Abstract
Introduction
SUNCT syndrome was described by Sjaastad et al. in 1989 (1). This condition is characterized by unilateral paroxysm with two components: periorbital pain and marked autonomic symptoms such as conjuntival injection, tearing and rhinorrhea. Single attacks last 10–60 s. Spontaneous remission can be followed by symptomatic phases when the attack frequency ranges from 20 to 80 per day (2, 3).
SUNCT syndrome is an uncommon form of the trigeminal-autonomic cephalalgias (TAC) (4). Although in most reported patients there is no association with an intracraneal pathology, various cases have been described as symptomatic SUNCT syndrome (5–8).
We report a patient with SUNCT syndrome and unilateral seborrheic dermatitis associated with brachycephaly.
Case report
A 24-year-old woman was sent to our neurology service with a 2-month history of headache. At the same time, her family realized that her face became red and greasy
The painful attacks were characterized by intense, lancinating jabs. They were unilateral and located in the right periorbital region corresponding to the first division of the trigeminal nerve. Sometimes the pain spread to the right upper lip. The attacks were accompanied by ipsilateral decreased palpebral fissure, lacrimation and conjuctival injection. During the paroxysms nasal stiffness was also evident but the patient didn't have rhinorrhea. The associated autonomic signs started and finished with the attacks. Bouts lasted for about 60 s with a frequency of 50 per day. Attacks could arise spontaneously or be triggered by stimuli in the trigeminal or extratrigeminal areas.
Neurological examination was normal except for a hypesthesia within the right V1 area. The right corneal reflex was reduced. In the general examination there was redness and severe scaling confined to the right side of the forehead, eyebrow, upper part of the cheek and right side of the nose and upper lip. The erythema was sharply limited at the mid-line. The appearance was typical of seborrheic dermatitis. Cultures were carried out from the skin of the right side of the face and Pityrosporum ovale was isolated. A brain computed tomography and magnetic resonance imaging showed a craniosynostosis consistent with brachycephaly. There was a relative enlargement in the transverse compared with the longitudinal diameter of the skull. The posterior cranial fossa was foreshortened without tonsillar herniation. The basal cisterns were markedly obliterated (Figs 1 and 2).
Sagital T1-weighted MR image showing a reduction in the longitudinal diameter of the skull consistent with barchycephaly.
Axial T2-weighted MR image depicting a foreshortened posterior fossa. Note the subarachnoid space obliterated, more notable in the right cerebello-pontine angle (arrow).
The patient was treated symptomatically with carbamazepine (800 mg/day), lithium carbonate (400 mg/day) and prednisone (75 mg/day) with progressive reduction of the attacks in 2 weeks; there was also a disappearance of the seborrheic dermatitis. For 10 months the patient remained asymptomatic taking lithium and carbamazepine. One year after, both drugs were withdrawn without any recurrence of the paroxism. The skin became entirely normal. The patient, after 2 years of follow-up has been without pain.
Discussion
Craneosynostosis is the result of premature fusion of one or more cranial sutures. Such conditions are estimated to occur in 1 in every 1000 births. The growth of the cranium is inhibited in a perpendicular direction to the involved suture, with a compensatory enlargement in other dimensions allowed by the patent sutures (9). The nervous system is usually normal in these conditions but a reduction in the volume of the cranium may result in an increasing of the intracranial pressure (10). In our case, the patient had a brachycephaly, with a head excessively wide due to premature bilateral closure of the coronal suture.
Our patient had a suggestive clinical picture of SUNCT syndrome. The diagnosis of trigeminal neuralgia (TN) is very unlikely because of the significant autonomic disturbance and the long duration of the pain symptoms (11). A diagnosis of cluster headache (CH), cluster-tic syndrome, as well as other TAC, is highly improbable from the clinical picture. However, the patient was treated with drugs effective in CH and TN with a partial effect and a progressive resolution of the pain, suggesting similarities between them (4, 12).
The aetiopathogenesis of TAC, including SUNCT syndrome, is unknown. It has been proposed that the activation of the trigeminal-autonomic reflex (TAR) would be the explanation of the features of the SUNCT syndrome. The TAR is defined by the trigeminal nerve and its functional connection to the parasympathetic neurones of the facial nerve. Goadsby and Lipton have suggested a pathophysiological explanation of the SUNCT syndrome. First, several triggering mechanisms may induce a trigeminal irritation; this accounts for the headache. Secondly, the signs of parasympathetic over-activity, such as lacrimation, conjuntival injection or rhinorrhea, are due to activation of the TAR throughout the superior salivatory nucleus (4). However, a dysfunction in the hypothalamus during a SUNCT attack has been recently reported (12).
The mechanism of SUNCT syndrome due to brain-stem pathology is unclear. Although it is admittedly possible that the presence of craneosynostosis and SUNCT syndrome in this patient was coincidental, we suggest that the clinical picture is the consequence of a transient rise in intracranial pressure leading to the irritation of the trigeminal nerve in a narrow cerebellopontine angle. Afterwards, the activation of the TAR generates the typical semeiology of SUNCT syndrome. On the other hand, the autonomic hyperactivity also accounts for the sebhorreic dermatitis of the patient.
Seborrheic dermatitis is characterized by areas of erythema and greasy scale on the face; these features were well illustrated by our patient. The question of whether the sebaceous glands are under direct nervous control still remains in doubt. However, several seborrhoea cases have been described following a nerve lesion (13) or other unrelated neurological disorders such as Parkinson's disease (14). We believe that the seborrheic dermatitis might have resulted from a parasympathetic lesion as some authors attribute an increase of sebum excretion to the hyperactivity of the parasympathetic system (15, 16).
In conclusion, our case shows again that TACs can be secondary to a lesion in the posterior fossa. As is well known, we also recommend that all patients with a SUCNT syndrome undergo MRI of the brain to screen for structural lesions.