Recently, I’ve had a spate of patients with extraordinarily painful short-duration headaches, who were all seen by general neurologists and came to my practice for a second or third opinion. In the spirit of light summer reading, I review 3 recent cases, describing the salient features, the diagnoses previously given, my diagnosis, and a treatment plan. Play along at home (or at the beach, up in the mountains, or wherever the warm weather finds you). See if you can figure out the correct diagnosis (no reading ahead!) and whether or not you agree with my diagnoses and treatments. If you don’t, let me know what you think (peter@neinh.com), and in a future column I will post your replies.

Case Histories

Case 1. Jaime’s Ever-Present Pain

Jaime is age 46 and married with 4 children. She reports no headache history until 2 years ago, when she developed attacks of sharp severe pain about her right eye, often radiating to the temple and occasionally down her right posterior neck (in fact, certain neck positions seemed to trigger an attack). Rarely, her pain would start in her right upper teeth and gums. Initially, she said the pain was present “all the time,” but detailed probing revealed she averaged 20 to 30 severe pain attacks of 5 to 10 minutes per day. Between these, she had a dull, bruised sensation. Jaime denied autonomic features, but when I viewed photos of her during attacks, I could make out subtle right eyelid edema and droop but no conjunctival injection. Jaime denied nasal congestion. Every now and then she was pain free, but that never lasted more than a few days. Jaime had seen a neurologist who diagnosed her with chronic migraine (CM) and prescribed propranolol (20 mg twice daily). After Jaime experienced severe hypotension while taking propranolol, her treatment was changed to zonisamide (up to 150 mg/daily), but this was not effective and was discontinued. Finally, onobotulinumtoxinA injections (2 cycles of 155 units) were tried—also without success.

Case 2. Lloyd’s Countless Lancinating Attacks

Lloyd is age 39, lives with his partner, and works as a television news producer. Referred by a local neurologist for potential enrollment in our study on chronic cluster headache (CH) treatment, Lloyd said his headaches began 6 years ago, as lancinating shocks of pain exclusively on his left upper face, eye, and temple. His left eye felt scratchy and raw, as if sand had blown into it, and the white of the eye looked crimson and watery more often than not. Lloyd’s pain would last 5 to 30 seconds and occurred countless times per day. As he sat across from me, Lloyd would stop midsentence and wince, eye shuttered, then resume talking. He told me his primary care doctor diagnosed trigeminal neuralgia (TN), and when he did not respond to carbamazepine and gabapentin, referred him to a neurologist. The neurologist diagnosed CH and after unsuccessful treatment trials of verapamil and sumatriptan nasal spray, referred Lloyd for possible enrollment in our study.

Case 3. Mel’s Posttraumatic Agonies

Melvin, age 82, is a retired dentist married to his high school sweetheart. The couple has 3 grown children and 7 grandchildren. Melvin’s medical history is complex, including diabetes mellitus, chronic kidney disease, ischemic heart disease (he had a 5-vessel coronary bypass 3 years ago), and atrial fibrillation treated with warfarin. When vacationing in Rome, Mel tripped while taking photos and tumbled down the Spanish Steps, striking his face, knocking out 3 teeth, and fracturing his left orbit. About 1 week later he awoke from sound sleep with agonizing discomfort deep behind his left eye socket, unlike any pain he ever experienced. It drove him from bed, and he paced the darkened hall as his wife slept peacefully. Accompanying the pain, was a socked-in sensation in his nose and left sinus. In the bathroom he turned on the light, frantically searching for over-the-counter painkillers. When he gazed in the mirror over the sink, Mel was startled to discover a crying eye, at half mast, red as an Empire apple. Although Mel was not a particularly religious man, he thanked the heavens when the agony resolved, as abruptly as it had begun, in about 1 hour. When an identical spell gripped him the next night, he sought medical care. Diagnostic studies showed normal findings of brain MRI, MR angiography, and MR veno-graphy. Imaging with CT showed the healing orbital fracture and nothing else. Mel’s blood test results were all normal. His headache frequency increased from nightly to 2 to 3 times per day, attacks lasting 30 to 75 minutes. Mel’s primary care physician assistant thought he had TN, and prescribed first small doses of baclofen, which had no effect, then carbamazepine, which caused intolerable side effects. Mel went to a neurologist, who diagnosed postconcussion syndrome (although Mel’s only concussion sign or symptom was the headaches) and prescribed acetaminophen and physical therapy.

Diagnosis and Treatments

Case 1. Jaime’s Ever-Present Pain

My diagnosis. Chronic paroxysmal hemicrania.

Treatment. Initially, 25 mg of indomethacin was prescribed but produced no response; however, 75 mg 3 times per day resolved the pain completely. On this regimen, even with a proton pump inhibitor, Jaime developed gastrointestinal distress that was intolerable. She now takes indomethacin 25 mg in the morning and 75 mg at dinner. She adds a 50 mg suppository as needed when attacks are particularly intense. Jaime describes this regimen as 75% effective.

