Bilateral trigeminal neuralgia and charcot-marie-tooth disease: diagnosis and successful microsurgical treatment of bilateral neurovascular compression.

Abstract

Background: The association of Charcot-Marie-Tooth (CMT) disease and trigeminal neuralgia (TN) is rare. CMT and bilateral TN is even rarer. Our literature review has revealed only 4 previous cases with CMT and bilateral TN. We report the case of a 23-year-old man with a prior diagnosis of CMT and unilateral deafness who initially presented with excruciating and lancinating right-sided facial pain.

Patient and method: Initially treated with percutanous ablation elsewhere with only short-lasting relief, the patient was on toxic doses of medication when he presented to us. Neuro-imaging using magnetic resonance imaging showed vascular compression as the possible cause of pain. At initial surgery, two artery loops were found compressing the right nerve superomedially and an artery and a vein was found compressing the nerve inferomedially. Relief was instantaneous and for 1 year there was no recurrence of right facial pain. Left-sided pain which had been minimal before microvascular decompression (MVD) became intolerable immediately after right-sided surgery, yet responded to mild doses of carbamazepine for almost a year. At one year, the patient had to undergo MVD for the left trigeminal nerve with complete relief over both sides of his face without any medication.

Results: The patient remains pain-free on both sides at 1-year follow-up after the second MVD.

Conclusions: The underlying neuropathy in CMT makes the trigeminal nerve more vulnerable to vascular compression than usual. The bilateral compression of the trigeminal nerve-pons junction in our patient suggests that the external pressure probably adds to the internal defects in central myelin formation, structure or maintenance. Nevertheless, the mid-term follow-up after MVD clearly shows that the causal treatment for TN can be successfully applied to patients with TN plus CMT.