SUNCT syndrome associated with pituitary tumor: case report

Abstract

-For twelve years, the subject of this report, a 38-year-old man, presented a clinical condition compatible with the SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) syndrome. He presented a stabbing and intense daily pain located in the left pre-auricular and temporal regions. Each of these intense pain attacks lasted around one minute and presented a f requency of two to eight times per day. The pain was associated with ipsilateral lacrimation, conjunctival injection and rhinorrhea. MRI revealed a pituitary tumor with little suprasellar extent. The subject's serial assays of prolactin, GH, TSH and ACTH were within normal levels. Following transsphenoidal hypophysect o m y, with complete removal of the tumor, the subject no more presented pain. The pathological diagnosis was non-secreting adenoma. Fourteen months after the surgery, he remains symptom-free.KEY WORDS: SUNCT, pituitary tumor, surgery, ultra-shorting headaches.Síndrome SUNCT associada a tumor de hipófise: relato de caso RESUMO -O paciente relatado neste artigo apresentou uma condição clínica compatível com síndro m e SUNCT (cefaléia de curta duração, unilateral, neuralgiforme com hiperemia conjuntival e lacrimejamento). Ele referia dor diária, intensa, em facada, localizada na região pré-auricular e temporal esquerd a s . Cada ataque de dor permanecia por cerca de um minuto, com freqüência de duas a oito vezes por dia. A dor se acompanhava de lacrimejamento ipsolateral, congestão conjuntival e rinorréia. A RM mostrou um tumor de hipófise com pouca extensão suprasselar. Dosagens de prolactina, GH, TSH e ACTH estavam em níveis normais. Foi então submetido a hipofisectomia transesfenoidal com remoção completa do tumor após o que a dor cessou completamente. O diagnóstico anátomo-patológico foi adenoma não secre t o r. Quatorze meses após a cirurgia, o paciente permanecia livre de dor. PALAVRAS-CHAVE: SUNCT, tumor de hipófise, cirurgia, cefaléia de curta duração. s y n d rome is the poor response to pharm a c o l o g i c a l t re a t m e n t s 3 , 4 . There have been re p o rts of impro v ement upon the use of amitriptyline, carbamazepine, gabapentin, prednisone, topiramate 5 , lamotrigine 6 , nifedipine and sumatriptan 4 . Anesthetic blockades do not work very well, although there have been re p o rts of improvement following the local opioid blockade of the superior cervical ganglion 7 . There have also been re p o rts of surgical pro c e d u res that worked eff e c t i v e l y 5 while others re p o rts have indicated a lack of response to such procedures 8 .This syndrome has been listed in the second edition of The International Classification of Headache D i s o rders together with trigeminal autonomic cephaThe SUNCT syndrome (short-lasting unilateral neur a l g i f o rm headache attacks with conjunctival injection and tearing) is an uncommon headache characterized by moderate to severe pain. Usualy, the locations with the most severe pain are the ocular/periocular regions and the fronta...

Lidocaína endovenosa no tratamento da síndrome SUNCT secundária a tumor de hipófise: seguimento de uma paciente por um período de quatro anos. Relato de caso

“…In this case, transsphenoidal hypophysectomy to treat pituitary macroadenoma has resulted in transient pain improvement. This fact contrasts with the report of other author who has observed pain relief with the surgical procedure 4 . There are different hypotheses to explain pituitary tumor-induced pain.…”

SUNCT and high nocturnal prolactin levels: some new unusual characteristics

“…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. There are seven cases reported of SUNCT syndrome secondary to pituitary adenomas in the medical literature, of which four cases had prolactinomas [10][11][12], two non-secreting macro-adenoma [13,14] and one case had micro-adenoma linked to acromegaly [15]. In all the case reports with prolactinoma, the tumour was ipsilateral to the side of the attacks, producing a possible mass effect or mechanical mode of action, which may have played a role in SUNCT onset.…”

SUNCT syndrome: report of a possible symptomatic case

“…there was no response to verapamil, sumatriptan tablet, carbamazepine, greater occipital nerve block or lamotrigine. diSCUSSioN several case reports have already been published about symptomatic sUNct: vascular malformations 10,11 , cavernous haemangioma 12 , dorsolateral brainstem infarction 13 , hIv infection 14 , basilar impression secondary to osteogenesis imperfecta 15 , craniosynostosis 16 , leiomyosarcoma of the cavernous sinus 17 , prolactinomas 18,19 , vertebral artery loop 20,21 , pituitary adenomas 22,23 and chronic maxillary sinus disease 24 are the underlying reported etiologies. the present case is striking for the difficulties we face in order to consider it a "symptomatic sUNct syndrome."…”

“…similar situations are seen in the literature. In some reports there was not a close temporal relationship between the onset of the pain and the occurrence of the associated disorder 10,11,15,16,[20][21][22] or remission of the pain after its proper treatment 12,17,20,21 . some authors seemed to be aware of such discrepancies and, accordingly, admitted that in most of such "secondary" cases were coincidental 25 .…”

“…"A close temporal relationship with the underlying cause of the symptoms and resolution with treatment of the associated disorder" is beyond any doubt an important criterion for symptomatic headache disorders; but if one proposes neuroplasticity as the underlying mechanism for such sUNct case, a new formulation for the above mentioned criterion should be considered. this case could technically be classified under an item like "A6.8", but considering the rarity of the condition, and the relative abundance of those cases 10,11,15,16,[20][21][22] , it is possible that they do not occur as a mere coincidence.…”