Bilateral Trigeminal Neuralgia and CMT:Diagnosis and Successful Microsurgical Tr

[Guest guest]

Zentralbl Neurochir. 2008 Jul 29.

Bilateral Trigeminal Neuralgia and Charcot-Marie-Tooth Disease:

Diagnosis and Successful Microsurgical Treatment of Bilateral

Neurovascular Compression.

Tekkok IH, Sumer M.

1Department of Neurosurgery, MESA Hospital, Ankara, Turkey.

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.