Abstract
Case report
We first saw this 11-year-old girl 7 months after she developed extremely severe, sharp, unilateral, side-locked headaches. The headache is located in the back of the eye, with occasional irradiation to the ipsilateral temple. The pain usually lasts from 40 to 80 s. Since the very beginning of these headaches, she has had from 20 to 30 attacks per day. The pain could not be precipitated from trigger areas or by trigger points. Migraine triggers were also not identified. Between the attacks she is completely symptom-free, although periorbital tenderness was sometimes reported. No refractory period was identified. She denies any premonitory features or auras preceding the headache, which begins abruptly. The attacks are extremely disabling and are associated with profuse tearing, strictly on the ipsilateral eye. She does not have other autonomic features and, specifically, she has no redness of that eye. Headache attacks were observed in the first and subsequent consultations by two of us (M.V., S.J.T.). We have followed her for 6 months.
In the last month she reports increasing severity of headache. She now has an average of 20 headache attacks per day. Occasionally she reports attacks lasting from 15 to 30 min. Currently she is severely disabled. Her disability score, as assessed by the Headache Impact Test (HIT-6), is 63 (severe disability).
Her past and family medical histories are unremarkable. Laboratory investigations included neuroimaging (contrast-enhanced computed tomography scan and contrasted brain and transition magnetic resonance imaging of the head, both normal). Routine blood work, biochemistry, coagulation studies, erythrocyte sedimentation rate, C-reactive protein, and protein-electrophoresis were unremarkable. Serological tests for rheumatoid factor, antinuclear antibodies, anti-DNA, anti-sm, and anti-ro antibodies were negative. Serum complement was also normal.
The primary diagnostic hypothesis was paroxysmal hemicrania. She was treated with indomethacin 25 mg t.i.d., for 2 months (3 mg/kg per day). She had absolutely no response to indomethacin. She has also been treated with amitriptyline 50 mg (2 mg/kg per day), valproic acid 125 mg t.i.d. (15 mg/kg per day), aspirin plus caffeine, acetaminophen, ibuprofen, mefenamic acid. None of these treatments provided consistent relief. She reported minimal control with gabapentin.
Discussion
The trigeminal autonomic cephalgias (TACs) are a group of primary headache disorders characterized by unilateral pain in the trigeminal nerve distribution, associated with ipsilateral cranial autonomic features (1, 2). This group of headache disorders includes cluster headache, paroxysmal hemicrania and short-lasting, unilateral, neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome) (1, 2) (Table 1). Despite their similarities, these disorders differ in their clinical manifestations and response to therapy (3).
The trigemino-autonomic cephalgias according to the second edition of the International Classification of Headache Disorders (2004)
SUNCT is a rare condition clinically characterized in 1989 (4). SUNCT is slightly more common in men (4, 5), and the mean age at onset is 50 years (6, 7). It is characterized by strictly unilateral attacks, side-locked in 88% of cases, more severe in the orbital or periorbital regions, forehead and temple (7–9). The pain is usually severe and neuralgiform. The typical duration ranges from 5 to 250 s, although the reported range of duration is 2 s to 20 min (9). Episodic and chronic forms of SUNCT have been described (9). The attack frequency varies from less than one attack daily to more than 60 attacks per hour (10). The attacks have a bimodal distribution, predominantly diurnal; only 1.2% of the attacks occur at night. Although unusual, paroxysms in SUNCT may overlap into a clinical status (11, 12). The remissions last for a few months, but can last from 1 week to 8.5 years (7–9).
Autonomic features are always very prominent in SUNCT. Case series show that virtually all attacks are accompanied by ipsilateral conjunctival injection (100%) and lacrimation (94%). Ipsilateral rhinorrhoea (54%), nasal congestion (48%), eyelid oedema (26%), ptosis (12%), miosis (4%), facial sweating (8%) and redness (2%) have also been reported (6–8).
By definition, the SUNCT classification criteria require conjunctival injection and tearing. Cases of SUNCT syndrome without conjunctival injection or tearing may have been erroneously mislabelled as other syndromes and thus may be underreported in the medical literature. The International Headache Society classification (ICHD-2) addresses this issue in appendix A3, by defining a syndrome of short-lasting, unilateral, neuralgiform headache attacks with cranial autonomic symptoms (SUNA) without both conjunctival injection and lacrimation (Table 2) (1).
Classification of SUNA according to the second edition of the International Classification of Headache Disorders (2004)
To the best of our knowledge, case reports of SUNA have not been published (P. J. Goadsby, personal communication). Here we report a case fulfilling ICHD-2 criteria for SUNA. Interestingly, although SUNCT predominates in middle-age men, our patient was a female child. We excluded secondary headaches by appropriate investigations, and paroxysmal hemicranias based on the lack of response to indomethacin. Additionally, the patient does not have trigger points, and the pain duration is longer than that usually observed in trigeminal neuralgia (13, 14). Primary idiopathic stabbing headache is another differential diagnosis we considered. However, in 80% of cases with this diagnosis, the pain lasts less than 2 s, and in 40% of the cases, patients report other migraine features (2). Additionally, the pain is usually (but not always) responsive to indomethacin. Cranial autonomic features are absent in this syndrome (3, 15).
As a cautionary note, the ICHD-2 presents in the appendix research criteria for a number of novel entities that have not been sufficiently validated by research studies (1). It is anticipated that a number of the disorders now in the appendix will move into the main body of the classification next time it is revised. Our patient also fulfils criteria for probable SUNCT (ICHD-2 code 3.4.3).
The TACs are a relatively poorly recognized group of primary headaches disorders. In this case report, we present a young female patient that fulfils ICHD-2 criteria for SUNA, a disorder just recently described and still poorly understood.