Both trigeminal autonomic cephalalgia and trigeminal neuralgia in a child

Abstract

Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are an uncommon syndrome [1] similar in frequency, duration, setting and severity to short-lasting unilateral neuralgiform headache attack with conjunctival injection and tearing (SUNCT syndrome). SUNCT and SUNA belong to a group of headache syndromes classified as Trigeminal Autonomic Cephalalgias (TACs) and were included in the second edition of the International Classification of Headache Disorders [2]. Some studies have focused on the association between Trigeminal Neuralgia (TN) and TACs with short-lasting attacks [3]. To our best knowledge, no cases of SUNA in childhood have been reported so far. We describe a child with a SUNA syndrome associated with TN probably triggered by inflammatory lesion of nasal mucosa. In April 2006, a 9-year-old child with Norrie disease [4] became agitated and complained of facial pain. Those attacks persisted for approximately half an hour, had a frequency of 2–3 per day and were refractory to NSAIDs. A few days before the beginning of attacks, the patient presented purulent rhinorrhea. An otorhinolaryngoiatric examination evidenced a piece of a toy into the right nostril. The toy was removed but it did not cure the pain. Ophthalmologic examination, brain magnetic resonance imaging, dental scan and EEG were normal. From September to November 2006, the child was treated with Pregabalin and then with Topiramate, without benefit on pain. At the time of our observation, in November 2006, the pain started to be well localized: it involved the forehead, the orbital and temporal region, prevalently on the right-side. Two types of attack were observed: single stabs (clusters with length of attack in range from a few seconds to 5 min) and another one lasting about 30 min. Some of the pain accesses were self-controlled by the patient by massaging the painful area. He had two to three attacks every day, with alternate prevalence of side: pain was accompanied by ipsilateral conjunctival injection (but not tearing), rhinorrhea, eyelid oedema and sweating. The patient was treated with various drugs (Pregabalin, Carbamazepine, Gabapentin), but only the association of Gabapentin and Lamotrigine modified the type of painful attacks. In fact, attacks affected the second division of the trigeminal nerve (V2) without cranial autonomic symptoms. Most of the attacks were still right-sided, lasting from a fraction of a second to 2 min, showing TN characteristics [2]. In June 2007, the accesses became more frequent and intense. The pain accesses were spontaneous or were triggered by the wind, chew, brushing, hot or cold feed. Oxcarbazepine (1200 mg/die) associated with Lamotrigine (200 mg/die) was introduced with a gradual discontinuation of Gabapentin, showing an almost complete remission. Reduction of therapy caused the reappearance of attacks so, at the moment, the child continues this therapy showing sporadic episodes of pain. The headache of this child fulfils the diagnostic criteria for chronic SUNA [2]. He has had more than 20 attacks R. Valeria P. Concetta P. Mariotti (&) Unit of Child Neuropsychiatry, Catholic University, L.go A. Gemelli, 8, 00168 Rome, Italy e-mail: pm@mclink.it