Can Guillain‑Barré syndrome present with normal deep‑tendon reflexes in children?

Can Guillain-Barré Syndrome Present with Normal Reflexes in Children?

Yes, GBS can present with normal or even hyperactive reflexes in a small subset of children, particularly in the early stages of disease, though this is uncommon and occurs in approximately 6-7% of pediatric cases. 1, 2

Understanding the Reflex Pattern in Pediatric GBS

Classic Presentation

• Diminished or absent deep tendon reflexes (DTRs) are the hallmark feature of GBS and occur in the vast majority of children, with loss of reflexes seen in all patients in some series and 82-93% in others 3, 4, 5, 2
• The classic sensorimotor form presents with rapidly progressive symmetrical weakness and sensory signs with absent or reduced tendon reflexes 3
• Decreased or absent reflexes are present in most patients at presentation and almost all patients at nadir 6

The Exception: Normal or Hyperactive Reflexes

• Approximately 6.6-7% of children with GBS may have normal tendon reflexes or even hyperreflexia, particularly in the early stage of disease 1, 2
• This atypical presentation can lead to diagnostic delay and misdiagnosis, as reflexes are considered a key diagnostic criterion 2
• The presence of normal or exaggerated DTRs early in the disease course does not exclude GBS, especially when other clinical features are consistent 2

Clinical Implications for Diagnosis

When to Suspect GBS Despite Normal Reflexes

• Acute symmetrical limb pain and gait disturbance should raise suspicion for GBS even if reflexes are initially preserved 5
• Distal lower limb weakness (present in 92% of pediatric cases) combined with neuropathic pain (75% of cases) are more sensitive early indicators than reflex changes 2
• Bilateral ascending weakness starting in the legs and progressing to arms and cranial muscles is the hallmark clinical pattern 6, 4

Diagnostic Approach in Atypical Cases

• Do not dismiss GBS based solely on preserved reflexes in the first few days of illness—serial neurological examinations are essential as reflexes typically become diminished as the disease progresses 2
• Obtain cerebrospinal fluid analysis looking for albumino-cytological dissociation (elevated protein with normal cell count), though this may be absent in the first week 7
• Electrodiagnostic studies (nerve conduction studies and EMG) should be performed to support diagnosis, as these can reveal characteristic patterns even when clinical examination is equivocal 7
• Look for the "sural sparing pattern" on nerve conduction studies—normal sural sensory nerve action potential with abnormal median/ulnar responses 7

Critical Pitfalls to Avoid

Delayed Diagnosis Risk

• Children with GBS are frequently misdiagnosed when presenting to orthopedic departments with limb pain and gait disturbance, particularly when reflexes are preserved early 5
• Among pediatric patients initially presenting to orthopedics, 60% were misdiagnosed in one series, leading to treatment delays 5
• Early diagnosis is essential to prevent delayed recovery, long-term weakness, and permanent functional disabilities 5

Monitoring for Disease Progression

• Even with normal reflexes initially, approximately 20% of children with GBS develop respiratory failure requiring mechanical ventilation, which can occur rapidly without obvious dyspnea 7, 4
• Serial vital capacity and negative inspiratory force measurements should be performed frequently 7
• Monitor for cranial nerve involvement (present in 47-58% of pediatric cases), particularly bilateral facial palsy 1
• Assess for dysautonomia including blood pressure/heart rate instability, as autonomic dysfunction is associated with catastrophic outcomes 2

Diagnostic Criteria Considerations

The diagnostic criteria for GBS may need revision to account for atypical presentations in children, as the traditional requirement for areflexia can lead to missed or delayed diagnoses in the 6-7% of pediatric patients with preserved reflexes 2. When clinical suspicion is high based on the pattern of ascending weakness, pain, and sensory symptoms, proceed with confirmatory testing (CSF analysis, nerve conduction studies) rather than excluding GBS based on reflex examination alone 2.