Jaw Pain in an Adolescent Boy
Ramesh Balasubramaniam, BDSc, MS,
Martin S. Greenberg, DDS and
Andres Pinto, DMD, MPH
Abbreviations: MRI: Magnetic resonance imaging • PSH: Primary stabbing headache • SUNCT: Short-lasting unilateral neuralgiform headache attack with conjunctival injection and tearing • TMJ: Temporomandibular joint • TN: Trigeminal neuralgia
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THE CHALLENGE
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A 14-year-old boy was referred to the Department of Oral Medicine at the Hospital of the University of Pennsylvania in Philadelphia for evaluation and treatment of jaw pain. The patient described intermittent pain localized to the right mandible that had begun two years before, with no antecedent traumatic event. In addition, he described brief, stabbing, electric, shocklike pain that occurred when eating and also spontaneously. On a numerical rating scale of 1 to 10, he rated the pain at 10. He reported that each painful attack lasted 10 seconds and that this occurred several times a day. Between pain attacks, the patient reported mild-to-moderate dull pain localized to the right masseter muscle area. The patient also described having intermittent bilateral asymptomatic temporomandibular joint (TMJ) clicking.
Reportedly, his dentist, in an attempt to control what the patient described as "muscle spasms," had treated the patient with an oral appliance and physical therapy. His dentist had also ordered magnetic resonance imaging (MRI) with and without contrast of the TMJs, which were unremarkable for intracapsular pathology.
At the time the clinician (A.P.) evaluated the patient at our clinic, his medical history, review of systems and family, and social and medication histories were unremarkable. He also was undergoing fixed orthodontic treatment without complications.
The clinical examination revealed a well-nourished and well-developed adolescent boy without affective distress. The findings of the head and neck examination were unremarkable. Examination of cranial nerves II to XII showed that the patient had diminished sensation (hypoesthesia) to light touch with a cotton swab and to cold, and he could not recognize pinpricks localized to the distribution of the right mandibular division of the trigeminal nerve. Functions connected with the other cranial nerves—visual acuity and fields, equal and reactive pupils, eye movement, masticatory muscle strength, facial expression, elevation and symmetry of the palate and uvula, contraction of sternocleidomastoid and trapezius, and extrusion of the tongue—were grossly intact. The patient’s reactions to palpation of his cervical and masticatory muscles and TMJs were unremarkable except for mild-to-moderate tenderness of the right masseter muscle. The patient could open his mouth to a maximum of 48 millimeters without pain or deviation. His functional jaw movements, including eccentric and protrusive movements with and without resistance, were unremarkable and produced no TMJ sounds. On oral examination, the examiner found enlarged tonsils, but otherwise an absence of intraoral pathology. Maxillary and mandibular dentitions were banded with braces.
Palpation of the right mandibular ramus area triggered the patient’s jaw pain (which was localized anatomically to the distribution of the right third division of the trigeminal nerve) that lasted approximately 30 seconds. After the pain, the patient experienced a refractory period during which the examiner’s attempts to trigger subsequent episodes were unsuccessful. After a few minutes, palpation of the right buccal mucosa area once again reproduced the pain attack. A panoramic radiograph showed that the patient had impacted third molars, but the results were otherwise unremarkable.
Can you make the diagnosis?
- masticatory myospasm
- primary stabbing headache
- pain disorder (somatoform)
- trigeminal neuralgia
- short-lasting unilateral neuralgiform headache with conjunctival injection and tearing
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THE DIAGNOSIS
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D. trigeminal neuralgia
The International Association for the Study of Pain defines trigeminal neuralgia (TN) as "a sudden, usually unilateral, brief stabbing recurrent pain in the distribution of one or more branches of the fifth cranial nerve."1 TN is an episodic neuropathic pain disorder that can occur with vascular compression of the trigeminal nerve or with no apparent cause (classical TN) or secondary to another disease process such as a demonstrable structural lesion or multiple sclerosis (symptomatic TN). It has a slight preponderance in women (1.5:1) and has an average age of onset of 50 years.2 Clinicians base the diagnosis of TN primarily on the patient’s medical history, supplemented by the findings of a physical examination and brain imaging studies.
The paroxysmal attacks consist of unilateral, brief, lancinating pain involving one or more divisions of the trigeminal nerve, commonly the second and third divisions.2 Patients often describe the quality of the pain as sudden, sharp, electric-shocklike, shooting, stabbing or burning, and as being of excruciating intensity. Each attack typically lasts from 10 seconds to two minutes with pain-free periods lasting seconds to hours between each attack. On occasion, patients describe having a dull "background ache" after a pain attack; this ache is the result of the ongoing protective contraction of the jaw muscles secondary to the TN pain attacks, which results in masticatory muscle tenderness. Stimuli such as shaving, chewing, talking and brushing teeth involving trigger zones within the distribution of the trigeminal nerve may provoke the attacks. Attacks also can occur spontaneously.
The clinical sensory examination, specifically of the trigeminal nerve, usually appears normal.2 A patient with TN attacks may have periods of remission, during which time the patient has no pain for months or years.
