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[Myelin diseases affecting both the central and the peripheral nervous system]
K Hagen1, S I Mellgren, G Bovim
1Nevrologisk avdeling, Norges teknisk-naturvitenskapelige universitet, Trondheim.
Summary
This review examines rare cases where both the central and peripheral nervous systems experience myelin disorders. It highlights conditions like multiple sclerosis and Guillain-Barré syndrome that can affect both systems.
Area of Science:
- Neuroscience
- Neuropathology
- Genetics
Context:
- Myelin, essential for nerve function, is produced by oligodendrocytes (CNS) and Schwann cells (PNS).
- Combined central and peripheral nervous system myelin disorders are exceptionally rare.
- This article reviews the infrequent co-occurrence of central nervous system (CNS) and peripheral nervous system (PNS) demyelinating diseases.
Purpose:
- To review and consolidate information on rare conditions involving simultaneous demyelination in the CNS and PNS.
- To discuss reported cases and genetic aspects of combined central and peripheral nervous system myelin disorders.
Summary:
- Rarely, conditions affect myelin in both the central nervous system (CNS) and peripheral nervous system (PNS).
- Examples include co-occurrence of multiple sclerosis with chronic inflammatory demyelinating polyneuropathy, and CNS demyelination in Miller-Fisher and Guillain-Barré syndromes.
- Metachromatic leukodystrophy, Krabbe disease, and adrenoleukodystrophy commonly present with concurrent CNS dysmyelination and peripheral nerve demyelination. A Norwegian family with autosomal dominant adult-onset disease affecting both systems was also described.
Impact:
- Enhances understanding of complex neurological disorders affecting myelin.
- Provides a reference for clinicians and researchers investigating rare combined CNS/PNS demyelinating diseases.
- Contributes to the knowledge base for diagnosing and potentially treating these uncommon conditions.