Twiddler’s syndrome in a patient with epilepsy treated with deep brain stimulation

Abstract

Twiddler's syndrome is the conscious or unconscious manipulation of implantable pulse generators (IPGs) or associated wire systems by the patient. Most commonly, this complication has been documented in patients with cardiac pacemakers, but there are reported cases in patients with deep brain stimulators. Twisting of stimulator systems results in dislodgement or damage to leads and loss of stimulation to the desired target. The present case illustrates a rare complication that may have serious consequences in patients with deep brain stimulators. A 21 year-old woman presented with recurrence of seizures from failure of her deep brain stimulator targeting the anterior nuclei of the thalamus to control refractory epilepsy, 6 months after it was implanted. Radiographic imaging revealed that the IPG had been twisted upon itself causing coiling and looping of extension wires. The patient denied any conscious manipulation of the system, which was subsequently surgically revised. Surgical revision achieved the desired stimulation and effects. On 4-month follow-up the deep brain stimulator remained stable and untwisted; however, it was subsequently removed in the fourth month because of infection at the extension site. In conclusion, twiddler's syndrome is a rare complication occurring in patients with deep brain stimulator implants and warrants awareness among neurologic and neurosurgical epilepsy specialists.