Benjamin M. Greenberg, MD


Medscape General Medicine. 2003;5(2) 

In This Article

Case Presentation

A mother brought her 4 1/2-year-old boy to the emergency room, after noticing him "limping."

The night before this, his parents had observed him favoring his left leg and were concerned that he had injured himself while jumping on his bed. But by the next morning the mother noticed that the boy was "wobbly" when he walked, "as if he were drunk." At the outside emergency room, lower-extremity and hip x-rays revealed no abnormalities and the patient was released.

Upon awakening the following morning, the parents found their son drooling and too weak to get out of bed. They returned to the emergency room, where an exam noted weakness in upper and lower extremities, pooling of secretions, areflexia, and a severely reduced forced vital capacity (FVC). The boy was intubated and transferred to Johns Hopkins Hospital.

The child had been delivered vaginally at 32 weeks' gestation, with no postdelivery complications. He had reached all of his developmental milestones, was generally healthy and up to date with immunizations. The boy had been sick with an upper respiratory infection (rhinorrhea, cough, and low-grade fever) 2-3 weeks earlier.

The patient was febrile with a stable blood pressure and pulse, and was sedated and connected to a ventilator

  • HEENT: Normocephalic, atraumatic, no lymphadenopathy, no masses. No meningeal signs.

  • CVS: Regular heart rate and rhythm, normal S1 and S2, no murmurs, rubs, or gallops.

  • Lungs: Clear to auscultation.

  • Abdomen: Soft, nontender, nondistended, with normal bowel sounds.

The boy was somnolent from the sedation, but his pupils were equal, round, and reactive to light and his oculocephalic and corneal reflexes were intact. He had a weak gag response. He was flaccid, but moving all extremities spontaneously, localizing noxious stimuli, and was areflexic with upgoing toes bilaterally.

Head CT: normal
Chest x-ray: normal
Blood chemistries: normal
Peripheral WBC count: 6080/mm3
CSF WBC count: zero
CSF protein: 34 mg/dL
CSF glucose: 79 mg/dL