Deep Brain Stimulation for Intractable Hiccups due to Diaphragmatic Dystonia: A First Reported Case

Chronic hiccups (singultus) are involuntary diaphragmatic contractions that, when persistent, can cause severe physical and psychological distress. While most cases are benign and self-limited, intractable hiccups may rarely indicate underlying neurological dysfunction. One exceptionally rare cause is diaphragmatic dystonia - a condition for which no established diagnostic criteria or treatment guidelines exist, and which has been described only in isolated case reports.

We present the first known case of deep brain stimulation (DBS) of the internal and external globus pallidus (GPi and GPe) used to treat chronic, treatment-resistant hiccups. A 70-year-old woman experienced 2 years of continuous hiccups unresponsive to multiple pharmacologic and surgical interventions. Extensive diagnostics excluded metabolic, neurological, gastrointestinal, and psychiatric causes. Diaphragmatic dystonia was suspected. Bilateral octa-contact DBS electrodes were implanted targeting the GPi and GPe. Intraoperative monopolar stimulation (1.5 mA, 90 µs, 130 Hz) was initiated on day of surgery. Electrodes were accurately placed without complications.

Near-complete resolution of hiccups occurred immediately postoperatively. Quality of life significantly improved: Numerical Rating Scale (NRS) from 8 to 0, EQ-5D-3L from 0.530 to 1.0, Fahn-Marsden Dystonia Scale from 13 to 0, and PHQ-9 from 16 to 4. At the 14-month follow-up, the improvement was fully maintained.

DBS targeting the globus pallidus may represent a novel and effective intervention for refractory chronic hiccups, particularly in cases suspected to involve diaphragmatic dystonia.