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Interesting Case Discussion


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Here's a good case for those of you out there to think about....

 

HPI: 72 y/o right hand dominant male farmer presents to the Center with a 4 week history of severe unbearable right arm pain. He is unable to localize this pain, and states that it involves the entire arm. It seems to extend from the right side of his neck into his arm. He also complains of pain into the right jaw and face, and even extension into the anterior neck. Complains of significant balance issues, but feels that these have been present for several years, and cannot determine if they have worsened. Denies any history of trauma, infection, or prior surgery. States that in January, he had the sudden onset of weakness and numbness through his entire left body, including both upper and lower extremities. This is still present, but not as bad. He was evaluate locally at that time, and had a head CT, which his doctor said showed a "stroke, he said there some stuff in the white matter". After his symptoms started 3 weeks ago, he had a non contrast cervical MRI, and was told that he had a radiculopathy, but then there was confusion on that. He was started on methadone and vicodin which isn't helping the pain at all. Patient becomes concerned by lack of progress and presents to the ED who refers them to you. He states, "I can't live with this pain, I would cut my arm off if I could"

 

PMH/PSH: CVA in January 2014, Borderline diabetes, CAD, OSA, COPD, history of MI 10 years ago, HTN, Hyperlipidemia, no spinal surgery history.

 

Exam: Obese 72 year old male in mild distress, appears diaphoretic, in severe discomfort. A&O x 3, Mental status intact, affect normal. CN II-XII grossly intact, Weakness noted in finger extensors, first interosseous, and wrist extensors of -1 in RUE (our normal scale is 0-I know it's different) Reflexes are normal but did require significant reinforcement efforts, clonus is negative, toes are downgoing, hoffman's is positive. Sensory was intact to pinprick. Vibratory sense was diminished in RUE at -2 at both ulnar styloid and 1st MCP, and -1 at olecranon when compared to the LUE. Tinels and phalens are negative, ulnar tinels was negative. Wright's Test was negative. Gait was wide based, short strided, toe walking and heel walking are intact, heel/toe was -3, romberg was 0 to -1.  Neck had normal ROM, no tenderness, no deformity noted. Shoulder provocative maneuvers are negative. Pulses are normal, Skin without rashes.

 

MRI: non contrast image, no radiology report. Shows no evidence of compressive lesion. There are scattered degenerative changes with degenerative disc disease from C3 to C7, there is uncovertebral hypertrophy, and facet arthropathy noted throughout, worse at C5-T1. Mild multilevel neural foraminal stenosis, severe on the left at C5-6, but not impressive on right side. No central canal stenosis noted. However, at C2-3, and C3-4, there are cord signal changes in several locations without evidence of compression.

 

Alright, so let's hear it, what's your differential, what are the next tests you want?

 

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This would be one of my weak areas in medicine. Would an EMG study offer much at this point ?  I think some basic lab work is appropriate ESR/CBC. From an ID perspective I would investigate sexual activity and travel history. Lyme can present with radiculopathy, as can varied stages of syphilis. If at risk I would consider RPR or VDRL w FTA confirm and a LYME AB w WB confirm. Could the shoulder be the course of symptoms ? consider MRI shoulder if nothing else pans out ....  Given his age maybe a CHEM screen as well includes check of protein levels and calcium which may suggest disorders which can cause nerve damage or malignancy. 

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Why was a non contrast C Spine MRI done? (as opposed to contrast which would show a nerve root compression - if there is one...)

 

Skin color? temperature? any trophic changes? Pulses?

 

The degree of pain and the patient's inability to localize it could lean towards a "shoulder hand syndrome" - not uncommon after CVA

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Just some ideas:

 

It's best to take an organized approach

  • Vascular - higher likelihood given history

Claudication pain is possible.  He may have had a possible stroke.  Could be extensive arterial calcifications causing the pain.  Doesn't explain the neuro sx well though.  VTE or emboli also possible.  Aneurysms can also cause pain and neuro sx.  Stroke, carotid stenosis, etc.  EKG should be in base workup but not likely to reveal diagnosis.

