Chronic orofacial pain is sometimes a vexing problem. The pain may be somatic, neurogenic, or psychogenic, and can originate from the teeth, gums, masticatory muscles, mandible, maxilla, and temporomandibular joint.1 Involuntary oromandibular movements can develop following dental procedures.2 Less well known to clinicians is the occurrence of sensory symptoms preceding or associated with cranial dystonia.3 An unusual patient with facial pain, ipsilateral focal facial dystonia, and a phantom sensation of supernumerary canine (“Dracula's”) teeth after gum resection is reported. She had previous history of migraine without aura for 40 years. This report illustrates the rare coexistence of facial dystonia, facial pain, and phantom phenomena in a patient that had been subjected to gum resection for hypertrophic gingivitis.
Objective.—To describe a patient with facial pain, ipsilateral facial dystonia, and phantom supernumerary teeth, beginning after resection of hypertrophic gums. She had familial sensorineural deafness.
Background.—Atypical cranial dystonia subsequent to dental procedures is associated with facial pain, dysesthesias, or phantom phenomena, but not with phantom supernumerary teeth. Patients with migraine are susceptible to experience cephalic phantom phenomena after dental procedures. Wynne syndrome is a hereditary autosomal dominant disorder characterized by congenital sensorineural deafness and supernumerary teeth.
Methods.—Clinical examination, computerized tomography of paranasal sinuses and facial bones, magnetic resonance imaging of the brain, cranial electrophysiological testing, and electroencephalogram.
Results.—A 52-year-old woman with history of migraine without aura for 40 years exhibited focal right facial involuntary tonic contracture accompanying chronic severe pain over the same area after gum resection. She reported a daily sensation of having two extra upper canine teeth pressing on her tongue, simulating vampire's (“Dracula's”) teeth. She had high-frequency bilateral sensorineural deafness. Her computerized tomography studies, brain magnetic resonance imaging, and cranial electrophysiological testing were normal.
Conclusion.—This patient with chronic migraine had atypical cranial dystonia beginning after a dental procedure. Her dystonia was complicated by the unusual phenomenon of phantom supernumerary teeth. This condition may be misdiagnosed as atypical or psychogenic facial pain when facial dystonia is localized or subtle and is, therefore, confused with an idiosyncratic gesture or habitual spasm. Her signs and symptoms are reminiscent of Wynne syndrome.
A 52-year-old woman was referred to a neurologist because of right facial pain radiating from the malar region diagonally to the right upper lip area. She had pain for several months following upper and lower surgical resection of hypertrophic gums. The pain was severe, constant, and interfered with her sleep. She had no gustatory sweating or flushing of her face or neck. She developed symptoms of depression because of the chronic pain. Injection of facial trigger points, root canals, and a course of antibiotics were ineffective in ameliorating her pain. She took doxepin, gabapentin, and nonprescription analgesics without experiencing any relief. An ear, nose, and throat examination had been normal, except for bilateral high-frequency hearing loss. She reported no flushing of the submandibular region and had no history of chronic bruxism or teeth clenching. Along with her pain, she developed an annoying mild tonic upward retraction of the right corner of her mouth. She reported a constant sensation of having two long extra upper canine teeth growing in front of her normal canines that felt like they were pressing on her tongue. The sensation was experienced as someone with vampirelike long upper canines (“Dracula's teeth”). She reported a history of severe pulsatile right frontal headaches with an average frequency of twice a month for the last 40 years and of weekly mild frontal headaches relieved by single doses of ibuprofen. Although she had history of familial sensorineural deafness, she had only high-frequency hearing loss improved by hearing aids. There was no family history of gum hyperplasia or supernumerary teeth. She complained of poor taste, forgetfulness, sleep fragmentation, and high-pitched ringing noises in her ears of long-standing. She had no burning of her tongue.
Her vital signs and general physical examination were normal. On neurologic examination, she exhibited tonic contraction of the right levator angular oris muscle resulting in mild upward pulling of the right corner of her mouth. She had no weakness of the palate and no atrophy, discoloration, or inflammation of the tongue. There were no fasciculations of the tongue. There were no objective sensory deficits. She had high arches to her feet but symmetric deep tendon reflexes. Muscle strength was normal, and there was no evidence of muscle atrophy. She complained of pain on the right side of her face. She had decreased hearing bilaterally. No dental or intraoral mucosal pathology was observed on inspection.
