Successful treatment of chronic paroxysmal hemicrania with posterior hypothalamic stimulation: technical case report

Chronic paroxysmal hemicrania (CPH) is a rare, unilateral primary headache syndrome. Recent studies suggest hypothalamic dysfunction as the likely cause of CPH. Therapeutic response to deep brain stimulation of the hypothalamus has been observed in the treatment of related trigeminal autonomic cephalgias. We explored the therapeutic effectiveness of posterior hypothalamic stimulation for the treatment of CPH in a patient intolerant of medical management.

A 43-year-old woman with CPH reported acute onset of lancinating, unilateral headache pain focused about the right orbit. These debilitating headaches were accompanied by ipsilateral nasal congestion, conjunctival injection, tearing, and ptosis lasting minutes before resolving spontaneously. The patient exhausted attempts at medical management.

A deep brain stimulator microelectrode was placed under stereotactic guidance. The posterior hypothalamic target was 3 mm posterior, 5 mm inferior, and 2 mm ipsilateral to the midcommissural point. The electrode was connected to a standard pulse generator and set to final amplitude of 1.5 V, a pulse width of 60 microseconds, and a frequency of 185 Hz.

The patient's headache symptoms were durably alleviated with intraoperative activation. No complications were observed. This preliminary success suggests a role for posterior hypothalamic stimulation as a safe and effective treatment in patients with medically refractory CPH. As a therapeutic incremental innovation, this off-label use of technology for symptomatic therapy contributes to results of studies that support a central pathophysiological role for hypothalamic dysfunction in headaches classified among the trigeminal autonomic cephalgias.