Difficulty Walking

Chief Complaint: A 57 year-old male presents with 5 days of difficulty walking.

DOMAIN A: DATA ACQUISITION

+ What are the key components of this patient’s history and physical examination?

Relevant History:

How did this begin? Onset? Context of symptoms?
Describe the walking difficulty in more detail, what it feels like, what particularly is difficult about walking?
Was there trauma/injury?
Is there numbness/tingling/weakness/bowel or bladder deficits?
Is there pain? Specifically neck or back pain?
Any falls recently?
Any history of diabetes?
Any recent infections or illnesses?
Any fever, chills, shortness of breath, nausea, vomiting, diarrhea, palpitations, rashes, or vision changes?
Any recent weight changes?
Past Medical/Surgical History?
Family History?
Social History? Work status?
Functional History and current functional status? Any assistive devices?

Relevant Physical Examination:

Vital signs
Gait analysis
Cranial nerve examination
Manual muscle testing, reflexes, sensation testing of all four limbs
Special testing as indicated by history/exam in addition to Babinski, Hoffman, straight leg raise, and slump sit tests.

DOMAIN B: PROBLEM SOLVING

The patient is a 57 year-old male, previously independent, with a history of hypertension and type 2 diabetes mellitus who presents with the gradual onset of 5 days of progressive difficulty walking. He denies falls or injury, but complains of back pain. He describes the difficulty as leg heaviness and unsteadiness, and has been resorting to using a walker for stability. He denies fever or chills, but feels like he might have had diarrhea 1 month ago. On exam he demonstrates intact strength except for 1/5 strength of plantarflexion, dorsiflexion, and great toe extension bilaterally. Reflexes are absent in bilateral achilles and 1+ in the patellae.

+ What is your differential diagnosis for this patient?

Guillain-Barre Syndrome (Acute Inflammatory Demyelinating Polyradiculopathy - AIDP), Chronic Inflammatory Demyelinating Polyneuropathy (CIDP), lumbosacral radiculopathy, diabetic radiculoplexopathy, polymyositis, Lambert-Eaton Myasthenic Syndrome (LEMS), myasthenia gravis, botulism, cervical myelopathy, sciatic neuropathy, lumbosacral plexopathy, conversion disorder, transverse myelitis, multiple sclerosis, mononeuritis multiplex, peripheral polyneuropathy, amyotrophic lateral sclerosis (ALS), PLS.

+ What are your next steps in the workup of this problem?

CBC, CMP, ESR, CRP
Lumbar puncture with CSF analysis
EMG/nerve conduction studies of bilateral lower extremities
MRI brain/CTL spine

+ The results of your workup are noted below:

Laboratory workup is grossly unremarkable.
Lumbar puncture with CSF analysis reveals increased CSF protein with normal white blood count (WBC).
EMG/nerve conduction studies of bilateral lower limbs are normal.
MRI brain and cervicothoracic spine is normal. MRI lumbar spine reveals increased signal in the lumbosacral nerve roots.

+ What is the most likely diagnosis?

Guillain-Barre Syndrome (AIDP).

+ Challenge Question #1

How do you explain the normal EMG/NCS findings in this patient?

+ Challenge Answer #1

With only 5 days of symptoms, it is likely too early for AIDP to be manifested on EMG/NCS at this point in time. Ideally we would repeat the EMG/NCS at roughly 2 weeks of symptoms for maximal diagnostic utility.

+ Challenge Question #2

Describe the electrodiagnostic findings you would expect to see in a case of demyelinating Guillain-Barre Syndrome (AIDP).

+ Challenge Answer #2

The earliest expected findings would be prolonged latency of F-waves. I would also expect to see prolonged distal latencies, conduction block at non-entrapment sites, and abnormal temporal dispersion. Needle EMG exam would likely reveal decreased recruitment of motor units.

+ Explain the etiology and mechanism of Guillain-Barre Syndrome (AIDP).

GBS/AIDP is the result of a bacterial or viral infection leading to the body producing an immune response against the foreign pathogen. Certain proteins on the foreign pathogen can mimic proteins found on peripheral nerve myelin or axons, leading to the body destroying these self proteins in the process of eradicating the infection. With myelin and potentially even axons destroyed, the peripheral nerves do not function properly, and weakness is developed.

DOMAIN C: PATIENT MANAGEMENT

+ How would you manage this patient?

I would recommend the patient be admitted to the hospital with stat neurology consult placed. ICU admission should be considered depending on the patient’s autonomic and cardiorespiratory status. I would recommend stat IVIG/plasmapheresis to help halt circulating antibodies from continuing to destroy myelin and/or axons. Supportive cardiorespiratory care as indicated by the patient’s clinical status. I would recommend physical and occupational therapy consults; ultimately the patient may require inpatient rehabilitation admission once medically stable. For pain I would advise acetaminophen, NSAIDs, potentially antineuropathic pain medication, and if severe enough, opioid pain medication. Repeat EMG/NCS should be performed in approximately 1 week.

DOMAIN D: SYSTEMS-BASED PRACTICE

+ What specific steps would you take to facilitate a hospital admission for this patient?

I would call the hospital directly and ask to perform a direct admission from clinic. I would ask to speak with the accepting physician and relay all pertinent patient information to facilitate a swift and safe transfer of care. If admission is not granted for any reason, I would recommend the patient present to the emergency department immediately for evaluation, and in that case I would call the ED directly to relay pertinent information and my recommendations for care. I would ensure that all questions are answered from all parties and that the patient is in agreement with the plan. I would educate the patient on rationale for all of my actions, and about the disease process and treatment options.

DOMAIN E: INTERPERSONAL AND COMMUNICATION SKILLS

You receive a call a few hours later by the internal medicine physician covering this patient. Pretend that I am this physician.

Internal medicine physician: “Why didn’t you start steroids? This is an inflammatory process. Every delay of care worsens his prognosis for walking again.”

+ Your response:

I would be delighted to discuss this case with you. Thank you so much for caring for this unfortunate gentleman. After receiving the diagnostic results, GBS/AIDP became the most likely diagnosis, and you are absolutely correct that immediate action is paramount to maintaining the best prognosis for functional recovery in this patient, which is why I immediately recommended hospital admission for stat IVIG/plasmapheresis. May I ask your further thoughts on this case, and what your understanding of the disease process is?

+ The internal medicine physician's response:

“Yeah, of course, it’s an inflammatory process, and you wasted time that could have been better served with an initial steroid bolus while he awaits admission. These patients do better with steroids, and you should know that. Why didn’t you start something?”

+ Your response:

This is a great question! The literature does not support the use of corticosteroids in GBS/AIDP. They have not been shown to provide functional benefit or treat the disease process. In CIDP, a disease related to GBS/AIDP, corticosteroids actually do serve a purpose and are indicated. However, in this patient’s case, steroids are unfortunately not indicated, and prompt removal of circulating antibodies via IVIG/plasmapheresis is of prime importance. I hope this helps to answer your question. I would otherwise be more than happy to further explain my rationale. Does that treatment plan and rationale sound all right, as I have outlined?

Note: Do not be adversarial; educate and align yourself with this treating physician, and ensure that proper treatment is being followed at the same time. Unnecessary corticosteroids would not be a harmless intervention, and should be minimized if possible.