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...