The following tests were normal: electroencephalogram, brain magnetic resonance imaging, cranial nerve testing (blink reflex studies, facial and mental nerve latencies), computerized tomography of the sinuses and of the facial bones. Complete blood count, routine blood chemistries, Lyme disease titer, thyroid profile, sedimentation rate, and serum protein electrophoresis were normal. The following antibody assays were negative: antinuclear antibodies, Sjogren syndrome antigens A and B, ribonucleoprotein, smooth muscle, and thyroid peroxidase.
Two neurosurgical consultants advised against performing any surgical procedures for the treatment of her facial pain. Eventually her facial pain, facial muscle contracture, phantom dental sensation, and symptoms of depression improved after treatment with doxepin 100 mg at bedtime and long-acting oxycodone 20 mg twice a day. No new symptoms or intercurrent illnesses have developed after 2 years of follow-up. She experiences no medication side effects.
Cranial dystonia may appear hours to months after dental procedures.2 It most often involves the masseters and pterygoideus muscles causing forceful opening or closing of the mouth. Other cranial muscles including the facial muscles become involved over time, producing blepharospasm. Atypical cranial dystonia is characterized from the onset by persistent pain and dysesthesia reminiscent of limb causalgia syndrome. The facial pain is felt over the area occupied by the dystonic muscles. Ghika et al reported that spontaneous cranial dystonia is often heralded by dysesthesias in the orofacial areas that are subsequently involved by the dyskinesia.3 Phantom phenomena can follow the deafferentation of many organs, but it is more striking in limb amputees.4-6 Phantom experience may be kinetic, static, painful, painless, transient, or persistent. It may be induced by drugs and can be ameliorated or triggered by unusual mechanisms.4,7-9 Two of the patients of Ghika et al (cases 10 and 11) with facial dysesthesias and cranial involuntary movements experienced phantom pain perceived over their dentures.3 Their patients described other phantom phenomena including mustardlike taste (case 3), swelling and burning of the tongue and gums (case 4), sensation of remnants of food in between the teeth (case 5), and a feeling of disarticulation of the temporomandibular joint (case 10).3 Induction of a phantom supernumerary limb following therapeutic cortical stimulation was previously reported in a patient with a thalamic stroke.10 To my knowledge, there are no earlier reports of patients with migraine and cranial dystonia experiencing phantom supernumerary teeth.
Phantom tooth pain seems to develop with greater frequency in chronic headache sufferers. Sicuteri et al conducted a survey on the frequency of phantom pain in a group of 251 patients with migraine without aura and 50 patients with episodic cluster headache.11 All the patients had persistent pain in edentate sites in the absence of local pathology, following tooth pulp extirpation. The authors compared the results of their survey with those obtained from a third group of 280 subjects without personal or family history of chronic headache. The highest incidence of phantom tooth pain was in the group of patients with cluster headache, followed by the group with migraine. None of the patients without history of chronic headaches reported ever having phantom dental pain. Subsequently, Nicolodi et al extended the above findings to a group of 53 patients submitted to eye removal for ocular cancer.5 The patients who experienced general phantom phenomena (photopsia, phantom pain, and painless phantoms) had history of chronic headache. These investigators concluded that those with headache possess basic dysfunctional cephalic sensory transmission that becomes apparent after traumatic or acute trigeminal deafferentation.5 Wynne et al reported an autosomal dominantly inherited syndrome characterized by gum hyperplasia, hypertelorism, sensorineural deafness, and supernumerary teeth.12 Although the present case is reminiscent of Wynne syndrome because she had gum hyperplasia and familial sensorineural deafness, she only had virtual supernumerary teeth. In addition, no member of her family had gum hypertrophy or supernumerary teeth. Patients with facial pain beginning after dental procedures may be incorrectly diagnosed as having atypical or psychogenic facial pain if they complain of unusual phantom phenomena. Careful observation during neurologic examination may discover the presence of cranial dystonia. If dystonia is found, the patient cannot be diagnosed with atypical or psychogenic pain.