Can Guillain-Barré Syndrome (GBS) present with a proximal paraparetic form and normal reflexes?

Yes, AIDP Can Present as a Proximal Paraparetic Variant with Normal Reflexes

Yes, Guillain-Barré syndrome (GBS), including AIDP, can present with a paraparetic variant affecting the lower limbs, and normal or even exaggerated reflexes can occur in a minority of patients, particularly in the pure motor variant with AMAN subtype. 1

Paraparetic Variant Recognition

• The paraparetic variant is a recognized distinct clinical form of GBS where weakness is specifically limited to the lower limbs rather than following the classic ascending pattern 1
• This variant is explicitly listed among the established GBS variants that include weakness limited to cranial nerves, upper limbs (pharyngeal-cervical-brachial), or lower limbs (paraparetic variant) 1
• The pure motor variant of GBS, which includes the paraparetic presentation, can manifest with weakness in specific body regions without sensory signs, occurring in 5-70% of cases 2

Normal or Exaggerated Reflexes in GBS

• In atypical GBS presentations, particularly the pure motor variant with AMAN subtype on electrophysiological examination, normal or even exaggerated reflexes might be observed throughout the entire disease course 1
• Up to 10% of patients with GBS may retain present or even brisk deep tendon reflexes, challenging the traditional diagnostic expectation of areflexia 3
• AMAN (acute motor axonal neuropathy) can specifically present with normal or exaggerated reflexes, distinguishing it from classic demyelinating GBS 2, 4
• Case reports document hyperreflexia with abnormal reflex spread in AMAN following Campylobacter jejuni infection, with increased motor neuron excitability persisting even after complete strength recovery 4

Proximal Weakness Pattern

• Lower extremity weakness in GBS is characteristically proximal in a significant percentage of patients, which differs from typical polyneuropathies that usually affect distal muscles first 2
• This proximal distribution pattern is a recognized feature that can occur in the paraparetic variant 2

Critical Diagnostic Pitfalls

• Do not dismiss GBS based on the presence of normal or brisk reflexes—this is a well-documented atypical presentation that occurs in approximately 10% of cases 3
• Many neurologists may not be aware that reflexes can remain present or appear brisk in GBS, leading to diagnostic delays and missed opportunities for early immunotherapy 3
• The paraparetic variant is frequently not recognized as GBS because it doesn't follow the classic ascending bilateral pattern, but it represents a legitimate variant requiring the same urgent evaluation and treatment 1
• Electrophysiological measurements might be normal when performed early or in patients with initially proximal weakness—repeat electrodiagnostic studies 2-3 weeks later are helpful if clinical suspicion remains high 2

Diagnostic Approach

• Maintain high clinical suspicion for GBS even when reflexes are preserved, particularly if the patient has:
  • Recent infection history (within 6 weeks) 5
  • Rapidly progressive proximal lower limb weakness 2
  • Pure motor involvement without sensory signs 1
  • Pain (muscular, radicular, or neuropathic) as an early symptom 5
• Obtain nerve conduction studies looking for AMAN pattern (motor axonal degeneration) which is strongly associated with preserved or exaggerated reflexes 2, 4
• Check for antiganglioside antibodies (particularly anti-GM1b and anti-GalNAc-GD1a) which are associated with AMAN and hyperreflexia 4
• Perform cerebrospinal fluid examination for albuminocytological dissociation, though this may be absent in the first week 6

Treatment Implications

• All grades of GBS, including atypical presentations with normal reflexes, warrant complete workup given potential for rapid progression to respiratory compromise 2
• Approximately 20% of patients develop respiratory failure, which can occur rapidly even without obvious dyspnea 6
• Initiate immunotherapy (IVIg 0.4 g/kg/day for 5 days or plasma exchange) in patients unable to walk unaided within 2-4 weeks of symptom onset, regardless of reflex status 6
• Neurology consultation should be obtained for all suspected cases 2