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Published on: February 15, 2022
[Ganglionopathies: evolving concept and ideas on management]
1Unité Nerf-Muscle, Service de Neurologie, CHU Vaudois, Lausanne, Suisse. thierry.kuntzer@chuv.ch
Sensory ganglionopathies, affecting dorsal root ganglion cells, often link to cancer or autoimmune issues. Distinctive signs include unsteady gait and hand movements, with global sensory nerve signal loss.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Context:
- Sensory ganglionopathies are neurological disorders impacting dorsal root ganglion cells.
- They are frequently associated with neoplastic (cancer-related) or dysimmune (autoimmune) disorders.
- Common triggers include chemotherapy drugs like cisplatin, vitamin B6 (pyridoxine) toxicity, and inherited conditions.
Purpose:
- To review recent advancements in understanding the mechanisms of sensory ganglionopathies.
- To outline the differential diagnosis of these conditions.
- To highlight the distinct characteristics of sensory nerve action potentials in chronic sensory ganglionopathies.
Summary:
- Sensory ganglionopathies present with unsteady gait and pseudoathetoid hand movements.
- Unlike other sensory neuropathies, they show a global reduction in sensory nerve action potential (SNAP) amplitudes, not just distal.
- This review covers mechanisms, differential diagnosis, hereditary neuronopathies, and infectious causes.
Impact:
- Enhances understanding of the underlying mechanisms of sensory ganglionopathies.
- Provides a framework for accurate differential diagnosis, distinguishing from other sensory neuropathies.
- Improves clinical recognition and management of patients with these complex neurological conditions.
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