Guillain-Barre Syndrome — Clinical Case

DCE Long Case

Patient brief (provided to trainee)

Patient: Mr Robert Davies, 54 years old, mechanic. [1]

Presenting illness (3 months ago): Four days of progressive, symmetric ascending weakness beginning in the feet, following a confirmed Campylobacter jejumi enteritis two weeks earlier. He reached nadir at day 7 (quadriplegia, grade 5, ventilated). He received IVIG 0.4 g/kg/day for 5 days. He was intubated for 5 weeks and a tracheostomy was formed at week 3. He is now being reviewed in the physician rehabilitation clinic; the tracheostomy is downsized and capped during the day, and he is weaning from the ventilator overnight. [1]

Past history: Hypertension, ex-smoker (30 pack-years), no prior neuromuscular disease. [1]

Current medications: Enalapril 10 mg daily, enoxaparin 40 mg subcutaneously daily, gabapentin 600 mg three times daily (for neuropathic pain), amitriptyline 25 mg at night, sertraline 50 mg daily (started week 4 for depression), a proton pump inhibitor, and regular paracetamol. [1]

Current functional status: Sits supported in a wheelchair; can stand with a hoist and two people but cannot step. Can feed himself with adapted cutlery. Has a suprapubic catheter for persistent urinary retention. Speech is now audible with the tracheostomy capped. [1]

Examination findings (trainee elicits):

• Alert, oriented, cooperative. Speech is dysphonic but fluent.
• Bilateral partial lower motor neuron facial weakness (improving). Eye movements full. Bulbar function intact with the cuff down.
• Tone reduced in all four limbs. Power: arms 3 out of 5 proximally and 2 out of 5 distally; legs 2 out of 5 proximally and trace movement at the feet. Symmetric.
• Reflexes absent in all four limbs. Plantars downgoing.
• Sensation reduced to pinprick and light touch in a glove-and-stocking distribution; joint position sense impaired at the toes, intact at the fingers.
• Calf tenderness and allodynia over the feet. No pressure ulcers.
• Tracheostomy in situ, capped during the day. Chest clear. Spontaneous respiratory rate 18, oxygen saturation 96 percent on room air. [1]

Investigations (current):

• Nerve conduction studies (2 weeks ago): severe axonal loss superimposed on demyelination (markedly reduced CMAP amplitudes, slowed conduction) — consistent with AIDP with secondary axonal degeneration, a marker of slow recovery.
• Renal function and full blood count normal. Enoxaparin therapeutic. [1]

Candidate's structured presentation (model)

Opening statement: [1]

"Mr Davies is a 54-year-old mechanic who, three months ago, developed severe Guillain-Barre syndrome, AIDP variant, following confirmed Campylobacter jejumi enteritis. He reached nadir at day 7 with quadriplegia (grade 5) and required mechanical ventilation for five weeks with a tracheostomy at week three. He received a full course of intravenous immunoglobulin. He is now weaning from the ventilator overnight, his tracheostomy is capped during the day, and he is reviewed in the rehabilitation clinic for ongoing management. [1]

His main problems are:

1. Residual severe quadriparesis (arms 3 out of 5, legs 2 out of 5) with secondary axonal loss on nerve conduction studies, indicating a slow recovery trajectory — intensive neurological rehabilitation required
2. Tracheostomy in situ, weaning from ventilatory support — respiratory and ENT input, decannulation planning
3. Persistent neuropathic pain and allodynia — optimise analgesia
4. Persistent urinary retention with a suprapubic catheter — urological review and trial of catheter-free management
5. Depression (on sertraline) and the psychological aftermath of prolonged intensive care — psychological support and screening for post-traumatic stress
6. Secondary prevention and general medical care — VTE prophylaxis while immobile, blood pressure control, smoking cessation, nutrition, skin and contracture prevention" [1]

Management plan: [1]

