Unsteady on his feet
Why is a little boy losing strength in his legs?
Vol.15, No.10, October 2008

Tony is a 3-year-old with a 3-day history of fever, coughing and abdominal pain. He’s had some intermittent vomiting but no diarrhea. His appetite has waned but urination is normal. Further questioning reveals a sore throat and rhinorrhea. Tony’s immunizations are up to date, he’s had no prior surgeries and isn’t on any medications. Examination shows the boy to be nontoxic and afebrile. The throat is mildly erythematous. Cervical nodes are enlarged. Heart is normal. Lungs are clear. Abdominal exam shows neither tenderness nor organomegaly. The diagnoses of viral upper respiratory infection and gastroenteritis are made and the child is sent home. His mother is instructed to give acetaminophen for fever and to push solid and fluid intake.

Six days later, Tony and his Mom are back. She now says the boy has trouble walking. The fever is gone but his appetite remains poor. The patient still has some vomiting but no coughing or diarrhea. He looks tired but alert. Gait is unsteady and wide based and he’s unable to walk without support. Arm strength seems normal but leg strength is noticeably weak with decreased tone. Neck is supple. A presumptive diagnosis is made and the patient is sent to the children’s hospital.

At the ER

Tony is seen in the emergency department and admitted. Initial vitals include: blood pressure 110/80, pulse 90-100, respiration rate 24/minute and temperature of 35.8°C. Oxygen saturation is 100% at room air. Neurology notes that the little boy has full range of motion of all extremities. He has no facial asymmetry and has full extraocular range of motion. There’s no clonus. Deep tendon reflexes, however, are absent and toes are downgoing.

Investigations done in the hospital include a computed tomography of the head, which is normal. A lumbar puncture shows slightly elevated level of protein but no white blood cells. Cerebral spinal fluid cultures are negative. Complete blood count shows mildly low hemoglobin of 96 and a normal white blood cell count. Blood cultures and stool cultures for virus/botulism are negative.

What’s wrong with Tony?

ANSWER
Guillain-Barré syndrome

A normal 3-year-old boy suddenly develops cold symptoms and abdominal pain. A week later, he’s having obvious difficulty walking. See page 11 for the complete case description.

Diagnosis and etiology

Tony has Guillain-Barré syndrome (GBS). The exact cause of GBS is unknown but it’s believed to come about when a person’s own immune system attacks the nerves resulting in bilateral weakness.

Symptoms

It usually starts in the extremities and can progress to respiratory paralysis and death, if not treated.

Often a viral illness such as an upper respiratory infection or gastroenteritis precedes GBS.

Investigations

There are no definitive tests for GBS and one must have a high index of suspicion to diagnose the condition.

Management

Treatment is supportive. Plasmapheresis and intravenous immunoglobulins have been shown to decrease duration and severity of the disorder.

Outcome

In this case, Tony was treated with intravenous immunoglobulin for five days. He was put on telemetry to monitor his cardiopulmonary status. His swallowing was also assessed. While in the hospital, Tony’s strength slowly improved and he was discharged after 10 days. Follow-up at the office showed the patient still had ataxia but was able to walk on his own.

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Clinical challenge image
Clinical challenge image
A nerve fibre with a thin myelin sheath after remyelination (top) Downgoing toes in Guillain-Barré patient (bottom)
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