PMC4414864_01
Familial pedigree. Autosomal-dominant inheritance is suspected. y, years. For each family member, age at the time Patient 1 first presented for medical attention is shown.
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PMC4414864_01_10194_2015_519_Fig1_HTML.jpg
Fig1
The index patient (Patient 1: IV-3 in Figure 1) was a 5-year-old girl with a history of recurrent, unilateral temporal headaches lasting 20-90 s associated with ipsilateral conjunctival injection, lacrimation, nasal congestion, rhinorrhea, and facial flushing. Autosomal-dominant inheritance of this disease was inferred from the pedigree (Figure 1).
Clinical findings. Flushing of (A) the face and (B) arm after a right-sided severe headache attack in Patient 1.
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PMC4414864_01_10194_2015_519_Fig2_HTML.jpg
Fig2
Unilateral, mainly right-sided, facial and upper-extremity flushing (Figure 2) appeared 5-10 min after severe attacks and lasted from half an hour to a few hours.
PMC4414864_02
PMC4414864_03
PMC4414864_04