A 9-year-old boy is brought to the emergency department due to lower extremity weakness and pain. The patient first noticed soreness in his legs yesterday and had difficulty standing this morning. He has a tingling sensation in his toes and fingers as well as lower back pain. Temperature is 37.2 C (98.9 F), blood pressure is 105/70 mm Hg, and pulse is 40/min. The patient is alert and responds appropriately. Heart and lung examinations are normal aside from bradycardia. The lower back pain is nonreproducible with palpation of the spinous processes or paraspinal muscles. Neurologic examination shows decreased strength and diminished reflexes in both lower extremities. There is pain and mildly decreased sensation with pinprick testing. Which of the following is most associated with this patient's underlying diagnosis?
Guillain-Barré syndrome | |
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This patient has Guillain-Barré syndrome (GBS), an acute demyelinating polyneuropathy. Initial presentation frequently includes paresthesia (tingling) of the fingers and toes; however, the hallmarks of the disease are ascending weakness and hyporeflexia originating in the lower extremities. Sensation is typically intact or mildly diminished. Neuropathic pain (eg, back pain without spinal tenderness) is a predominant finding in children, and autonomic dysfunction (eg, bradycardia) occurs in many cases.
Most patients with GBS have an antecedent respiratory or gastrointestinal infection, which stimulates production of antibodies that cross-react with peripheral nerve axons (ie, molecular mimicry). Campylobacter jejuni infection is a common foodborne, diarrheal illness and the most frequent precipitant. Other infections (eg, cytomegalovirus), recent immunization, surgery, and trauma have also been associated with GBS.
Supportive laboratory findings include cerebrospinal fluid albuminocytologic dissociation (ie, elevated protein with normal leukocytes). Management includes monitoring of respiratory and autonomic function as well as immunotherapy (eg, intravenous immunoglobin).
The majority of patients recover fully; however, Campylobacter-associated GBS tends to have a worse prognosis with a slower return to health and increased residual deficits.
(Choice A) Perinatal asphyxia leading to cerebral palsy can present in early infancy with abnormal tone and delayed motor milestones. This 9-year-old's acute neurologic changes are unlikely to be related to a perinatal event.
(Choice B) Inherited muscular dystrophies present gradually with progressive muscle weakness. Muscle pain can occur, and reflexes may be decreased with muscle atrophy. However, the acute nature of this patient's illness makes GBS more likely.
(Choice C) Todd paralysis is a postictal condition that can occur in patients with a seizure disorder and is characterized by brief, unilateral weakness. This patient's progressive symptoms in the absence of seizure are not consistent with Todd paralysis.
(Choice D) Polio causes acute flaccid paralysis and is more likely in a child with an incomplete vaccination history. In contrast to GBS, weakness associated with polio is asymmetric and typically preceded by aseptic meningitis (eg, fever, headache, fatigue).
(Choice E) Living in a homeless shelter increases the risk of infections such as tuberculosis. Weakness due to spinal tuberculosis develops over months and is typically associated with constitutional symptoms (eg, fever, weight loss).
Educational objective:
Diarrheal illness (eg, Campylobacter jejuni infection) is the most frequent precipitant of Guillain-Barré syndrome (GBS), an acute demyelinating polyneuropathy characterized by ascending weakness and hyporeflexia. Other common findings of GBS include paresthesia, neuropathic pain, and autonomic dysfunction.