A boy with bilateral SUNA: A case report

Abstract

Background

Short-lasting unilateral neuralgiform headache attacks (SUNA) is a primary headache characterized by frequent attacks of severe headaches in association with ipsilateral cranial autonomic features. SUNA is defined as a strictly unilateral pain and bilateral cases are very unusual, so secondary causes should be searched for vigorously if there are bilateral symptoms. Despite a number of therapeutic trials, effective management for the majority of SUNA patients is not available at present. Management of SUNA is often difficult.

Case

We report the case of a young boy with bilateral SUNA attacks, with no detected underlying cause, who is responsive to indomethacin.

Conclusion

Rarely, primary SUNA can present with bilateral symptoms. According to our experience in this case, indomethacin should always be offered to patients with suspected SUNA.

Introduction

Short-lasting unilateral neuralgiform headache attacks (SUNA) is a rare trigeminal autonomic cephalalgia. According to ICHD-3 beta (1), diagnosis of SUNA requires short attacks of moderate or severe unilateral head pain, with orbital, supraorbital, temporal or other trigeminal distribution, lasting for 1 to 600 seconds and occurring as single stabs or series of stabs; attacks have a frequency of at least 1 a day for more than half of the time when the disorder is active. The pain attack should be accompanied by at least one cranial autonomic symptom ipsilateral to the pain; most often lacrimation and redness of the eye. To date, the patho-physiological mechanisms in SUNA remain uncertain.

In some patients, an underlying pathology such as a pituitary adenoma, sinusitis, meningioma and a severe basilar impression in association with osteogenesis imperfecta has been reported (2,3,4,5). To date, the patho-physiological mechanisms in SUNA remain uncertain.

SUNA attacks that simultaneously affect both sides have only been reported in three cases, and two of these had an underlying relevant pathology (6,7,8). Despite a number of therapeutic trials, satisfactory management of SUNA patients is often difficult (9). Here we report a case of a young boy with bilateral SUNA attacks, with no detected underlying cause, who is responsive to indomethacin.

Case

A 16-year old boy was referred to our tertiary headache center in March 2015 with symptoms that began in 2012 (at the age of 13) as bilateral severe pain, followed by autonomic symptoms that always occurred in the same pattern. The patient described the sudden onset of severe pain localized behind both eyes and in the forehead, with an intensity of 5–9 on the visual analogue 0–10 scale (VAS). A single attack lasted up to 10 seconds, and was immediately followed each time by severe tearing from both eyes (during the visit the boy had two typical attacks with pronounced tearing and conjunctival injection bilaterally). There were no trigger factors. Within the past 12 months the patient had experienced worsening of the frequency, with up to 60 attacks per day at the time he was referred to our center. Both treatments with sumatriptan nose spray applied twice daily over two weeks and the standard cluster treatment with oxygen inhalation had had no effect.

Past history: as a three month old child, the boy had been operated on for intestinal invagination; at the age of four he was treated with antibiotics because of a skin infection with streptococci, and at the same age he was treated for hay fever. The patient started having headache attacks that clinically resembled migraine without aura at the age of six; the frequency of attacks was one to two per month. A single attack lasted half a day, and all attacks were successfully treated with simple analgesics. These headache attacks ceased at the age of 14; at that time his other type of headache had already begun. The patient does not drink alcohol nor smokes, does not take any kind of drugs and is active in sports. He lives with his parents, who are healthy; he denies any problems in the household or at school, except for the troublesome everyday headache attacks.

The neurological examination was normal

Work-up

The MR scanning with angiography was unremarkable and revealed no neurovascular contact, nor any pituitary abnormality. Laboratory findings, which included routine blood samples, and endocrinology tests FSH, HS, corticotrophin, cortisol, LH, testosterone, TSH, T3, and T4 were completely normal. An ECG revealed bradycardia with a prolonged QT interval, with no clinical implications. Opthalmological examinations were unremarkable.

As initial treatment, the patient was given indomethacin 25 mg and was recommended to increase the dose from two to six tablets per day with an adjuvant proton pump inhibitor tablet once per day. In the follow-up visit two months later the patient had experienced a remarkable improvement, having only seven to eight (occasionally more than 10) attacks daily. The pain attacks were milder, and the autonomic symptoms had completely disappeared. The symptoms subsided with only 50 mg of indomethacin; the patient chose not to proceed with a higher dose due to possible side effects. The patient was also offered treatment with lamotrigine, but chose not to start with this medication because he was satisfied at that time with the indomethacin treatment. The patient had two controls, and after 10 months of follow up he still responded satisfactorily to indomethacin and did not want to discontinue the treatment.

