Introduction: Guillain-Barre Syndrome (GBS) is an acute post-infectious demyelinating disorder characterized by motor, sensory, and autonomic nervous system impairment. In affected patients, cross-reactive autoantibodies attack the host’s axonal antigens, causing an inflammatory polyneuropathy. Case Description: Our case report is about a previously healthy, fully vaccinated 4-year-old male who presented with an atypical hospital course that ultimately led to the diagnosis of the acute motor and sensory axonal neuropathy (AMSAN) variant of GBS. The patient presented to the ED three times before being admitted to the hospital for evaluation of worsening neck and back pain with increased fussiness. He underwent a full meningitis/encephalopathy workup including imaging upon admission, with the lone remarkable finding as mildly elevated CSF protein levels (71 mg/dL). Physical exam remained consistent for 5 days post-admission without evidence of diminished muscle strength or areflexia, albeit limited due to the patient’s resistance to leaving his bed during examination, stating that his pain was too severe. There was suspicion of a behavioral component as the patient’s pain was distractible. Consultants from pediatric neurology and psychology were suspicious for an infectious and behavioral etiology respectively rather than neurologic impairment. Later, infectious lab work was consistent with an acute CMV infection and his symptoms were then attributed as such. Physical therapy was then consulted to assess the patient’s ambulation for discharge planning, however, they were acutely concerned with the patient’s resistance to ambulation and did not recommend discharge. Heeding this advice, along with the parents’ intuition that an underlying cause remained unresolved, the patient remained hospitalized. Within 48 hours of the PT consult, the patient became progressively lethargic with incontinence and was noted to have areflexia, which shifted the differential towards GBS and prompted treatment with a course of IVIG. Nerve conduction studies showed mixed axonal and demyelinating process that is consistent with a GBS-AMSAN variant. Discussion: We believe this case is indicative of the variability in symptomatic expression of GBS in pediatrics. While the preceding CMV infection is not necessarily unusual, the patient’s initial complaint of neck and back pain without classic MRI changes proved difficult to interpret. It is important to recognize that young children can present with nonspecific clinical features including poorly localized pain, refusal to bear weight, meningism, or unsteady gait. Additionally, although the AMSAN variant is more common in adults, this case affirms that consideration is warranted in children. Unfortunately, affected pediatric patients are equally susceptible to the longer recovery time and increased risk of permanent neurologic impairment associated with this disease. Conclusion: Ultimately, we hope our challenges in navigating this case will encourage other pediatric institutions to include GBS on their differential in the setting of post-infectious generalized pain and irritability, and when applicable, will promote earlier diagnosis and effective treatment.
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February 23 2022
Atypical Presentation of a Guillain-barre Syndrome Variant in a 4-year-old
Ned Shashoua, BA;
Ned Shashoua, BA
(1)Florida Atlantic University, Boca Raton, FL
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Kranthi Nomula, MD;
Kranthi Nomula, MD
(2)Joe DiMaggio Children’s Hospital, Hollywood, FL
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Lindsay Weiss, MD
Lindsay Weiss, MD
(2)Joe DiMaggio Children’s Hospital, Hollywood, FL
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Pediatrics (2022) 149 (1 Meeting Abstracts February 2022): 810.
Citation
Ned Shashoua, Kranthi Nomula, Lindsay Weiss; Atypical Presentation of a Guillain-barre Syndrome Variant in a 4-year-old. Pediatrics February 2022; 149 (1 Meeting Abstracts February 2022): 810.
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