Guillain-Barré
Patient Presentation
C.F., an 18 year old male with no significant past medical history, awoke on a Saturday morning with numbness and tingling in his hands. Later that morning, he was noted to have a hoarse voice and was brought to an emergency room by his parents, only to be discharged home with no tests obtained or prescriptions given. Later that night, he awoke with shortness of breath and was brought back to the emergency room where he was intubated for airway protection. He was then transferred to another facility where he was treated for status asthmaticus and placed on Fentanyl® and propofol drips for sedation. Three days later, as the sedation was weaned, he was noted to have no movement and was not responding appropriately to stimuli; appearing paralyzed with cranial nerve palsy. He was then transferred to another facility that had a strong neurology service and could provide a higher level of care.
Differential List
Several different diagnoses that could be the causes of paralysis were discussed when the patient was admitted. These included botulism, myasthenia gravis, Lyme disease because the patient was noted to have a red induration on his right arm, or Guillain-Barre after it was noted he recently underwent a cycle of Augmentin® to treat an ear and sinus infection a few weeks prior.
Diagnosis
Several tests were performed to diagnose the problem; An MRI/MRA of the head and neck was obtained that was WNL. Lyme serologies were negative. The medical team also performed a caloric reflex test on the patient with results consistent with brain death. An EMG was obtained that showed AMSAN (Acute Motor and Sensory Axonal Neuropathy). A repeat lumbar puncture showed protein of 75 (elevated) in the CSF. These results plus consideration of presenting symptoms is what lead the neurologists to the diagnosis of Guillain-Barre.
Treatment
Initially, the patient was hypertensive and tachycardic, which was treated first with a labetalol infusion and was eventually replaced with labetalol 400mg PO TID. For the Guillain-Barre, the patient underwent six cycles of plasma exchange. Ten days after admission he had a tracheostomy placed for airway protection since he was not able to be weaned from the ventilator following several bronchoscopies for bilateral lower lobe infiltrates and pneumonia. Two weeks after admission he received a PEG tube for medications and enteral feedings. He remained on a Fentanyl® drip for pain which was eventually replaced with Neurontin® 1200 mg PO TID, amitriptyline 75mg PO QHS and oxycodone ER 160mg PO BID for his pain and neuropathies. Physical therapy and occupational therapy came to work with him four to six times per week as he began regaining strength and muscle so he could be discharged to a rehabilitation facility.
Outcomes
Seven weeks after admission, the patient was discharged to an acute rehabilitation facility. By that time, he was weakly moving all extremities at strength of 2/5 (only able to slide them on the mattress but could not lift them against gravity) and was able to nod and shake his head. He had been weaned to tolerating 40% trach collar (no ventilator support involved, only supplemental oxygen) 24 hours a day and was eating a regular diet with nectar thick liquids, no longer requiring use of his PEG tube. Approximately 4 months following discharge the patient returned to the facility for a follow up appointment when he was able to stand and walk short distances with the assistance of a walker, and his tracheostomy had been removed. About six months after discharge he was walking without assistance, climbing stairs, driving a car again and was planning to resume a full course load at college that fall.