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Published on: June 2, 2014
Chronic paroxysmal hemicrania: from the index patient to the disease
1Department of Neurology, Trondheim, Norway. tora.rui@ntnu.no
Insights
Chronic paroxysmal hemicrania (CPH) is distinct from cluster headache, showing different clinical features and responses to treatment. Indomethacin proved effective for CPH, unlike typical cluster headache medications.
Area of Science:
- Neurology
- Headache Medicine
Background:
- Chronic paroxysmal hemicrania (CPH) is a rare headache disorder.
- Distinguishing CPH from cluster headache is crucial for effective treatment.
Purpose of the Study:
- To detail the clinical presentation and diagnostic journey of the first documented patient with CPH.
- To differentiate CPH from cluster headache based on clinical, autonomic, and therapeutic variables.
Main Methods:
- Longitudinal case study of a patient diagnosed with CPH.
- Comparison of clinical attack characteristics, ocular variables, and autonomic features with known cluster headache patterns.
- Evaluation of treatment response to indomethacin versus cluster headache medications (ergotamine/sumatriptan).
Main Results:
- The patient experienced CPH with unique attack frequency and duration.
- Ocular variables and pupillary reactions differed significantly from cluster headache.
- Indomethacin was highly effective for CPH, while cluster headache drugs were ineffective.
- Autonomic features like sweating patterns were distinct from cluster headache.
Conclusions:
- The presented case definitively established CPH as a distinct headache entity.
- CPH exhibits unique clinical and therapeutic profiles differentiating it from cluster headache.
- Indomethacin is a key therapeutic agent for CPH.
Abstract:
The first patient with chronic paroxysmal hemicrania (CPH), a 41-year-old woman, first seen in 1961, was followed until an adequate treatment was found, 12 years later. Clinically, attack frequency and duration differed widely from the general pattern of cluster headache. Ocular variables, such as intraocular pressure and corneal indentation pulse amplitudes, also differed in our case (clear symptomatic side increment during attacks) and cluster headache. Pupil reactions to directly and indirectly acting sympathicomimetic drugs were also vastly different in our case and cluster headache: no signs of Horner s syndrome in our patient, while cluster headache exhibits a "Horner-like pattern." In cluster headache, there is a relative hypohidrosis in the forehead on the symptomatic side if body temperature is increased, and a clear hyperhidrosis on direct parasympathomimetic stimulation. This was not so in our case. Indomethacin was highly effective in our case, while "cluster headache drugs," such as ergotamine/sumatriptan, were ineffective. Indomethacin was inactive in cluster headache. Accordingly, our case seemed to differ decisively from cluster headache: CPH had been discovered.
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