 

  • Infectious - very unlikely

Syphilis is a possible cause of the neuro sx but unlikely to cause significant pain unless there's an aneurysm of some sort.  It's also statically unlikely.  Lyme's is more common but fits the sx even less.  Overall, infection is fairly unlikely given time course.  Prion disease is always possible, but he probably would not have survived 2 years.  (Typically 6 months or so).  Might get Lyme titer/RPR, but it's probably not necessary.

 

  • Traumatic - maybe

Could always consider syringomylia or spinal stenosis.  MRI not very suggestive.  No history of trauma.  Fully explore history.  Consider other causes of nerve compression.

 

  • Autoimmune - maybe

He's 72.  Pretest probability of autoimmune is rather low.  Reasonable to explore.  MS unlikely. GBS is possible but doesn't typically cause pain.  Whole grab bag of other demyelinating diseases.  ESR/CRP can help rule out autoimmune.  I would definitely get them both.  EMG may be further helpful after other causes explored.  I would always want to know if there are fasiculations and cranial nerve exam.

 

  • Metabolic - fairly unlikely

Diabetic neuropathy could fit.  Even a long history of very mild diabetes can cause neuropathy even if well controlled.  However, this is highly unlikely or the case wouldn't be interesting.

 

  • Idiopathic/iatrogenic - maybe if all other options explored

Really just means we don't know.  Fairly common actually.  Diagnosis of exclusion.  I would definitely review his medications for iatrogenic causes.

 

  • Neoplastic - low likelihood if imaging negative

Highly likely in this 72 year old.  Probably would have been detected on imaging however.

 

  • Congenital - highly unlikely

Not likely in the elderly

 

  • Other- Possible

ALS high on my differential though it typically doesn't cause pain.

 

 

Thoughts:

ALS high on my differential.  Vascular and autoimmune causes as well.  Would search other causes if those are ruled out.

 

Next step:

Await full MRI read or have a look myself. 

 

I suck at neuro.  If I actually saw the patient, I'm sure my differential would be larger.

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Thoracic outlet syndrome? - Was onset gradual or sudden?

High ischemic/cardiovascular risk is worrisome: Is an embolism impinging on a nerve plexus?  Is he anticoag'd?  I'd want to get a MRI brain

History of measles vacc?  

Would get a cbc, cmp, too.

 

I'd guess I would start with that before I start looking for zebras

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The hallmark in this history was the left sided numbness and weakness that occured back in January with the cord signal changes noted on the MRI. No spasticity on exam, and CN exam was normal.

 

We typically do not use gadolinium for spine MRI's if the suspicion is nerve root impingement/radiculopathy etc. However, we also have a neuro radiologist review every brain/spine MRI before the patient is allowed out of the room. If there is an indication for gadolinium we do it.

 

This was an outside MR, and not sure what the procedure was where this patient had it done.

 

My working differential was: tumor, infection, or demyelinating process (Transverse Myelitis, Neuromyelitis Opticans, Multiple Sclerosis, etc.) as you can get isolated extremity neuropathy with MS.

 

Got NMO antibodies, EMG, and MR with contrast of both cervical and thoracic spine. MR confirmed demyelinating disease in both cervical and thoracic spine. NMO antibodies negative.

 

Final diagnosis was MS. Started on gabapentin for pain control.

 

Follow up with Neurology confirmed this, pain still poorly controlled, primary progressive multiple sclerosis.

 

Interesting case. Remember, radiculopathy is not always radiculopathy.

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New onset MS in a 72 year old male with the predominant symptom severe pain?  Very, very atypical.

 

Did you find oligoclonal bands?  Was the ANA elevated?  Did a steroid burst improve symptoms?  Did you have more than one MRI showing migrating lesions?  Anything else to support or just the MRI?

 

Yeah, radiculopathy can do crazy things.  Cool case.

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Yep, LP with elevated OP, and elevated protein at 58 with 11 oligoclonal bands. ANA, CMV, RPR, EBV, and NMO all negative. TSH normal. Sed rate WNL.

 

Both outside MR and internal MR with Gad showed lesions. Our Neurologists decided against steroid burst as primary complaint was neuropathic pain.

 

Very cool case.

 

Mike

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