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Focal chronic inflammatory demyelinating polyradiculoneuropathy: Onset, course, and distinct features
Charline Benoit1, Juliette Svahn2, Rabab Debs1
1Département de neurophysiologie clinique, Hôpital de la Pitié-Salpêtrière, APHP Paris VI Université, Paris, France.
Journal of the Peripheral Nervous System : JPNS
|March 4, 2021
Summary
Focal chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) can present differently. This study distinguishes focal plexus neuropathy (F-PN) from other focal CIDP subtypes, showing F-PN has a better prognosis and remains localized.
Area of Science:
- Neurology
- Immunology
- Neurophysiology
Background:
- Focal chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is characterized by localized nerve involvement.
- Autoimmune neuropathies like Lewis-Sumner syndrome (LSS) and multifocal motor neuropathy (MMN) can mimic focal CIDP presentations.
- Distinguishing subtypes of focal CIDP is crucial for accurate diagnosis and prognosis.
Purpose of the Study:
- To differentiate between focal demyelinating plexus neuropathy (F-PN) and other focal CIDP subtypes with monomelic onset.
- To analyze clinical characteristics, electrophysiological findings, MRI features, and long-term outcomes of these distinct focal CIDP entities.
- To clarify the relationship between focal CIDP presentations and the potential evolution to LSS or MMN phenotypes.
Main Methods:
- Retrospective analysis of 30 focal CIDP patients with monomelic onset of at least two years.
- Classification of patients into focal demyelinating plexus neuropathy (F-PN), focal sensory/sensorimotor neuropathy (F-SMN), and focal motor neuropathy (F-MN) groups.
- Assessment of nerve conduction studies, somatosensory evoked potentials (SSEPs), plexus MRI, and clinical outcomes including the ONLS score.
Main Results:
- F-PN patients (n=18) showed distinct features, with most having proximal SSEP abnormalities (80%) and specific MRI findings, unlike F-SMN (n=7) and F-MN (n=5) patients.
- F-PN impairment predominantly remained monomelic (94%), contrasting with F-SMN (57%) and F-MN (40%) patients who developed abnormalities in other limbs.
- F-PN patients exhibited a significantly better prognosis, with no patients scoring >2 on the ONLS scale, compared to F-SMN (43%) and F-MN (40%) patients.
Conclusions:
- Focal CIDP encompasses distinct entities: focal plexus neuropathy (F-PN) and monomelic sensory-motor or motor neuropathies of peripheral nerves.
- F-PN is characterized by a distinct, long-term, focal, and benign clinical course.
- F-SMN and F-MN subtypes are more likely to evolve towards Lewis-Sumner syndrome or multifocal motor neuropathy phenotypes.
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