J Korean Med Sci.  2012 Jan;27(1):104-106. 10.3346/jkms.2012.27.1.104.

Migraine-like Headache in a Patient with Complement 1 Inhibitor Deficient Hereditary Angioedema

• Chung JY
• Kim M

• KMID: 1792989
• DOI: http://doi.org/10.3346/jkms.2012.27.1.104

Abstract

We report on an angioedema patient with a genetic defect in complement 1 inhibitor, manifesting migraine-like episodes of headache, effective prophylaxis with Danazol, and triptan for a treatment of acute clinical episode. The patient was 44-yr-old Korean man with abdominal pain and headache, who was brought into the Emergency Department of Seoul National University Hospital, Seoul. He suffered from frequent attacks of migraine-like headache (3-7 per month), pulsating in nature associated with nausea. Severities were aggravated by activity and his headache had shown recent progression with abdominal pain. No remarkable findings were observed on radiologic examination, brain magnetic resonance images and intracranial and extracranial magnetic resonance angiography. Danazol 200 mg every other day was subsequently used. Following administration of Danazol, symptoms showed improvement and the patient was discharged. While taking Danazol, the migraine-like episodes appeared to be prevented for about 2 yr. At the eighth month, he suffered a moderate degree of migraine-like headache; however, administration of naratriptan 2.5 mg resolved his problem. A case of genetic defect of C1-INH deficiency presented with headache episodes, and was controlled by Danazol and triptan. It suggests that pathogenic mechanism of headache in hereditary angioedema may be mediated by the neurogenic inflammatory-like physiology of migraine.

Keyword

Angioedema; C1-Inhibitor Deficiency; Danazol; Migraine-Like Headache; Triptan

MeSH Terms

Adult
Angioedemas, Hereditary/*complications/diagnosis/*genetics
Brain/radionuclide imaging
Complement C1 Inhibitor Protein/*genetics/metabolism
Danazol/therapeutic use
Estrogen Antagonists/therapeutic use
Humans
Magnetic Resonance Angiography
Male
Migraine Disorders/*diagnosis/drug therapy/*etiology
Piperidines/therapeutic use
Tryptamines/therapeutic use
Vasoconstrictor Agents/therapeutic use

Figure

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  Fig. 1 Brain MRI and MR angiography of this patient. (A) T1-weighted saggital magnetic resonance image shows no significant brain parenchymal abnormality. There is no evidence of subdural fluid collection or other mass lesion. (B) T2-weighted axial scan shows symmetric apprearence of cerebral hemisphere without remarkable findings in suprasellar cistern. (C) Intracranial vessels including, internal carotid arteries, middle cerebral arteries and its bifurcation, anterior cerebral arteries, vertebrobasilar arteries do not show any stenosis or occlusion. (D) Neck vessels, subclavian artery, common carotid arteries with bifurcation and origin of bilateral vertebral arteries are within normal limit.

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