HEADACHE ASSOCIATED WITH THE CHIARI TYPE-I MALFORMATION

The main purpose of the present study was to define and understand mor e clearly the headache, and in particular the long-lasting attacks or continuous pain associated with the Chiari type I malformation. Of 34 patients with the malformation, the 20 patients who had or had had hea dache problems were interviewed according to a standardized questionna ire. Many patients had several headache types. Ten patients had shortl asting 'cough headache' attacks lasting less than 5 minutes, 14 patien ts had relatively long-lasting attacks lasting from 3 hours to several days, and 8 patients had continuous headache. Unlike the short-lastin g cough headache attacks, long-lasting attacks were usually not precip itated by Valsalva-like maneuvers. With discriminant analysis, this he adache could rather well be differentiated from that of migraine and c ervicogenic headache patients. In many respects, however, this headach e resembled cervicogenic headache with occipital and neck pain, pain i n the arm, restriction of neck movement, and dizziness often accompany ing the headache. Dizziness was the most distinguishing feature in the Chiari patients. These features, together with a beneficial effect of surgical treatment in some patients, suggest a causal relationship be tween the malformation and headache. From the histories of a few illus trative cases, it is suggested that the malformation may cause long-la sting headache attacks or continuous head pain by compression of the b rainstem, central cord degeneration or intracranial hypertension.