Bilateral trigeminal neuralgia in association with a possible Charcot-Marie-Tooth disease: A case report

Abstract

Introduction: Trigeminal neuralgia is described by the International Headache Classification 3rd edition as a chronic, painful, and sporadic condition characterized by electric shock-like hemifacial pain. The initial management of trigeminal neuralgia is with medication, if there is persistence of pain, surgical management is the gold standard. Microvascular decompression is a non-destructive technique that improves pain in 98% of cases. In the literature, only 5 cases of CMT-associated trigeminal neuralgia that were surgically managed with microvascular decompression have been published. Case report: A 58-year-old male patient with a personal pathological history of CMT diagnosed at 34 years of age, initially presented with incapacitating right hemifacial electric shock-like pain. The pain had been tolerated with pharmacological management for 5 years. Since the patient had a partial response to carbamazepine and the pain increased suddenly, a trigeminal nerve decompression was performed. Surgical findings were arachnoid adhesions and contact with superior cerebellar artery, the adhesions were liberated and Teflon was placed in the contact area. The patient had no further pain.Four years later, the patient presented again with a similar clinical picture characterized by left hemifacial pain with no control of pain with medication therefore, surgical management was decided. This time around, the surgical findings were adhesions between the trigeminal nerve and the anteroinferior cerebellar artery, which were liberated and teflon was placed between the nerve and the artery. After surgery, the patient had no pain. Discussion: Very few cases of surgical management of trigeminal neuralgia in association with CMT have been reported. The surgical findings, in this case, showed adhesions bilaterally, which could be another factor contributing to the bilateral neuralgia. Microvascular decompression has proven to be a good option for surgical management. Conclusion: The surgical management for trigeminal neuralgia in association with CMT is still an understudied subject, and yet microvascular decompression is a suitable technique for this condition, because it may resolve the vascular decompression by the placement of teflon and may also liberate adhesions if present. Keywords: Bilateral trigeminal neuralgia, Charcot-Marie-Tooth disease, Surgical management, Microvascular decompression, Arachnoid adhesions