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Washington University Experience | PERIPHERAL NEUROPATHY | 6 AXONAL DEMYELINATION (BASIC PROCESS) | 5A1 Demyelination (Case 5) Plastic 40
Case 5 History ---- The patient is a 57-year-old male with quadriplegia and respiratory failure with a presumed diagnosis of "recurrent GBS" that has been refractory to repeated IVIg infusions. NCS/EMG showed absent sensory and motor responses with diffuse denervation. Total protein in CSF is high (1060). Total spine MRI showed multilevel root thickening and enhancement at cervical, dorsal and lumbosacral levels. Right upper extremity ultrasound showed diffuse enlargement of the right ulnar and median nerves, left vagus and visualized left cervical nerve roots. Operative procedure: Sural nerve and deltoid muscle biopsy. The final diagnosis on this case was demyelinating neuropathy most consistent with a CIDP-like disorder. ---- 5A1-3 There is significant loss of axons overall. In addition, there is no definitive evidence of macrophages actively engulfing myelin debris. The scattered remaining axons show absence of myelin (arrow, 5A2), particularly large axons. Regenerative clusters and remyelination are not appreciated.
