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Isaac Syndrome with Intractable Neuropathic Pain Features: A Case Report
Mustafa Al-Chalabi1, Nicholas R DelCimmuto2, Pratyush Pavan Devarasetty2
1Department of Neurology, University of Toledo, Toledo, Ohio, USA.
Case Reports in Neurology
|May 9, 2022
Summary
Isaac syndrome (IS), a rare peripheral nerve hyperexcitability disorder, can cause severe neuropathic pain. This case highlights carbamazepine and subcutaneous immunoglobulin as effective treatments for this challenging symptom.
Area of Science:
- Neurology
- Immunology
- Clinical Neuroscience
Background:
- Isaac syndrome (IS) is a peripheral nerve hyperexcitability disorder linked to voltage-gated potassium channel (VGKC) complex antibodies.
- Typical IS symptoms include muscle twitching, stiffness, hypertrophy, and dysautonomia, with neuropathic pain being an uncommon manifestation.
Observation:
- This report details a patient with IS presenting with muscle twitching and severe, intractable neuropathic pain.
- Diagnostic findings included elevated VGKC complex antibodies and neuromyotonic discharges on electromyography/nerve conduction studies.
Findings:
- Neuropathic pain in this IS case was refractory to multiple treatments, including opiates, benzodiazepines, anticonvulsants, and intravenous immunoglobulin (IVIg).
- Optimal pain management was achieved with long-term carbamazepine and subcutaneous immunoglobulin (SCIg), demonstrating a significant response where IVIg showed limited efficacy.
Implications:
- This case underscores that neuropathic pain, though rare, can be a profoundly distressing and resistant symptom in Isaac syndrome.
- The combination of carbamazepine and SCIg offers a potential therapeutic strategy for managing refractory neuropathic pain in IS patients.