1. Neurological rehabilitation: Intensive multidisciplinary inpatient rehabilitation — physiotherapy for strength, transfers, gait re-education and prevention of contractures; occupational therapy for activities of daily living and home modification; splinting of the wrists and ankles to prevent contractures. The nerve conduction study showing secondary axonal loss predicts slow recovery (axonal regrowth at around 1 mm per day); I would set realistic functional goals over 12 to 24 months and measure progress with the GBS disability scale and the MRC sum score.
2. Respiratory and tracheostomy: Continue collaborative weaning with the intensive care and respiratory teams — progressively longer periods off the ventilator overnight, tracheostomy downsizing, cuff deflation, and a decannulation pathway once he can protect his airway and clear secretions. Monitor for sleep-disordered breathing.
3. Pain: Titrate gabapentin (with renal-function-appropriate dosing) and consider pregabalin; continue amitriptyline for its neuropathic and sleep benefits; add short-course opioids for flares but avoid pure opioid monotherapy. Reassess regularly.
4. Urology: Arrange urodynamics and a trial without catheter; if retention persists, consider clean intermittent self-catheterisation or a long-term plan. Exclude a urinary tract infection.
5. Psychological: Continue sertraline, screen for post-traumatic stress disorder and anxiety with a validated tool, and arrange clinical psychology input; involve his family in his rehabilitation goals.
6. Medical: Continue enoxaparin while he is immobile and transition to oral anticoagulation only if there is another indication; optimise blood pressure; reinforce smoking cessation; ensure pressure care, nutrition, and a bowel regime.
7. Follow-up: Regular physician review to monitor recovery, watch for treatment-related fluctuation or progression beyond eight weeks (which would re-diagnose him as acute-onset CIDP and move to corticosteroids), and coordinate the transition from inpatient rehabilitation to community supports and a return-to-work plan. [1]

Examiner discussion questions

Q: "His nerve conduction studies show secondary axonal loss. What does that mean for his prognosis?" [1]

"Axonal loss superimposed on demyelination predicts a slower and less complete recovery than pure demyelinating AIDP. Demyelination remyelinates over weeks to months, but axonal degeneration requires axonal regrowth at around one millimetre per day, or about one inch per month, so recovery from the axonal component can take one to two years and may be incomplete. His Campylobacter antecedent and his severe nadir (ventilated, grade 5) are adverse prognostic factors that the Erasmus GBS Outcome Score captures. I would set realistic expectations: a meaningful chance of independent ambulation and self-care, but a likely prolonged rehabilitation and a possibility of residual weakness and fatigue. I would measure his trajectory with serial MRC sum scores and GBS disability grades." [1]

Q: "It is now three months. Could this be CIDP rather than GBS?" [1]

"The time course is the key discriminator. Guillain-Barre syndrome reaches its nadir within four weeks and then plateaus and improves. CIDP progresses or relapses beyond eight weeks. Mr Davies reached a clear nadir at day seven and has been improving since, which is monophasic GBS — not CIDP. I would only re-diagnose him as acute-onset CIDP if he developed a clear secondary deterioration or continued progression beyond eight weeks, at which point the management would shift to corticosteroids (which are effective in CIDP but not in GBS). At three months with a improving trajectory, he remains a GBS patient in the recovery phase. The Hughes Cochrane review confirms steroids have no role in GBS recovery." [1]

Q: "He is still on enoxaparin. When do you stop it?" [1]

"The risk of venous thromboembolism in Guillain-Barre syndrome is high — around 30 percent without prophylaxis — and the risk persists while the patient is significantly immobile. I would continue pharmacological prophylaxis with low-molecular-weight heparin plus intermittent pneumatic compression while he is unable to mobilise independently, and transition off it once he is ambulating and his risk falls. I would not transition to long-term oral anticoagulation unless he develops a separate indication, such as atrial fibrillation or a confirmed thromboembolic event." [1]

Q: "How would you address his return to work as a mechanic?" [1]

"Honesty and planning. Given his axonal loss and slow recovery, a return to manual mechanical work within the next six to twelve months is unlikely. I would involve an occupational physician and a vocational rehabilitation service early, focus his therapy on the hand and upper-limb strength he needs for fine motor tasks, and explore workplace modifications, graduated return, or retraining options. Returning to work is a powerful driver of psychological recovery, so I would frame the conversation positively and set staged, achievable goals while being transparent about the timeline." [1]

DCE Short Case — Neurological Examination

Instruction

"Examine this patient's neurological system. They had Guillain-Barre syndrome three months ago. You have 7 minutes for examination and 8 minutes for discussion." [1]

Key signs the patient demonstrates

• Flaccid, symmetric weakness — worse in the legs than the arms, distal worse than proximal.
• Areflexia — reflexes absent or markedly reduced in all four limbs.
• Downgoing (flexor) plantar responses — a lower motor neuron pattern.
• Bilateral partial lower motor neuron facial weakness — improving but still present.
• Glove-and-stocking sensory loss with impaired joint position sense at the toes.
• Calf tenderness and allodynia — residual neuropathic pain. [1]

Systematic examination routine

1. Observe from the end of the bed: wheelchair posture, tracheostomy, accessory muscle use, ability to speak.
2. Bedside respiratory: state you would check forced vital capacity and inspiratory pressures first, and assess the tracheostomy and cough.
3. Cranial nerves: facial power (bilateral lower motor neuron pattern), eye movements, bulbar function with the cuff down, tongue and palate.
4. Motor: tone (flaccid), power (symmetric, legs worse than arms, distal worse early), and reflexes (the key finding — areflexia).
5. Sensory: pinprick, light touch, joint position sense, vibration.
6. Coordination: limited by weakness; test what is possible.
7. Gait: if able — assess with assistance, looking for a steppage or stamping gait. [1]

Presentation template

"I examined Mr Davies's neurological system. He is alert and cooperative, seated in a wheelchair, with a capped tracheostomy. He speaks fluently but quietly. [1]

On cranial nerve examination there is bilateral partial lower motor neuron facial weakness, with reduced eye closure and a symmetrical smile. Eye movements are full and bulbar function is intact with the cuff down. [1]

In the upper limbs, tone is reduced. Power is 3 out of 5 proximally and 2 out of 5 distally, symmetric. In the lower limbs, tone is reduced. Power is 2 out of 5 proximally with trace movement at the feet, symmetric. Reflexes are absent in all four limbs. The plantar responses are downgoing. [1]

Sensation is reduced to pinprick and light touch in a glove-and-stocking distribution, and joint position sense is impaired at the toes but intact at the fingers. There is calf tenderness and allodynia over the feet. Coordination is limited by weakness. [1]

In summary, these findings — symmetric flaccid quadriparesis with areflexia, downgoing plantars, bilateral lower motor neuron facial weakness, and glove-and-stocking sensory loss — are consistent with a recovering Guillain-Barre syndrome, three months after a severe AIDP presentation. The axonal component predicts a slow but ongoing recovery." [1]

Discussion questions

Q: "Localise the lesion." [1]

"The pattern is that of a polyradiculoneuropathy — symmetric, flaccid, areflexic weakness involving all four limbs and the lower motor neuron portion of the facial nerves, with distal-predominant sensory loss. The lesion is in the peripheral nerves and nerve roots. The downgoing plantars confirm that the corticospinal (upper motor neuron) pathway is intact, localising the problem below the anterior horn cell. The bilateral facial nerve involvement places the facial lesion at the lower motor neuron (facial nucleus or nerve) level, consistent with a peripheral polyneuropathy rather than a central process." [1]

Q: "How would you distinguish residual GBS recovery from critical illness neuropathy?" [1]

"This can be difficult because both produce axonal loss and weakness after a prolonged intensive care stay. The discriminating features favouring residual Guillain-Barre syndrome are the clear antecedent Campylobacter illness, the documented CSF albuminocytologic dissociation and demyelinating nerve conduction study at presentation, and the symmetric ascending pattern at onset. Critical illness neuropathy and myopathy typically arise in the context of sepsis, multi-organ failure and prolonged neuromuscular blockade or steroid use, and produce a predominantly axonal, sensorimotor picture that is hard to separate electrophysiologically from axonal GBS. In practice the two coexist and contribute to the slow recovery. I would base the distinction on the history and the early electrophysiology, and focus the management on intensive rehabilitation, which benefits both." [1]

Q: "What is the role of further immunotherapy at three months?" [1]

"None, unless the diagnosis changes. Guillain-Barre syndrome is monophasic and the immunoglobulin he received acutely is the only disease-modifying therapy indicated. Further immunotherapy would only be appropriate if he developed a treatment-related fluctuation (a relapse within two months, re-treated with a second IVIG course) or if he progressed beyond eight weeks, which would re-diagnose him as acute-onset CIDP and prompt corticosteroids. At three months with an improving trajectory, he needs rehabilitation and medical support, not more immunotherapy." [1]