Migraine Presented as Harlequin Syndrome
Anand Kumar1, Deepika Joshi1, Varun Singh1, Vijay Nath Mishra1, Abhishek Pathak1, Rameshwar Nath Chaurasia1
1Institute of Medical Sciences BHU
Objective:

NA

Background:
Harlequin syndrome is a rare autonomic disorder characterised by unilateral facial flushing and sweating with contralateral anhidrosis induced by exercise, heat, and emotion. It is usually idiopathic but could be the first manifestation of several serious underlying medical conditions. Here, we are presenting a rare case of migraine presented as Harlequin syndrome.
Design/Methods:
NA
Results:

A 17-year-old young female presented with a 4-month history of left hemicranial headache associated with reddish discoloration of left half of the face.  Pain is pulsatile in character associated with photophobia, phonophobia, and occasional nausea.  Duration varied from 4 to 12 hours. Reddish discoloration of the face was proportionate to the pain intensity and disappeared on subsidence of pain. Pain and discoloration were precipitated by any mental stress like solving mathematics, and subsided by sleep or taking analgesics. Due to this unusual reddish discoloration of their face, she avoids going outside and felt embarrassed. No history of associated redness of eyes, nasal congestion, or watering. Biochemical parameters and magnetic resonance imaging with angiography (MRI with MRA) of the brain, were normal.

The patient was diagnosed as a case of migraine with Harlequin syndrome and started on migraine prophylaxis. After one month of follow-up, her headache and redness frequency and intensity were reduced significantly.

Conclusions:

Harlequin syndrome is a rare cranial autonomic dysfunction. Here we report a very rare association with migraine. With proper diagnosis and appropriate management such patients improve clinically as well as psychologically.

10.1212/WNL.0000000000202758