Comment. Paroxysmal hemicrania (PH) is a rare primary headache (Table). In the family of trigeminal autonomic cephalalgias (TACs), PH is important to recognize because it is exquisitely responsive to indomethacin. Other treatment options include verapamil, topiramate, and lithium.1-3

Case 2. Lloyd’s Countless Lancinating Attacks

My diagnosis. Severe unilateral neuralgiform pain with conjunctival injection and tearing (SUNCT)

Treatment. Clearly Lloyd was not a candidate for our CH study. I suggested several treatment options to him and his referring neurologist, and he was lost to follow up.

Comment. Also TACs, SUNCT and its cousin, short lasting neuralgiform headaches with cranial autonomic symptoms (SUNA) are rare, with a prevalence of 6.6 per 100,000 persons and an incidence of 1.2 per 100,000.3-5 Distinguishing features of SUNCT include very short duration side-locked excruciating pain attacks with a reddened eye, tearing, or both. Typically, attacks occur from 10 to 400 times per day, and unlike CH, there is no circadian pattern. The first division trigeminal nerve distribution, prominent autonomic features, and lack of refractory period readily distinguish SUNCT from TN.3

Case 3. Mel’s Posttraumatic Agonies

My diagnosis. Posttraumatic headache with cluster headache phenotype.

Treatment. After neither occipital nerve blocks nor oxygen proved effective, we worked with Mel’s cardiologist to slowly introduce and titrate verapamil to a maximally tolerated dose of 280 mg per day. Triptans were contraindicated, so we tried combined therapy with melatonin 10 mg, intranasal lidocaine, a noninvasive vagus nerve stimulator, and CBD/THC drops at night. Mel has received a loading dose of galcanezumab and will continue monthly injections. We were awaiting Food and Drug Administration (FDA) approval of an implantable sphenopalatine ganglion (SPG) stimulator, which is now indefinitely delayed. On this regimen, Mel’s attacks are down to 2 to 3 per week. He has joined the online support group Cluster Busters and is contemplating a trial of psilocybin if he can access it.

Comment. Most posttraumatic headaches present with a migrainous phenotype, followed by a tension-type pattern. Posttraumatic cluster headache presentation is rare but has been reported.6 Initial failure to recognize the correct phenotype deprived Melvin of targeted specific therapy. Even when recognized, his medical comorbidities limited aggressive treatment (eg, triptans contraindicated; verapamil dose limited), but with combination therapy Melvin’s attacks have decreased from 30 to 60 per month to 8 to 12 per month

Discussion

Trigeminal autonomic cephalalgias can be vexing, difficult to recognize, and hard to treat (Table). Nevertheless, the well-versed general neurologist should be up to the task of diagnosing and at least taking a stab at evidence-based treatment before referral to a headache center. Here are some TAC truisms to keep in your back pocket and pull out as needed:

  • TACs are moderate-to-severe-to-excruciating short-lasting unilateral (side-locked) primary headaches
  • TACs may be episodic or chronic, defined by whether there are pain-free intervals of predetermined length (not how many days per month attacks occur, as in migraine)
  • Some TACs are exquisitely responsive to indomethacin, so make sure you can recognize these., and when unsure, try indomethacin anyway
  • Ipsilateral autonomic features are common but may be subtle
  • The shorter the name, the longer the symptom duration—cluster, a short name, has the longest duration of the TACs, and short-acting unilateral neuralgiform pain with conjunctival injection and tearing (SUNCT)—a mouthful—has the shortest. (Rule devised by Larry Newman MD, professor of neurology at New York University and past president of the American Headache Society)
  • Treatment of TACs may be difficult, but options are available and rational polypharmacy is usually the rule rather than the exception

I hope you got all of the cases presented correct, and cheers to you if you did. If not, I have provided a few insightful references below to reacquaint you with these menaces we call TACs. Think of it as light summer reading on the beach, in the mountains, or wherever the sultry summer air finds you. Happy summer!

1. Sjaastad O. Chronic paroxysmal hemicrania. In: Vinkin PJ, Bruyn GW, Klawans HL, Rose FC. (eds). Handbook of Clinical Neurology. Amsterdam; Elsevier Science; 1986;48:257.

2. Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain. 1997;120(1):193.

3. Headache Classification Committee of the International Headache Society. The International Classification of Headache Disorders, 3rd ed. Cephalalgia. 2018;38:1.

4. Arroyo AM, Durán XR, Beldarrain MG, Pinedo A, García-Moncó JC. Response to intravenous lidocaine in a patient with SUNCT syndrome. Cephalalgia. 30(1):110-112.

5. Pomeroy JL, Nahas SJ. SUNCT/SUNA: a review. Curr Pain Headache Rep. 2015;19(8):38.

6. Lambru G, Chan CK, Matharu MS. Post-traumatic cluster headache: a clinical phenotype study of 16 patients. J Headache Pain. 2013;14(Suppl 1):46.