MS Case Studies 2–5

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Headshot of Dawn Elise DeWitt, MD, MSc, CMedEd, MACP, FRACP, FRCP-London · Senior Associate Dean, Collaboration for InterProfessional Health Education Research & Scholarship (CIPHERS)
Dawn Elise DeWitt
MD, MSc, CMedEd, MACP, FRACP, FRCP-London · Senior Associate Dean, Collaboration for InterProfessional Health Education Research & Scholarship (CIPHERS)
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Table of Contents

Case study 2: Mariah—Where is the lesion?

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A 32-year-old woman presents with right-leg weakness and has to use a stick for support. She also has numbness in the left foot and leg. She has been well, but had a measles vaccine 2 weeks ago from her GP. Strength in her major leg muscles is 3+/5 on the right and 5/5 on the left. Light touch is normal bilaterally. Pain sensation is reduced on the left. Joint position is absent in the right great toe. She has hyperreflexia and an upgoing toe on the right.

Question

Brown-Séquard Syndrome

 

  • Hemisection of the spinal cord.
  • Ipsilateral.
    • Weakness.
    • UMN signs.
    • Loss of position sense.
  • Contralateral loss of pain and temperature.
  • Light touch sensation usually intact.
Researchgate.net. CC BY 3.0.

Case study 3: Brad—Hands tingling

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A 24-year-old University rowing captain was fit and healthy until last night when he noticed some vague tingling in his hands. He attributed it to rowing in the cold, but today his legs feel weak, and he could barely turn the “rather stiff” door handle to get out of his apartment. He is a muscular young man with intact sensation and 4-/5 strength in all major lower-extremity muscle groups. His hands, wrists, and biceps and triceps, and shoulder strength are normal.

Question

Guillain-Barré syndrome (GBS)

  • Respiratory failure is the do not miss life-threatening complication of this condition.

  • Progressive rapid ascending demyelinating polyneuropathy.

    • Resp or GI infxn triggers T-cell attack.

    • DDx: Botulism, West Nile, myasthenia, Lyme, tick paralysis and others. Note Miller-Fisher GBS variant (GQ1b ganglioside antibodies + CN findings, ataxia).

  • Worsens over 2–4 weeks: Resp failure is biggest risk.
  • Recovery usually spontaneous.
  • May have paresthesias, but sensation is usually normal; diffuse arreflexia is common.
  • Treatment is IVIG (or plasmapheresis)—avoid steroids!
  • 80% are ambulatory at 6 months; relapse in 6%.

Case study 4: Daniel—Cook got burned

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A 33-year-old man presents with 3 burns on his upper arms. He works nights as a cook at a local fast-food restaurant and says he gets burned because he can’t feel it when he gets splashed by hot oil while cooking. Exam shows loss of pain and temperature sensation over the arms and shoulders bilaterally with some involvement over his upper chest/back.

Question

In the central cord (in the neck).

Question

The most likely diagnosis is syringomyelia. You should also consider MS in this man. Hemorrhage or tumor affecting the central spiral cord should also be considered.


Syringomyelia

 

UpToDate, 2008.

 

Central cord lesion caused by CSF collection.

Usual onset in 3rd–4th decade; men > women; slow progression.

Loss of pain/temperature via disruption of the decussate spinothalamic fibers (dissociated sensory loss).

Extension to posterior columns loss of position and vibratory sensation in the feet.

Extension to anterior horns (motor neurons) hand amyotrophy with claw hands and muscle wasting of hands arms.

Question

  • If he had extension to the posterior columns, he would develop loss of position and vibratory sensation in the feet.
  • If he developed extension to the anterior horns, where the motor neurons are located, he would develop hand symptoms, including muscle wasting and contractures.

Case study 5: Cynthia—Difficulty swallowing

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A 62-year-old professor of Family Medicine with a 2-year history of pre-diabetes confides that she is having difficulty with slurred speech that is embarrassing in her high-profile academic job. She has noticed that her gait is a little unsteady, and her right leg feels a bit weak when she climbs stairs. This started a couple months ago and has gradually gotten worse.
ROS reveals one episode of meat getting stuck “high up” in her esophagus—it took her a while and a lot of water to get it down, but she attributed it to long-standing reflux and taking PPIs. Her only other medical issue is being overweight with “pre-diabetes” diagnosed 5 years ago, but she lost 10 lbs., and her glucose has remained in the pre-diabetes range. She drinks 1–2 glasses of wine most evenings but specifically does not binge drink and uses no other drugs/substances except vitamin D.

Unpack the case

The timing is progressive over months, making it unlikely to be a stroke. While she has pre-diabetes, it would be highly unusual to have these symptoms as complications of diabetes.

These symptoms could be described as:

  • Speech “Bulbar.”
  • Swallowing “Bulbar.”
  • Lower-extremity weakness (distinguish LMN from UMN/central with testing).

Question

ALS

  • Degenerative disease of motor neurons, leading to progressive weakness, atrophy, death.
  • Upper-motor neuron (UMN) plus lower-motor neuron (LMN).
  • Bulbar: Dysarthria and dysphagia.
  • Weak extremities: Often isolated extremity weakness at presentation.
  • Frontotemporal dementia in 50% of patients.
  • Differentiate from MS: Oculomotor palsy, incontinence, and tremor rare in ALS but common in MS.
  • DX: Both UMN + LMN and EMG evidence of LMN signs in at least 2 or more ALS regions.
  • DDx is Multifocal motor neuropathy (no UMN), Cervical cord compression (LMN @ level and some UMN below that), vitamin B12 deficiency, copper deficiency. Exclude Lyme (can be focal), hyperparathyroidism, and thyrotoxicosis.

Image credits

Unless otherwise noted, images are from Adobe Stock.