Discussion

We present here the case of a 16-year-old boy with short lasting attacks of pain behind both eyes and in the forehead, accompanied by pronounced tearing and responsive to indomethacin. This is the first reported case of a SUNA syndrome in such a young patient (the symptoms appeared when he was 13 years old); further, the attacks are bilateral. Our patient fulfils all of the criteria for the SUNA syndrome, except part of the B criterion, unilaterality. Criterion B requires severe, unilateral pain, with orbital, supraorbital, temporal and/or other trigeminal distribution, lasting for 1–600 seconds and occurring as single stabs or in a saw-tooth pattern. Criterion C requires at least one cranial autonomic symptom ipsilateral to the pain. For 3.3.1. SUNCT (Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), both conjunctival injection and lacrimation should be present, while in 3.3.2. SUNA (short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms) only one of or neither conjunctival injection and lacrimation should be present. SUNCT may be a sub-form of SUNA (1).

As a differential diagnosis, we can consider primary stabbing headaches, which is a type of headache where pain can occur bilaterally and which is responsive to indomethacin, though it is never accompanied by pronounced autonomic symptoms. Trigeminal neuralgia can be considered as a differential diagnosis, though there were no trigger factors, and pain is usually unilateral and not accompanied by autonomic symptoms. The main implication of our paper is that a SUNA-like headache can be presented bilaterally.

Until now there have been only three reports of bilateral SUNA, two of which had an underlying pathology (6,7,8). Despite a thorough work-up, we have not found an underlying pathology in our patient.

SUNA itself is a very rare primary headache, for which the etiology is not yet understood. Neuroimaging studies have substantially advanced our understanding of the mechanisms underlying primary headache syndromes. Functional neuroimaging studies in trigeminal autonomic cephalalgias, including SUNA, report specific activation of the hypothalamus in association with the headaches (10). Furthermore, structural imaging studies report that a significant proportion of patients have an aberrant vascular loop in contact with the ipsilateral trigeminal nerve (11), like neurovascular contact can be found in patients with trigeminal neuralgia (12). Therefore, it has recently been suggested that SUNA and trigeminal neuralgia may constitute a continuum of the same disorder, rather than separate clinical entities (13). It seems that in symptomatic SUNA cases, the most frequent underlying pathologies are pituitary adenomas and/or vascular contact with the trigeminal nerve (4,2,8,11,13).

Despite a number of therapeutic trials, effective management for the majority of SUNA patients is not available at present, and some patients remain refractory to any kind of treatment. Based mostly on open-label trials, some patients seemed to benefit from some pharmacological or invasive procedures. Reports of responsiveness to gabapentin (7) or lamotrigine (8) have been published, and some patients may respond to oxcarbazepine and topiramate (6,14). At present, the drug of choice for SUNA seems to be lamotrigine followed by gabapentin and topiramate; in some cases dual antiepileptic therapy will give relief to the patient (15). During the worst periods, intravenous lidocaine may decrease the flow of SUNA attacks (16), and a case responsive to clomiphene citrate has been published (17).

Although indomethacin is the drug of choice in a subset of trigeminal autonomic cephalalgias, as well as in Valsalva-induced headaches, primary stabbing headache, and hypnic headache, to date there have been no reported cases of proven efficacy for indomethacin in SUNA patients. Due to a persistent response to indomethacin in our SUNA patient, we do not agree with the proposed recommendation that the lack of response to indomethacin is useful in diagnostic terms as suggested in one study (18).

Invasive therapy should only be offered as a last resort, since no convincing controlled results have been obtained as yet. Procedures such as bilateral occipital nerve stimulation have been shown to be an effective and safe treatment option in nine patients (19), while superior cervical ganglion opioid blockade has given relief in one patient (20). The two SUNA patients who underwent deep brain hypothalamic stimulation obtained substantial and persistent relief (21,22).

In conclusion, our case report is unusual due to the fact that SUNA syndrome occurred in a 13-year-old boy, symptoms were bilateral and responded well to indomethacin. This case report adds to the present knowledge of such a rare syndrome.

Clinical implications

Improvement in diagnostics: SUNA syndrome can appear bilaterally.

Improvement in diagnostics: SUNA can appear in young patients.

Improvement in treatment: in the treatment of SUNA, indomethacin should be considered.