We found this patient’s medical conditions to be consistent with the diagnosis of TN. The etiology and pathophysiology of TN remain enigmatic. The most commonly accepted theory regarding the cause of classical TN suggests distortion, compression or both of the trigeminal nerve root fibers by either the superior cerebellar artery or anterior inferior cerebellar artery. Researchers postulate that the demyelination, dysmyelination or both of the nerve in time leads to spontaneous firing of the afferent nerve, facilitated by central disinhibition of the trigeminal brain stem complex.3,4 Symptomatic TN can occur in up to 10 percent of patients who have classical TN symptoms. Common underlying causes include multiple sclerosis, basilar artery aneurysm, neoplastic conditions such as acoustic neuroma and meningioma, arterial or venous compression, and brain stem infarction.3,5 Clinicians treating patients with TN should order imaging studies of the patient’s brain to rule out pathology of the central nervous system (CNS). The teenaged patient in this case had sensory deficits, which is an atypical presentation of TN. His case further emphasizes the need for brain imaging.
TN can be managed with either pharmacological or surgical therapies. The goal of pharmacological therapy is to manage TN pain attacks until the patient experiences remission from them. The clinician then can taper the patient’s medications slowly. Antiepileptic drugs (such as carbamazepine, oxcarbazepine and gabapentin) are the main treatment options for patients with TN, although clinicians may prescribe other medications for patients whose TN is refractory to usual treatments (Box 1
).
Clinicians reserve surgical therapy for TN for those patients whose symptoms are resistant to pharmacological treatments or who are unable to tolerate the side effects of the medications prescribed. Surgical options for TN include microvascular decompression, stereotactic radiosurgery (gamma knife), glycerol injection, radiofrequency thermocoagulation and balloon microcompression, among others.6
As previously mentioned, the patient in this case is considered young for a diagnosis of TN; in addition, he had an atypical presentation that included sensory nerve deficits. We ordered an MRI with and without contrast of the brain to rule out symptomatic TN. We also referred the patient to the hospital’s neurology department for further evaluation of the right mandibular division of the trigeminal nerve deficit. Meanwhile, we determined classical TN to be the initial presumptive diagnosis and prescribed 100 milligrams of gabapentin to be taken three times a day. We instructed the patient’s parent to increase the dosage gradually to 300 mg three times per day during a four-week period if the patient reported no benefit or side effects at lower doses. The patient underwent an ultrathin MRI with contrast five weeks later; through this procedure, the radiologist detected a hyperintense C-shaped lesion surrounding the right trigeminal nerve at its brain stem origin, with no significant postcontrast enhancement (Figure).
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Figure. Magnetic resonance image with contrast (T1-weighted) highlighting a hyperintense C-shaped lesion surrounding the right trigeminal nerve at its brainstem origin (arrows).
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Because of the patient’s young age and worsening symptoms despite pharmacological therapy, a neurosurgical procedure was performed after extensive discussion of treatment options with his neurologist. The lesion was removed and histopathology confirmed a diagnosis of lipoma. Only six cases are reported in the literature of secondary TN caused by lipoma at the brain stem origin of the trigeminal nerve.7–12
At this patient’s one-year follow-up appointment, TN attacks had improved significantly but they continued to occur occasionally at a lesser intensity. Today, the patient remains in the care of his neurologist, who continues to manage his symptoms with neuroleptic drugs.
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DIFFERENTIAL DIAGNOSIS
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Clinicians base the diagnosis of TN on the patient’s history, clinical examination and imaging of the brain. Doctors must rule out numerous clinical entities that can mimic TN13 (Box 2). For this particular case, we offered four other possible diagnoses for the patient’s presentation: masticatory myospasm, primary stabbing headache (PSH), somatoform pain disorder and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT).
Masticatory myospasm.
Masticatory myospasm is an involuntary, CNS-induced, tonic muscle contraction, resulting in increased electromyographic activity. Contrary to long-held beliefs in dentistry, masticatory myospasm is an uncommon source of myogenous pain that clinicians can identify easily via clinical presentation. The etiology of masticatory myospasm is unknown; however, it may be related to muscle fatigue, changes in electrolyte balance or deep pain input from pre-existing muscle soreness. The patient usually reports a sudden onset of pain and jaw tightness, as well as a change in jaw position. Clinically, this may manifest as a marked restriction of jaw range of movement, acute malocclusion, jaw pain at rest that increases with function and tenderness on palpation of the muscle(s) in spasm.14 Apart from the sudden onset of pain and masseter muscle tenderness, this patient’s history and clinical characteristics did not fit this diagnosis. The patient’s masseter muscle tenderness likely was secondary to ongoing protective contraction of the masticatory muscles from the TN pain attacks. This is similar to the presence of masticatory muscle tenderness or pain in a patient with a painfully impacted third molar.
PSH.
