Efficacy of Spinal Cord Stimulation Using Differential Target Multiplexed Stimulation for Intractable Pain of Hereditary Neuropathy with Liability to Pressure Palsies: A Case Report

Abstract

Hereditary neuropathy with liability to pressure palsies is an extremely rare genetic disorder; it is an autosomal dominant disorder with a high incidence of neuropathic and/or musculoskeletal pain. A case of achieving pain relief by spinal cord stimulation using differential target multiplexed stimulation for a 44-year-old female patient with hereditary neuropathy with liability to pressure palsies who was experiencing severe pain in her back, face, and all four limbs is presented. In her early teens, the initial symptoms were numbness and weakness of a limb after movement, which improved spontaneously. Transient pain in her back followed by systemic and persistent muscle weakness and pain developed. Deletion of the gene for peripheral myelin protein 22 was detected by peripheral nerve biopsy. The diagnosis of hereditary neuropathy with liability to pressure palsies was made in her early thirties. A spinal cord stimulation trial was performed because her severe pain continued despite administering many medications. Therefore, two spinal cord stimulation systems were implanted at the C3-5 and Th8-9 levels by two procedures. Pain in her back, arms, and legs decreased from 8 to 1, 5 to 1, and 6 to 2 on the numerical rating scale, respectively. Furthermore, opioid usage was tapered. The pain of hereditary neuropathy with liability to pressure palsies has a complicated pathogenesis and is resistant to pharmacological treatment. Spinal cord stimulation using differential target multiplexed stimulation may be a viable treatment option.

Keywords: DTM; hereditary neuropathy with liability to pressure palsies; neuropathic pain; spinal cord stimulation.

Fig. 1

The schematic diagram shows the pain locations, including her back, all four limbs, and face. The numerical rating scale scores for back pain are 8 (red color), and bilateral arms and legs are 5 and 6, respectively (gray color).

Fig. 2

A: Magnetic resonance image shows disc herniations at C5/6 and C6/7 (thin arrow) and syringomyelia at the Th2 level (thick arrow). B, C: Dynamic magnetic resonance images demonstrate protruding disc herniations compressing the spinal cord (B: retroflexion, C: anteflexion).

Fig. 3

A, B: Two percutaneous, 8-contact leads of the first implantation are located at the C3-5 levels (A: anterior-posterior view, B: lateral view). C, D: Two additional percutaneous 8-contact leads of the second implantation are located at the Th8-9 levels (C: anterior-posterior view, D: lateral view). E: Finally, four leads and two implantable pulse generators are implanted.