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

Abstract

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.

Dual Anchor Internal Pulse Generator Technique May Lower Risk of Twiddler’s Syndrome: A Case Series and Literature Review

“…Also the shape of bulky IPG with a single anchoring is theoretically more susceptible to twiddling than a dual anchor flat IPG. Further case reports of TS may support this preliminary observation .…”

“…According to the observations of some authors, TS—although rare—may affect approximately 1% of implanted patients . This can mean that this complication has been an unrecognized and underreported phenomenon because in the literature we have found only 8 articles reporting 11 patients with the diagnosis of TS requiring surgical revisions in all cases . As it was mentioned above in all of these reported cases the IPGs with two anchoring holes were used.…”

“…There are only a few case reports and one case series including three patients presenting the recognition and management of this hardware-related complication (6). In all of these reports the predisposing factors of the development of TS have been identified and include loose subcutaneous tissue, obesity, older individuals undergoing DBS procedures, obsessive-compulsive behavior of patients implanted with DBS hardware, creation of large pockets for the IPG or inappropriate IPG fixation to the fascia or subcutaneous tissue (2)(3)(4)(5)(6)(7)(8)(9)(10). The location of IPG implantation may also play a role in the development of TS.…”

Challenges in PD Patient Management After DBS: A Pragmatic Review

“…Exceptional complications have been reported, like twiddler syndrome, in which spontaneous or intentional rotation of implantable cables or IPG resulting from external manipulation, dislodges leads, ultimately requiring surgical revision . Twiddler syndrome risk might be reduced with non‐absorbable sutures and dual anchor IPG caps .…”

“…Exceptional complications have been reported, like twiddler syndrome, in which spontaneous or intentional rotation of implantable cables or IPG resulting from external manipulation, dislodges leads, ultimately requiring surgical revision . Twiddler syndrome risk might be reduced with non‐absorbable sutures and dual anchor IPG caps . Other rare complications include development of structural lesions around implanted DBS leads such as large aseptic cysts, which usually resolve after lead removal, and non‐infectious peri electrode edema that may produce disorientation, gait instability, headache, seizure, or acute confusion …”