The International Classification of Headache Disorders II describes PSH as "transient and localized stabs of pain in the head that occur spontaneously in the absence of organic disease of underlying structures or of the cranial nerves."15 PSH may present as a single stab or a series of stabs typically involving the first division of the trigeminal nerve (orbital, temporal and parietal areas); however, other areas of the face may also be involved. Each stab typically lasts less than three seconds and recurs with irregular frequency. PSH has a predominance in women (5:1) and occurs more commonly among patients with a history of migraine or cluster headaches.15,16 This patient’s short-lasting pain attacks could be triggered by touching certain areas of the face and lasted 10 seconds, making his symptoms inconsistent with PSH. Typically, PSH cannot be triggered, and the pain usually lasts for about three seconds. Also, the combination of this patient’s being male and having facial location of PSH attacks made the PSH diagnosis highly improbable.
Pain disorder (somatoform).
Somatoform disorders are mental disorders in which physical symptoms suggest a general medical condition; however, the patient had insufficient physical signs to support the diagnosis.17 Specifically, the diagnosis of pain disorder, which is a subtype of somatoform disorders, centers on the patient’s primary complaint of pain existing in the absence of an obvious pathology to explain the pain. The condition gradually develops during the patient’s teenage years and is established by the age of 30 years. Other characteristics include significant social and occupational distress and impairment.
Psychological factors may have an important role in the onset, severity, exacerbation and maintenance of the pain.4 If the presence of mood, anxiety or psychotic disorder can better explain the pain complaint, the clinician still should not render the diagnosis of a pain disorder without an assessment from a mental health care professional. Clinicians do not commonly diagnose this condition among orofacial pain patients. Differentiating pain disorder from neuropathic pain, specifically TN, can be challenging, as neither condition is associated with any obvious source of pathology. Of interest is that clinicians once considered TN to be caused by mental disorders.4 Therefore, the clinician should base the diagnosis of pain disorder on a clear role of the above-mentioned characteristics, rather than the inability to find an organic basis for the pain complaint.4,17,18 Regardless, if the clinician suspects somatoform disorder, he or she should recommend that the patient be evaluated by a clinical psychologist or psychiatrist for a formal diagnosis. Although TN is rare among young patients, pain disorder as a diagnosis for this patient also was unlikely because his history and examination was otherwise consistent with TN. The patient did not exhibit signs and symptoms of distress and impairment. In addition, psychological factors were not instrumental in the onset, severity, exacerbation and maintenance of the patient’s pain.
SUNCT.
SUNCT is a syndrome characterized by strictly unilateral pain attacks localized to orbital, supraorbital or temporal areas or combinations of these sites, accompanied by cranial and facial autonomic features. Typically, the autonomic features include ipsilateral conjunctival injection and lacrimation. The attacks are short-lasting and frequent, with at least three attacks and up to 200 attacks per day, and each attack lasts between five and 240 seconds.15 There also are reports19,20 of SUNCT’s being preceded by TN and affecting the ophthalmic branch of the trigeminal nerve and, hence, there are suggestions that SUNCT merely may represent a variant of TN.21 However, clinicians may differentiate SUNCT from TN on the basis of the patient’s lack of autonomic features during TN attacks. Also, unlike TN, SUNCT attacks almost always affect the ophthalmic branch; however, SUNCT pain can affect the midface and teeth.22 Additionally, SUNCT does not have a refractory period between attacks.16 Specifically, this patient’s pain attacks were not accompanied by cranial or facial autonomic effects, and the patient experienced a refractory period between attacks, during which time he could not trigger the pain.
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CONCLUSION
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TN is a painful and disabling orofacial pain condition that is diagnosed on the basis of the patient’s history and clinical examination. Clinicians should order imaging studies of the patient’s brain to rule out an underlying disease process such as a demonstrable structural lesion or multiple sclerosis.
An oral health care provider may be the first health care provider a patient with TN visits before receiving a diagnosis. Therefore, dentists should be knowledgeable about this episodic neuropathic pain disorder so that they can render an accurate diagnosis and avoid the pitfall of implementing unnecessary and inappropriate traditional dental treatments in attempting to alleviate this condition.
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FOOTNOTES
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When this article was written, Dr. Balasubramaniam was a fellow, Department of Oral Medicine, School of Dental Medicine, University of Pennsylvania, Philadelphia. He now is the co-director, Perth Orofacial Pain and Oral Medicine Centre, St John of God Hospital, Suite 319, 25 McCourt St., Subiaco, Western Australia, Australia, 6008, e-mail "rbmesh{at}yahoo.com.au". Address reprint requests to Dr. Balasubramaniam.
Dr. Greenberg is a professor and the chair, Department of Oral Medicine, School of Dental Medicine, University of Pennsylvania, Philadelphia.
Dr. Pinto is an assistant professor, Department of Oral Medicine, School of Dental Medicine, University of Pennsylvania, Philadelphia.
Disclosure. The authors did not report any disclosures.
Diagnostic Challenge is published in collaboration with the American Academy of Oral and Maxillofacial Pathology and the American Academy of Oral Medicine.
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REFERENCES
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THE CHALLENGE
THE DIAGNOSIS
