A 52-year-old female has a five-year history of facial pain that only occurs at night, awakening her from sleep about two in the morning. She describes a burning or pressure pain in the right upper teeth with an intensity of 10/10, which then spreads to the entire right face, associated with nausea, vomiting once, light and noise sensitivity but no eye redness or tearing, nares congestion or drainage, ptosis, or miosis. The pain lasts about 20-30 minutes. During an attack, she feels like she can’t lie down and has to get up and move around. The pain may recur a second time within a two-hour span. The pain may occur daily for six to eight weeks and then go away for about six to nine months before recurring. She saw a neurologist and was diagnosed with trigeminal neuralgia.

What is the diagnosis?

Episodic cluster headache (at least two cluster periods lasting from seven days to one year (when untreated) and separated by pain-free remission periods of ≥1 month).

Cluster headache is misdiagnosed more than 80 percent of the time when first seeing a physician and even when seeing a neurologist, especially in a case like this where there are atypical features.1

The duration of each headache (untreated, cluster has a duration of 15-180 minutes), nocturnal awakening, and duration of the bouts are all typical of cluster.

In one study, migrainous symptoms of light and noise sensitivity were reported by 70 percent and vomiting or nausea in more than 20 percent.2 Perhaps 14 percent of cluster patients report an aura, including visual and paresthesia.3 Gaul, et al. found headaches occurring between 1-6 a.m. in about 75 percent of patients.

There are two less common features in this case. The patient does not have associated cranial autonomic symptoms (CAS). In a series of 95 cluster headache patients, 95 percent had CAS with the following: conjunctival injection and/or lacrimation, 95 percent; conjunctival injection, 62 percent; lacrimation, 95 percent; nasal congestion and/or rhinorrhea, 77 percent; nasal congestion, 45 percent; rhinorrhea, 65 percent; eyelid edema, 21 percent; and forehead/facial sweating, 57 percent.4 The Gaul et al. study of 209 consecutive patients with episodic and cluster headache found at least one CAS in 99.5 percent.

The International Headache Society criteria for cluster require either or both of at least one CAS or a sense of restlessness or agitation. Gaul, et al. found restlessness in 83 percent of cases, as in this case.

And the distribution of pain? Gaul, et al. found periorbital pain location reported by more than 75 percent of patients, followed by occipital neck region and orofacial pain. They note that the orofacial localization and some patients reporting toothache-like pain (40 percent) may lead to unnecessary dental treatments including extractions. Based upon other series, the pain is behind the eye in about 90 percent of cases, over the temple in 70 percent, and over the maxilla in 50 percent.5 The pain is often described as sharp, stabbing, piercing, burning, or pulsating. About 15 percent report that the pain shifts sides between bouts of attacks and, less often, during a bout, but never during a single attack.

What about the features of
trigeminal neuralgia?

By International Headache Society criteria, the duration of each paroxysm of pain has a duration of a fraction of a second to two minutes. In a prospective series of 158 patients with classical TN, the average age of onset was 52.9 years with 60 percent females affecting the right side of the face in 56 percent, left side 41 percent, and bilateral three percent.6 Pain was reported in the following distributions: V1, four percent; V2, 17 percent; V3, 19 percent; V1+V2, 10 percent; V2+V3, 33 percent; and V1+V2+V3, 13 percent. Thirteen percent had a more dull persistent pain at the onset of the disorder (“pretrigeminal neuralgia”) while 87 percent had stabbing paroxysmal pain. The paroxysmal pain was rated on average 10/10 by 58 percent of the patients. Forty-nine percent of the cohort reported concomitant persistent pain along with the paroxysmal pain.

Forty percent of patients suffered from more than 10 paroxysms of pain per day. Painful awakening at night because of pain attacks at least occasionally was reported by 49 percent. Trigger factors were reported by 91 percent including the following: chewing, 73 percent; touch, 69 percent; brushing teeth, 66 percent; eating, 59 percent; talking, 58 percent; and cold wind, 50 percent.

During attacks of pain, 31 percent of patients experienced ipsilateral autonomic symptoms, most commonly conjunctival tearing or injection. Of the surgery naïve patients, 29 percent had sensory abnormalities on exam, most commonly hypesthesia confined to the painful area of the face. Most patients (63 percent) had periods of remission with the average number per year of disease of .44 with 37 percent having months of remission and 63 percent experiencing years of remission. n

Reprinted with permission from Broca’s Area, the newsletter of the Texas Neurological Society. http://www.texasneurologist.org

1. May A, Evans RW. The sexagenarian woman with new-onset cluster headaches. Headache. 2011;51:995-8

2. Gaul C, Christmann N, Schröder D, et al. Differences in clinical characteristics and frequency of accompanying migraine features in episodic and chronic cluster headache. Cephalalgia. 2012 ;32(7):571-7.

3. Evans RW, Krymchantowski AV. Cluster and other nonmigraine primary headaches with aura. Headache. 2011;51:604-8.

4. Lai TH, Fuh JL, Wang SJ. Cranial autonomic symptoms in migraine: characteristics and comparison with cluster headache. J Neurol Neurosurg Psychiatry. 2009;80:1116-9.

5. Nesbitt AD, Goadsby PJ. Cluster headache. BMJ. 2012 Apr 11;344:e2407

6. Maarbjerg S, Gozalov A, Olesen J, Bendtsen L. Trigeminal Neuralgia – A Prospective Systematic Study of Clinical Characteristics in 158 Patients. Headache. 2014 Sep 18.