Current developments have refined the diagnostic framework for Guillain-Barré Syndrome (GBS). A recently published article in Nature Reviews Disease Primers emphasizes the critical importance of these advances in clinical practice.
GBS subtypes, such as acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor-sensory axonal neuropathy (AMSAN), are distinguished based on nerve conduction studies. However, Leonhard et al. highlight that this approach may be oversimplified. These subtypes are challenging to differentiate clinically and rely mainly on nerve conduction studies. The authors advocate for the standardization of electrophysiological studies and the development of unified protocols to streamline the diagnostic workflow in clinical practice.
The authors suggest that utility of lumbar puncture in diagnosing GBS should be reconsidered. Its role in the diagnostic workflow should shift from confirmation to exclusion of other conditions. While the classic finding of albuminocytologic dissociation is supportive of GBS, it is nonspecific and appears in fewer than 50% of cases within the first week of symptoms, increasing up to 80% after the first week. Instead, cerebro-spinal fluid analysis should be examined to exclude alternative diagnoses (e.g. West Nile virus, sarcoidosis, malignancy).
This review highlights the need to update our understanding of GBS and its pathogenesis. Electrophysiological diagnostics should be able to identify early and characteristic features of GBS. Lumbar punctures should focus on ruling out other conditions. Efforts must be made to prioritize accessible and personalized treatments, especially in low-income areas.
Key Points:
- Dichotomous classification of GBS (axonal vs demyelinationg) rely on limited nerve conduction studies and might not be relevant in early diagnostic work-up.
- Lumbar puncture should primarily exclude alternative diagnoses, while albumino-cytologic dissociation is nonspecific.
- Access to treatments like immunoglobulins and plasma exchange remains a major challenge in low-income settings.
- Improved understanding of the pathogenesis and biomarkers is essential for better diagnostics and therapeutic approach.
References:
Leonhard, S.E., Papri, N., Querol, L. et al. Guillain–Barré syndrome. Nat Rev Dis Primers 10, 97 (2024). https://doi.org/10.1038/s41572-024-00580-4
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