June 11, 2012
A 10-year-old girl with quadriparesis and bilateral absent patellar and achillan reflexes.
Guillain-Barré Syndrome
- Guillain Barré syndrome (GBS) is characterized by demyelination and axonal degeneration of peripheral nerves.
- Clinical presentation is usually characterized by rapidly progressive, ascending, and symmetric paralysis of the extremities. Loss of tendon reflexes and mild sensory signs can be present.
- The cranial nerves are affected in 30-40% of cases.
- Diagnostic confirmation is based on compatible clinical findings and paraclinical features. Magnetic resonance imaging (MRI) of the spine is useful to evaluate for other conditions. Imaging is not used routinely to diagnose GBS.
- Key Diagnostic Features: MRI findings are characterized by thickening and marked enhancement of the anterior spinal nerve roots, especially in the region of the cauda and conus medullaris.
- DDx: AIDS-related polyradiculopathy, arachnoiditis, sarcoidosis, metastatic conditions
- Rx: Plasmapharesis and immunoglobulin therapy
