Adam T. Schulman, MD
Resident Physician
Mount Sinai Hospital
NYC, New York, United States
Jacob S. Levine, MD
Resident Physician
Mount Sinai Hospital
NYC, New York, United States
Renee Li, BS
Medical Student
Jefferson Medical School
NYC, New York, United States
Vincent Huang, MD
Attending Physician
Mount Sinai Hospital
New York, New York, United States
Pure sensory ganglionopathy
Case Description:
A 40-year-old female with no past medical history developed leg pain and quadriparesis over multiple weeks. EMG was consistent with pure sensory ganglionopathy. A broad workup investigating potential paraneoplastic, immunologic, rheumatologic, cardiac, or endocrine causes was largely inconclusive though weakly associated with new onset lupus. The patient was treated for lupus and discharged to acute rehab. She presented with considerable pain described as constant, burning, and radiating from her lower back to the posterior, bilateral lower extremities. Her pain regimen included neuropathic and nociceptive medications, titrated to: gabapentin 1200mg TID, nortriptyline 75mg qHS, lyrica 200mg TID, voltaren gel TID, lidocaine patches, magnesium gluconate 500mg qHS, and tramadol 50/100mg as needed. Duloxetine and capsaicin cream were trialed but discontinued due to adverse drug reactions. Cognitive training was also key.
Discussions:
Sensory ganglionopathies (SG) are a rare type of nervous system disease involving degeneration of sensory neurons in the dorsal root ganglia. It can present as pain, burning, and dysesthesia. Etiologies can be paraneoplastic, autoimmune, or idiopathic. Its clinical presentation has been well described, but the literature on pain management in SG is limited. Neuropathic pain, such as that caused by SG, is often treated using a multimodal approach. First-line pharmacotherapy includes antidepressants (TCAs, SNRIs) and antiseizure medications (gabapentin, pregabalin), and second-line includes lidocaine, capsaicin, and tramadol. Lidocaine, capsaicin, and tramadol are also effective for nociceptive pain. Some evidence shows that combining antidepressants and antiseizure medications can be at least as effective as monotherapy at higher doses. Cognitive techniques such as tactile desensitization may reduce pain and dysesthesia. This patient benefited from a multimodal regimen, including medications targeting neuropathic and nociceptive pain and tactile desensitization. Although this rare disorder requires individualized care, this case can guide pain management for other patients with SG.
Conclusions:
