A 74-year-old woman had a 5-year history of constant burning pain and
numbness of the central face of subacute onset. The central region of
the face, oral cavity, and nose lacked all sensation. Corneal reflexes
and the jaw jerk were absent. Blood tests, rectal biopsy, neurodiagno
stic studies, and surgical exploration of the trigeminal nerve were no
rmal. Blink reflexes were absent. Facial nerve motor latencies and EMG
of the facial and masseter muscles were normal. Responses to the ther
moregulatory sweat test, intradermal histamine, and simulated diving w
ere present. Oral administration of 500 mg L-dopa aggravated her pain
and produced transient hypalgesia in the C2 through C5 dermatomes. Inf
raorbital nerve biopsy demonstrated loss of large myelinated fibers. I
n conclusion: (1) Only the central region of the face is exclusively s
upplied by the trigeminal nerves. (2) Somato-autonomic reflexes couple
d with electrophysiological studies localized the lesion to the large
fibers. (3) Large fiber loss and central brain stem reorganization may
explain the burning pain. (4) Dopamine may modulate trigeminal nocice
ption.