SUNCT syndrome in two patients with prolactinomas and bromocriptine-induced attacks

Abstract

We report two patients with prolactin-secreting adenomas, SUNCT (short-lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing) syndrome, and bromocriptineinduced attacks.Case reports. Patient 1. A 40-year-old woman had her first headaches at age 27. The attacks consisted of a severe burning pain in the left parietal area, lasting 15 to 30 seconds. This was accompanied by conjunctival injection and tearing of the left eye, triggered by physical exercise or sexual activity. Three years later an amenorrhea-galactorrhea syndrome occurred. A CT scan revealed a pituitary adenoma, with suprasellar and left lateral extension, invading the left cavernous sinus (figure). Prolactinemia was 100 ng/mL (normal range, 9 to 20 ng/mL), without other endocrine abnormalities. An adenomectomy was performed but the tumor could not be removed entirely. Postoperatively, the prolactinemia normalized, then increased again during the following months. Bromocriptine treatment was started. Two hours after the first oral dose of 1.25 mg, the patient experienced repetitive severe headache attacks for 48 hours straight that were similar to those she had previously. The same phenomenon occurred after the intake of 0.25 mg lisuride and 0.5 mg cabergoline. After IM injections of 50 mg long-acting release bromocriptine, the attacks persisted for 7 days and were not prevented by carbamazepine or domperidone administered before the injections. After radiotherapy of the pituitary area and the left cavernous sinus was performed, the headaches became rare. Prolactin serum levels increased again after 2 years, although MRI showed total regression of the tumor. The patient was prescribed 75 mg/d quinagolide, a nonergot inhibitor of prolactin secretion. This did not trigger the neuralgiform attacks, and prolactinemia normalized after 6 months.Patient 2. A 24-year-old woman experienced for 4 months short attacks of severe left periorbital pain, lasting 15 to 30 seconds. This was accompanied by redness and tearing of the left eye, and a sensation of left nasal obstruction. These attacks usually occurred five to 10 times per hour for 2 or 3 hours, several times per week, and could be triggered by mild efforts such as bending or standing up. Four years later, she reported amenorrhea, and MRI showed a pituitary microadenoma with a left lateralization. Prolactinemia was 57 ng/mL. Bromocriptine treatment was started, and 3 to 4 hours after the first oral dose of 1.25 mg, she experienced a series of severe headache attacks for several hours that were similar to those she had previously. Bromocriptine treatment was interrupted and replaced by lisuride, which also triggered the attacks. A thyroid-stimulating hormone test to study the prolactin secretion was performed on three occasions, and each time induced severe bouts of attacks. Lisuride treatment was stopped after 1 year, and she experienced only rare headaches afterwards, usually triggered by movements. She underwent surgery for the microadenoma 10 months later.Discussion. T...

SUNCT and high nocturnal prolactin levels: some new unusual characteristics

“…We found that the rise of serum PRL during attacks was not accompanied by a TSH rise. This case, along with other similar reports in the literature, supports the hypothalamic connection to this trigeminal autonomic cephalalgia [7]. The pathophysiology of SUNCT is unknown, though fMRI studies in attacks have documented activation of the ipsilateral hypothalamic grey [5].…”

“…Therefore, dopaminergic hypofunction could be the consequence of serotoninergic hyperfunction, because of the inhibitory effect of serotonin on dopamine neurons. In fact, in patients with SUNCT the treatment with dopamine-agonists can determine various responses, with worsening in some cases [7] and improvement in others [11,12]. Most cases of SUNCT syndrome are primary but several cases of SUNCT are secondary and occur in patients with pituitary tumours, further supporting involvement of the hypothalamic-hypophysial axis.…”

High prolactin levels as a worsening factor for migraine

“…This evidence shows that a tumour mass is not correlated to the presence or intensity of the headache, thus, the cause of the headache lies in something other than the volume of the tumour. The same concept was also considered by other authors who described the presence of headache in patients with microprolactinoma [15,16]. Referring to the type of headaches related to prolactinomas, the articles published up to now have cited cases of trigeminal autonomic cephalalgias (TACs) [15], short lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) [16,17] and migraine in only one case [17].…”

Influence of lamotrigine over the sunct syndrome: one patient follow-up for two years

“…The etiology is unknown, but recently cases secondary to cerebellopontine angle arteriovenous malformations 12,13 , brain stem cavernous hemangioma 14 , a posterior fossa lesion in a patient with AIDS 3 , prolactinomas 15 , and corneal lesion 16 have been described.…”