Two More Secondary Headaches Mimicking Chronic Paroxysmal Hemicrania. Is This the Exception or the Rule?

Abstract

Two patients with headaches meeting the criteria of chronic paroxysmal hemicrania, as defined by the International Headache Society classification, are presented. Further investigations revealed parasellar pituitary microadenoma in the first patient and a maxillary cyst in the second. Surgical removal of the lesions resulted in complete relief from headaches. The clinical features of this rare syndrome are discussed and suggest that a more detailed laboratory study and clinical follow-up is necessary in patients with chronic paroxysmal hemicrania.Key words: chronic paroxysmal hemicrania, secondary headache, headache diagnosis Abbreviations: CPH chronic paroxysmal hemicrania (Headache 1996;36:511-513) Chronic paroxysmal hemicrania (CPH) is a rare disorder which resembles cluster headache and is characterized by short attacks, lasting from 2 to 45 minutes. It is associated with autonomic nervous system changes and responds to treatment with indomethacin (150 mg/day or less). 1 It is considered a variant form of cluster headache but with differences in duration and frequency of attacks, sex distribution, and therapeutic response to indomethacin. 2,3 Recently, a number of reports have described the coexistence of CPH and intracranial lesions or vascular disorders. [3][4][5] Two patients with CPH are presented. Laboratory examination revealed a tumor in the right parasellar area of the first patient, and physical examination of the second patient revealed a maxillary cyst. CASE HISTORIESPatient 1.-A 20-year-old man, with no family history of headache, was seen for the first time in our department in March 1994. His symptoms had started 1 year previously and consisted of attacks of severe throbbing and pulsating pain located in the right supraorbital area. The headache, lasting 10 to 20 minutes occurred 5 to 10 times a day; pain was associated with lacrimation, nasal congestion, rhinorrhea, ptosis, and severe unilateral eyelid edema. Many of the attacks occurred during sleep. At times, ptosis and eyelid edema persisted between attacks. Physical and neurological examinations did not reveal any abnormalities. A brain CT scan taken at this time was normal. Cluster headache was diagnosed and treatment with carbamazepine (400 mg daily) and amitriptyline (25 mg daily) produced a significant decrease both in frequency and intensity of the attacks. Six months later, however, the attacks occurred with greater intensity and higher frequency, and the patient was totally incapacitated. At this time, he was admitted to our department. Physical and neurological examinations yielded normal results, except for right eyelid edema. Routine hematology and biochemical screening were normal. Indomethacin (75 mg per day) was prescribed, and the intensity and frequency of attacks markedly decreased. Nevertheless, a repeat brain CT scan demonstrated a right parasellar hypodense area; coronal MRI with intravenous Gd-DPTA showed a signal intensity slightly less than that of the normal pituitary gland. On the basis of these findings, the dia...

Chronic Paroxysmal Hemicrania, Hemicrania Continua and Sunct Syndrome in Association with Other Pathologies: A Review

“…However, as the effect of indomethacin in CPH can be exerted at different doses, IHS criteria require an absolute effect at a dose of 150 mg daily or below. This is not the case in all reports presented in this review: in two cases (23, 32), indomethacin was ineffective; in five cases [31, 35 (case B), 37 (cases A and B), 40] the dose of indomethacin needed to obtain therapeutic effect was higher than 150 mg, ranging from 200 to 350 mg; in one case [36 (case A)] the dose of 75 mg was partly effective (but the higher dose was not tested), in two cases (25, 33), the dose of indomethacin was unspecified, and in four cases [24, 27 (case B), 28 (case A), 30] indomethacin was not administered. It is of interest that in two cases sumatriptan was effective (30, 31), unlike in genuine cases of CPH (41).…”

Chronic Paroxysmal Hemicrania as a Manifestation of Intracranial Parotid Gland Carcinoma Metastasis — A Case Report

“…The case described by Sjaastad et al (6), according to comments from the authors, was a symptomatic CPH, which means that the algic symptoms caused by the tumour are identical to those seen in primary CPH patients. The same may be said about the cases described by Gatzonis et al (7). In the second case described by these authors, the presence of a maxillary cyst could hardly explain the CPH‐like symptoms and a complete pain relief was obtained after the surgical removal.…”

“…Some authors (8) claimed the involvement of structures in the cavernous sinus and the hypothalamic nuclei in the floor of the third ventricle were in the core of the pathophysiology of CPH. Cases with involvement of cavernous venous sinus or its vicinities (maxillary sinus, sella turcica) could lend support to this view (6–9).…”

“…Cerebral angiography was undertaken in 16 patients and in two cases, little aneurysms were disclosed, both of them, in the posterior communicating artery. However, secondary causes of headaches that mimic CPH have been reported in recent years (4–8).…”

Chronic Paroxysmal Hemicrania in a Patient with a Macroprolactinoma

“…A 20‐year‐old man with PH for at least 1 year was found to have a microadenoma of cells producing TSH, ACTH and prolactin (6). His headache attacks fulfilled all but one of the ICHD‐II criteria for chronic PH, i.e.…”