A Misdiagnosis of Atypical Trigeminal Neuralgia
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RESIDENT & FELLOW SECTION Clinical Reasoning: A misdiagnosis of atypical trigeminal neuralgia Jaclyn R. Duvall, MD, and Carrie E. Robertson, MD Correspondence Dr. Robertson Neurology 2019;93:124-131. doi:10.1212/WNL.0000000000007790 ® [email protected] Section 1 A 47-year-old man presented with right-sided facial pain that started 2 years prior. He described the pain as extremely intense, stabbing along the right jaw, lasting 5–60 seconds. This pain was exacerbated by chewing, and to a lesser degree, by brushing his teeth. The pain was so intense that he avoided eating when possible, leading to a 20-pound weight loss. When he did eat, he would try to chew on the left side of his mouth. Around the onset of these symptoms, he also noticed a persistent numbness and burning extending from the right lower earlobe to the lateral angle of the jaw that was exacerbated by turning his head to the right. The patient was given a diagnosis of atypical trigeminal neuralgia (TN) and sent to our headache clinic for further management. Questions for consideration: 1. What features are typical and atypical for classical TN? 2. What is your differential diagnosis in this patient presenting with facial pain? GO TO SECTION 2 From the Department of Neurology, Headache Division, Mayo Clinic, Rochester, MN. Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. 124 Copyright © 2019 American Academy of Neurology Copyright © 2019 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Section 2 refractory period following a paroxysmal pain, where addi- tional pain cannot be triggered.1 In contrast to the classic TN is characterized by “recurrent paroxysms of unilateral pain paroxysmal, shooting, stabbing, or shock-like qualities used in the distribution(s) of one or more divisions of the tri- to describe neuralgic pain, neuropathic pain tends to involve 1 geminal nerve,” most often the maxillary and mandibular more persistent burning, tingling, or numbness. If the pa- divisions. As outlined by the International Classification of tient has features of neuropathy (numbness), persistent 1 Headache Disorders, 3rd edition, TN pain : pain, bilateral pain, isolated supraorbital involvement, or a lack of sensory triggers, an alternative diagnosis should be A. Does not radiate beyond the trigeminal distribution considered. This could include dental pathology, parotid B. Lasts from a fraction of a second to 2 minutes (recurrent pathology, or an alternative neuralgia/neuropathy. See the paroxysms may occur in clusters lasting longer) table for a comprehensive overview of conditions that may C. Is severe in intensity and electric shock–like, shooting, mimic TN. stabbing, or sharp in quality D. Is precipitated by innocuous stimuli within the trigeminal Our patient described paroxysmal severe shooting facial pain, nerve distribution lasting seconds at a time. He reported innocuous triggers, including chewing and possibly brushing his teeth. In- Patients tend to be asymptomatic between paroxysms, terestingly, however, he denied all other classical TN triggers, though a subset of patients may develop a prolonged con- including cutaneous triggers. He also described an atypical tinual background pain, with fluctuations in intensity and persistent pain that was more neuropathic (numbness/ periods of remission and recurrence that parallel the par- burning) than neuralgic, and this extended over the angle of 2 oxysmal pain. Approximately 99% of patients with TN the jaw and into the pinna of the ear, both territories that are report at least some of their attacks being associated with outside of the distribution of the trigeminal nerve. Given the 2–4 atrigger. In one large study, the most commonly de- atypical presentation, we were concerned about an alternative scribed triggers were gentle touching of the face (83% of diagnosis. patients) and talking (59%), followed by chewing (41%) and tooth brushing (36%).3 When cutaneous triggers are Questions for consideration: present, the most common trigger zones are around the 1. What additional historical details should be asked to mouth and nose, though anywhere on the face may be narrow the differential? described.3,5 Some patients may notice a transient 2. What additional examination tests might be helpful? Neurology.org/N Neurology | Volume 93, Number 3 | July 16, 2019 125 Copyright © 2019 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Table Conditions that may mimic trigeminal neuralgia Most common Pain Condition location characteristics Aggravating factors Things you should know or look for Dental Caries Affected tooth Intermittent to Sweet foods, hot