Abstract
Introduction
Short-lasting unilateral neuralgiform headache attacks with conjuctival injection and tearing (SUNCT) are an infrequent form of strictly unilateral headache accompanied by local autonomic signs.
The natural history of SUNCT is so far poorly understood, since the first case of the disease was described in 1989 by Sjaastad et al. (1), and few cases have been reported since. SUNCT is not reported in children; the mean age at onset was 50.7 years (range: 23–77 years) in a recent clinical review (2).
To our knowledge, no cases with onset during childhood have been reported so far. Here we describe a 10-year-old female affected by SUNCT.
Case report
A 10-year-old girl began, in November 1999, to suffer from painful attacks in the frontal area. The attacks were mostly right-sided; some of them (very few) were located on the opposite side. Pain was moderate to severe and stabbing in quality. The length of the attacks ranged from 2 to 180 s, being usually around 5–30 s. At the beginning (first 2 months) the attack frequency was extremely high: 10–180 per hour. However, the frequency of the attacks fluctuated during the day, showing two peaks (lasting 1 h each) of maximum frequency, in the morning during the first hour after wake-up and in the late evening. No attacks occurred during the night. Pain was accompanied by ipsilateral conjunctival injection, marked lacrimation and, sometimes, nasal obstruction. No obvious precipitating mechanisms of attacks were identified. She was pale and very tired after the attacks. During these 2 months, physical activity provoked an increase in attack frequency. Indomethacin (100 mg daily), as well as several other NSAIDs (aspirin, nimesulide, ketoprofen) and acetaminophen, were used, in an attempt to reduce the frequency of the attacks, without effect. After these unsuccessful therapeutic attempts, she did not take any other medication.
In January 2000, the frequency of the attacks spontaneously decreased to 4–8 attacks a day. They occurred usually in the afternoon and were followed by a nap. Afterwards, she was pain-free until the day after. During March 2000, short bouts of attacks were separated by remissions lasting 3 or 4 days. In the following 3 months the attacks almost disappeared (less than one a week).
Medical history was unremarkable, except for the occurrence of mononucleosis at the age of 4 years and chickenpox at 6 years.
Physical and neurological examinations were normal, as well as routine blood tests, sinus CT scan and brain MRI with gadolinium.
Discussion
The characteristics of the painful attacks in our patient—short-lasting, very frequent unilateral stabbing pain, accompanied by ipsilateral lacrimation and conjunctival injection—fit well with most features of the syndrome originally described by Sjaastad (1) and with the SUNCT diagnostic criteria recently proposed by Goadsby and Lipton (3).
SUNCT is usually considered a side-locked headache, even though a spreading of the pain to the contralateral side has been described in some cases (2). In our case, most attacks were right-sided; sporadically, however, they occurred on the other side. In addition, some attacks were shorter (2 s) than generally reported and, in some occasions, the frequency of the attacks (up to 180 per hour) was higher than previously described (4). These mild discrepancies with the usually described clinical picture do not question, in our opinion, the diagnosis of SUNCT, since the pivotal features of the disease (unilateral pain, short duration and high frequency of the attacks, autonomic signs ipsilateral to pain) were all present. On the other hand, it is very likely that the small number of cases reported so far do not exhaustively express the clinical variability of the disease. Moreover, this is the first case concerning childhood; therefore, we do not know if, at that age, some clinical characteristics are different from those observed in adults.
The presence of some (few) ultra-short attacks could raise the possibility of a co-occurrence of SUNCT and idiopathic stabbing headache, which may start in childhood (5). Even though we cannot discard with absolute certainty this hypothesis, it seems unlikely for the following reasons:
Except for duration, the attacks were stereotyped, including the occurrence of autonomic signs. Most attacks lasted 5–30 s and only a few of them exceeded these limits, either upward (up to 180 s) or downward (up to 2 s). Ultra-brief attacks were particularly perceived during peaks of very high attack frequency and seemed to be abortive crises, intermingled with longer attacks.
During a trial of indomethacin, ultra-short attacks did not disappear or diminish, as one would expect if we dealt with idiopathic stabbing headache, which is usually an indomethacin-sensitive headache (6).
The natural history of SUNCT is poorly understood: generally a periodic pattern has been reported, with active periods erratically alternating to remission phases. In our case, after an active period lasting 2 months, the disease substantially subsided even if it did not disappear completely. A prolonged follow-up of this patient with an early onset of the disease may be useful to better understand the natural course of SUNCT.