Approach Considerations
The diagnosis of chronic paroxysmal hemicrania (CPH) is extremely important because it may lead to lifelong treatment with a potentially noxious drug.
Perform lab studies to evaluate structural, metabolic, and other secondary causes of headache and facial pain. Baseline routine blood tests may be needed to exclude contraindications to certain drugs and to avoid complications from long-term use of various medications.
The INDOTEST (indomethacin 50mg intramuscular [IM] test dose) may be a useful tool in assessment of unilateral headache. Perform this test in a standardized manner.
No characteristic electrocardiographic patterns have been found during attacks of CPH, but marked variations in heart rate and rhythm abnormalities, including bradycardia, sinoatrial block, bundle branch block with episodes of atrial fibrillation, and multiple extrasystoles, have been observed.
Orbital phlebography may be abnormal in some patients, but the significance of this finding has not been established.
In a study of 3 patients with CPH, a slightly lower cerebral vasomotor reactivity was observed in the medial and posterior cerebral arteries on both sides and in the anterior cerebral artery on the symptomatic side than has been found in healthy subjects. These observations may imply an abnormal vascular reactivity in CPH.
In another study, as compared with cluster headache (CH), CPH attacks did not demonstrate any changes in visually evoked event-related potentials (ERPs), latencies, and amplitudes.[13]
Perform ophthalmic evaluation, if needed, to assess ocular pathology such as glaucoma or orbital pseudotumor.
Imaging studies
Computed tomography (CT) scanning or, preferably, magnetic resonance imaging (MRI) of the brain may be needed to rule out structural pathology. Neuroimaging study findings, including those from MRI, are usually normal in patients with CPH.
Consider obtaining an MR angiogram or arteriogram, if necessary, for atypical presentations. Electroencephalography, brain mapping, and other radiologic studies are not required for patients with typical presentations.
Procedures
Consider lumbar puncture, if necessary, for atypical presentations.
Sjaastad O, Dale I. Evidence for a new (?), treatable headache entity. Headache. Jul 1974;14(2):105-8. [Medline].
Sjaastad O, Dale I. A new (?) Clinical headache entity "chronic paroxysmal hemicrania" 2. Acta Neurol Scand. Aug 1976;54(2):140-59. [Medline].
Headache Classification Committee of the International Headache Society. Classification and diagnostic criteria for headache disorders, cranial neuralgias and facial pain. Headache Classification Committee of the International Headache Society. Cephalalgia. 1988;8 Suppl 7:1-96. [Medline].
Russell D, Vincent M. Chronic paroxysmal hemicrania. In: The Headaches. 2nd ed. Philadelphia: Lippincott Williams & Wilkins; 2000:741-9.
Goadsby PJ, Lipton RB. A review of paroxysmal hemicranias, SUNCT syndrome and other short-lasting headaches with autonomic feature, including new cases. Brain. Jan 1997;120 ( Pt 1):193-209. [Medline].
Matharu M, May A. Functional and structural neuroimaging in trigeminal autonomic cephalalgias. Curr Pain Headache Rep. Apr 2008;12(2):132-7. [Medline].
Leone M, Bussone G. Pathophysiology of trigeminal autonomic cephalalgias. Lancet Neurol. Aug 2009;8(8):755-64. [Medline].
Sjaastad O, Apfelbaum R, Caskey W, Christoffersen B, Diamond S, Graham J, et al. Chronic paroxysmal hemicrania (CPH). The clinical manifestations. A review. Ups J Med Sci Suppl. 1980;31:27-33. [Medline].
Kudrow DB, Kudrow L. Successful aspirin prophylaxis in a child with chronic paroxysmal hemicrania. Headache. May 1989;29(5):280-1. [Medline].
Gladstein J, Holden EW, Peralta L. Chronic paroxysmal hemicrania in a child. Headache. Oct 1994;34(9):519-20. [Medline].
Broeske D, Lenn NJ, Cantos E. Chronic paroxysmal hemicrania in a young child: possible relation to ipsilateral occipital infarction. J Child Neurol. Jul 1993;8(3):235-6. [Medline].
Seidel S, Lieba-Samal D, Vigl M, Wöber C. Clinical features of unilateral headaches beyond migraine and cluster headache and their response to indomethacin. Wien Klin Wochenschr. Sep 2011;123(17-18):536-41. [Medline].
Evers S, Bauer B, Suhr B, Voss H, Frese A, Husstedt IW. Cognitive processing is involved in cluster headache but not in chronic paroxysmal hemicrania. Neurology. Jul 22 1999;53(2):357-63. [Medline].
Shabbir N, McAbee G. Adolescent chronic paroxysmal hemicrania responsive to verapamil monotherapy. Headache. Apr 1994;34(4):209-10. [Medline].
Dahlöf C. Subcutaneous sumatriptan does not abort attacks of chronic paroxysmal hemicrania (CPH). Headache. Apr 1993;33(4):201-2. [Medline].
Hannerz J, Jogestrand T. Intracranial hypertension and sumatriptan efficacy in a case of chronic paroxysmal hemicrania which became bilateral. (The mechanism of indomethacin in CPH). Headache. Jun 1993;33(6):320-3. [Medline].
Pascual J, Quijano J. A case of chronic paroxysmal hemicrania responding to subcutaneous sumatriptan. J Neurol Neurosurg Psychiatry. Sep 1998;65(3):407. [Medline]. [Full Text].

