Atypical Guillain-Barré syndrome

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Abstract

Case Presentation: An 8-year-old patient presented with a 15-day course of muscle weakness and pain, preceded by upper respiratory tract infection. Initially, he complained of hand paresthesia, and later, loss of strength in the wrists, forearms and arms. No decrease in sensitivity of upper and lower limbs at all times. No recent vaccination history. Brain MRI was normal. Neurological physical examination with negative meningeal signs, preserved gait and significant decrease in strength in upper limbs, impossibility of moving the hands, strength grade 3 proximal and grade 4 distal. Little reduction in lower limb’s strength. Normal reflexes. Neuraxial MRI showed a T2 hypersignal of the ventral horns of the spinal cord in its distal aspect next to the conus medullaris at the level D11-12. CSF showed protein-cytologic dissociation. Established diagnosis of Guillain-Barré syndrome with atypical presentation. Treatment with human-Immunoglobulin 2g/kg. After treatment there was a noticeable improvement in muscle strength and upper limb movements. Patient referred to the neuropediatric outpatient clinic and motor physiotherapy after discharge.

Discussion: GBS has an Incidence of about 100.000 new patients every year worldwide. It’s an immune-mediated, heterogeneous peripheral neuropathy syndrome with several variants. Is often precipitated by infection; Campylobacter jejuni, CMV, EBV, HIV, Zika virus, and Influenza-A. Symptoms start with numbness or paresthesia in the hands and feet, then a heavy, weak feeling in the legs. Weakness ascends typically reaching maximal severity within four weeks from symptom onset. Most patients generally require hospitalization for treatment, with close cardiopulmonary monitoring performed. This case report is a valuable contribution to GBS literature as it highlights an unusual presentation of the condition in an 8-year-old patient. GBS is a severe and rare neurological disorder that can lead to respiratory failure and paralysis by affecting the peripheral nervous system. The case underscores the importance of considering GBS in patients experiencing muscle weakness and paresthesia, even in the absence of typical symptoms like reduced sensitivity and gait disturbance.

Final Comments: The patient's muscle weakness only in the upper limbs, along with an uncommon T2 hypersignal in the distal aspect of the spinal cord's ventral horns, is noteworthy. The diagnosis of GBS is further supported by protein-cytologic dissociation in CSF.

Publication History

Article published online:
12 May 2025