or cold Visible decay continuous dull stimuli Cracked tooth Affected tooth Intermittent Biting/chewing, hot or cold Often difficult to visualize crack dull or sharp stimuli Dry socket Affected tooth Continuous, Hot or cold stimuli Loss of clot, exposed bone deep, sharp TMJ dysfunction (TMJ disease) Jaw and surrounding Constant or Forced mouth opening, jaw May be complication of arthritis, jaw muscles, radiation to intermittent, manipulation, chewing locking or popping ear and temple tender, aching Sinusitis Nasal passages, Pressure, Bending over Fever, swelling, purulent nasal above the eyes, tenderness, discharge, reduced sense of smell and cheeks aching taste, positive CT or endoscopy Parotid Sialadenitis Submandibular or Gradual, Tender to palpation Swelling, erythema over the gland, parotid region constant fever, chills Salivary stone Submandibular or Intermittent, Salivation (eating or smelling Tenderness at gland, palpable stone, parotid region dull foods) lack of salivary flow FBS Submandibular or Paroxysmal Salivation (eating or smelling No sensory triggers, history of prior parotid region severe, sharp foods) head/neck surgery, improves after a few bites Neoplasm Benign/malignant Submandibular or Persistent dull Swallowing, opening mouth Lump or swelling, facial numbness, parotid region pain widely muscle weakness if masseter involved Giant cell arteritis Temporal region or Typically Scalp or vessel tenderness Visual disturbance, jaw claudication, holocephalic continuous dull fevers/chills, night sweats, weight loss, pain (new or ↑ESR/CRP, temporal artery biopsy worsening) Carotid Evaluation for Horner for all carotid pathology (ptosis/miosis/anhidrosis) Dissection Neck, lower face/jaw, Thunderclap or Head/neck movement May have preceding trauma to neck or retro-orbital acute tearing whiplash injury pain or progressive or throbbing Carotidynia Tender carotid Variable Pressure over carotid artery; Controversial—Imaging may reveal bifurcation, may temporal coughing, swallowing, focal eccentric thickening of the radiate to ipsilateral patterns, Valsalva, cold weather, head carotid wall leading to luminal face or ear aching/ movement narrowing, but is otherwise normal tenderness, sore throat Primary headache disorders Migraine Hemicranial > Pulsating or Physical activity, lights, sounds, Nausea, sensory phobias (light/ holocephalic throbbing, certain foods, hormonal sound), stereotypical attacks, family lasting 4 hours changes history or more SUNCT Orbital, supraorbital, Severe, Usually spontaneous; can Autonomic features (conjunctival or temporal stabbing, have sensory triggers include injection, tearing, rhinorrhea, facial lasting a few touching face/scalp, chewing, flushing/sweating, ptosis/miosis) seconds to talking, coughing, blowing a couple of nose, light minutes Neuropathies Allodynia and hyperesthesia commonly seen with all neuropathies Continued 126 Neurology | Volume 93, Number 3 | July 16, 2019 Neurology.org/N Copyright © 2019 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Table Conditions that may mimic trigeminal neuralgia (continued) Most common Pain Condition location characteristics Aggravating factors Things you should know or look for TNO Unilateral distribution Numbness, Light touch Further diagnostic evaluation of trigeminal nerve, burning, indicated especially if progressive as V2/V3 most common continuous, this may be the first manifestation of paresthesia or tumor or relapse of prior neoplastic dysesthesias process, trauma is most common mechanism leading to TNO (often iatrogenic) Idiopathic Neoplastic Inflammatory/autoimmune Traumatic Blunt, dental Anesthesia dolorosa Numb chin syndrome Unilateral lower lip Numbness Light touch, biting lip Red flag association with breast, lung, (“mental neuropathy”) and chin and lymphoproliferative malignancies, although dental procedures are most common culprit Postherpetic neuralgia Dermatome of single Continuous or Light touch in affected Vesicles or scarring from herpetic nerve or nerve root intermittent, dermatome rash; despite its name, postherpetic electrical, neuralgia is actually a neuropathy or burning, sharp neuronopathy Burning mouth syndrome Mouth, gums, lips Burning, No clear factors May be seen with xerostomia, continuous candidiasis, GERD, poor oral hygiene Other neuralgias Glossopharyngeal neuralgia Ear, base of tongue, Paroxysmal Swallowing, yawning, 10% of cases associated with syncope/ (CN IX) tonsillar fossa, angle severe, coughing, touching the ear arrhythmia, consider Holter monitor of the jaw electrical, shooting, stabbing, sharp Nervus intermedius Deep inside and Paroxysmal Touch over or within the ear May occur with Bell palsy; disorders of neuralgia (“geniculate