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Washington University Experience | PERIPHERAL NEUROPATHY | 7B CIDP | 21K Case 21 Denouement
This was a puzzling case and a very difficult nerve biopsy to interpret in a patient with a complex and protracted course. There are many demyelinated axons with nearby macrophages; however, the presence of a myelinated axon with intact myelin attacked by a macrophage which has only begun to phagocytose myelin is very rare. That pathologic process is seen only in the demyelinative form (AIDP) of Guillain-Barre syndrome and chronic inflammatory demyelinative neuropathy (CIDP). In demyelinated syndromes one might expect little axonal degeneration; however, there is often significant axon loss. I have seen cases of fulminant GBS in which entire fascicles showed degenerating axons. There is a single epineurial vessel in this case which appears to have fibrin deposition in its walls and possible recanalization. CIDP and GBS typically have perivascular inflammation which usually involves small vessels and does not result in frank angionecrosis. In this case the inflammation may have been substantial enough to result in functionally significant vasculopathy resulting in ischemic changes superimposed on axonal degeneration. ---- Diagnosis: Neuropathy characterized by fulminant active axonal degeneration, demyelination and rare macrophage-mediated demyelination.
