsteve]

Sx began 2009.

Frontiers | Differentiating Slowly Progressive Subtype of Lower Limb Onset ALS From Typical ALS Depends on the Time of Disease Progression and Phenotype

BackgroundFlail leg syndrome (FLS) is a regional variant of amyotrophic lateral sclerosis (ALS) with the characteristics of slow progression and the symptoms...

www.frontiersin.org

Really slow ALS

 

[SSdoc33]

Its just old GBS

 

[KateFan]

Add mitochondrial disease to DD.

Niacin Cures Systemic NAD+ Deficiency and Improves Muscle Performance in Adult-Onset Mitochondrial Myopathy - PubMed

NAD⁺ is a redox-active metabolite, the depletion of which has been proposed to promote aging and degenerative diseases in rodents. However, whether NAD⁺ depletion occurs in patients with degenerative disorders and whether NAD⁺ repletion improves their symptoms...

pubmed.ncbi.nlm.nih.gov

Wonder if they'd add NA to the treatment?

 

[Ducttape]

do you have many GBS patients?
pain is essentially neuropathic in etiology so do you typically run the list on neuropathics ? really sad cases as they seem pretty miserable when recovery doesn't seem likely

my understanding is that 70% recover...

 

[MitchLevi]

my understanding is that 70% recover...

Never heard that

 

[SSdoc33]

my understanding is that 70% recover...

But once you've had it for a decade, 0% recover

 

[clubdeac]

yeah I thought GBS had a high recovery rate. when's neurology going to see her? 6 months? that's what it is around here. crazy impossible to get them in with neuro

 

[runfastnow]

GBS - 80% able to walk independently at 12 months (which doesn't mean perfect), 50% back to baseline at 12 months, 10% severe motor dysfunction forever.
Kissel JT, Cornblath DR, Mendell JR. Guillain-Barre syndrome. In: Diagnosis and Management of Peripheral Nerve Disorders

CIDP - ~60%respond to immunotherapy to some extent, 30% achieve cure/remission, 10% no response to anything.
Chronic inflammatory demyelinating polyneuropathy disease activity status: recommendations for clinical research standards and use in clinical practice.
Gorson KC

 

[Ducttape]

yeah, thats what i found in search just now.

Guillain-Barré Syndrome

Guillain-Barré syndrome (GBS) (pronounced Ghee-yan Bah-ray) is a rare neurological disorder in which a person’s immune system mistakenly attacks part of their peripheral nervous system—the network of nerves that carries signals from the brain and spinal cord to the rest of the body.

www.ninds.nih.gov

Fortunately, 70% of people with GBS eventually experience full recovery.

What Is Guillain-Barré Syndrome?

Guillain-Barré syndrome is a rare autoimmune disorder. Educate yourself about its causes, symptoms, and treatment methodologies.

www.webmd.com

Most people recover, even those with severe cases. In fact, 85% of people with GBS make a full recovery within 6 to 12 months. Once you get better, the chance of it returning is very small.

70-80% complete recovery at 3 years.

 

[Ligament]

Can't believe husband is a surgeon and this has been around since 2009 and has never had even the most basic of workups? Something very fishy going on here. Get to an academic neurology department for a full w/u immediately.

 

[MitchLevi]

Can't believe husband is a surgeon and this has been around since 2009 and has never had even the most basic of workups? Something very fishy going on here. Get to an academic neurology department for a full w/u immediately.

I know.

This also highlights the fact physical therapists occasionally say stupid S that no one wants to debate bc they don't want to look stupid.

So much MSK goes that direction.

She told me...Her husband agreed with this, that her therapist had explained to both of them that her "glute med doesn't fire." For years that's been the issue.

I looked directly at him and said, "Does that not sound ridiculous to you? What process could occur that would selectively knock off her glute med exclusively?"

So I stood her and walked her down the hallway and showed him the glaring drop foot she's got...

I will be seeing her Tuesday of this week, and I have no doubt I'll be sending her out for an exhaustive workup.

 

[Ducttape]

may i ask why you didnt at the first appointment? not that this was an emergency, but i would have punted this to neuro right from the onset..

 

[SSdoc33]

may i ask why you didnt at the first appointment? not that this was an emergency, but i would have punted this to neuro right from the onset..

Thats bc you are not a physiatrist

 

[MitchLevi]

may i ask why you didnt at the first appointment? not that this was an emergency, but i would have punted this to neuro right from the onset..

That will take 6 months to get in, and this is non-emergency. Nothing anyone can say or do will change the clinical presentation of this case. Tomorrow we're gonna discuss AFO most likely. I brought it up last visit briefly. That's something I guess.