Microvascular Decompression for Trigeminal and Glossopharyngeal Neuralgia in a Patient with Diagnosis of Charcot–Marie–Tooth Disease

Introduction: Charcot–Marie–Tooth (CMT) is an inherited chronic peripheral neuropathy characterized by atrophy, weakness, and distal sensory loss.[1] [2] Although CMT typically affects peripheral nerves, few case studies reported rare coincidence of trigeminal neuralgia (TGN) in CMT patients.[3] [4] [5] Etiology of TGN in this group of patients is believed to be from underlying CMT-induced neuropathy. In this report, we present a case of intractable trigeminal and glossopharyngeal neuralgia (GPN) in a patient diagnosed with CMT. Indication for microvascular decompression and long-term follow-up is highlighted.

Case Report: A 44-year-old male presented with 14-year history of intractable right TGN and GPN. Past medical history also includes childhood polio and congenital talipes equinovarus. Since initial diagnosis, patient was maintained on carbamazepine and due to progressively worsening symptoms, and he was trialed on indomethacin for hemicrania continua, valproic acid, baclofen, dexamethasone, oxycodone, and hydromorphone. Despite multiple cranial nerve neuropathies, unified diagnosis of CMT was not made until preoperative hospitalization where electrophysiological studies confirmed severe bilateral sensorimotor peripheral neuropathies.

Brain MRI and MRA studies ([Figs. 1] and [2]) demonstrated close proximity of the superior petrosal vein to the right trigeminal nerve, and no underlying source for glossopharyngeal neuralgia.

Given failure of maximum medical management microvascular decompression of trigeminal nerve and exploration of lower cranial nerves was planned. Intraoperative findings indicated thickened arachnoid adhesions, compression of trigeminal nerve by the superior petrosal vein (SPV), and compression of lower cranial nerves (CN XI–XI) by loop of posterior inferior cerebellar artery (PICA) ([Figs. 3] and [4]). After meticulous dissection of arachnoid adhesions, decompression of involved nerves was achieved by coagulation of SPV and placement of pledgets between PICA and lower cranial nerves. Patient experienced immediate postoperative pain relief and remains pain-free on 4 years of follow-up.

Conclusion: It is hypothesized that CMT predisposes individuals to periaxonal inflammation leading to thick arachnoid adhesions and subsequent neurovascular entanglement. Therein, CMT as the pathophysiology of TGN should not preclude diagnostic workup of vascular compression. Additionally, despite nonconvincing MRI findings, microvascular decompression should be considered in selective case.[1] [3]

Publication History

Article published online:
01 February 2023

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