Twiddler syndrome in a patient with tremor dominant Parkinson's disease. A case report and literature review

Twiddler syndrome is described as a spontaneous rotation or intentional external manipulation of implanted cardiac or occasionally deep brain stimulation (DBS) devices. We report this hardware related complication in a patient with tremor dominant Parkinson's disease (PD), who underwent unilateral subthalamic nucleus (STN) DBS and subsequently developed twiddler syndrome. The clinical course of twiddler syndrome in this patient is described. Some surgical nuances which may prevent its occurrence are suggested. Our case report indicates that twiddler syndrome occurs in DBS patients. Impedance check of DBS hardware, plain chest X-ray, or palpation for a knobbly extension lead through the skin above the IPG allows the correct diagnosis and subsequently a prompt surgical revision. Our subsequent literature review revealed only 10 patients with twiddler syndrome in DBS patient population worldwide. This number may suggest that this syndrome may be unrecognized or underreported, given the number of patients with movement disorders implanted with DBS hardware worldwide.