Twiddler (or Not) Syndrome: Questioning etiology for an uncommon form of hardware malfunction in deep brain stimulation

Abstract

Background:Hardware failure or malfunction after deep brain stimulation is an infrequent but costly occurrence with currently available systems.Case Description:The authors present the case of a 65-year-old female patient with predominantly tremoric Parkinson's disease who, 4 months after bilateral subthalamic nucleus stimulation with very good clinical results, began to display signs of recurrent disease and an increasingly smaller response to stimulation. Radiological studies, changes in electrode impedance and surgical findings and results established the diagnosis of Twiddler syndrome. Close patient follow-up, lack of a psychiatric history and physical examination findings were, however, contrary to the previously described causative mechanism.Conclusion:The clinical and radiological setup of Twiddler syndrome must be readily recognized. Its causative mechanism should remain under discussion, and intraoperative technical details may help to explain its occurrence.

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

“…These preliminary observations are hard to be compared with another case reports where only IPGs with two anchoring holes were implanted (2)(3)(4)(5)(6)(7). According to the observations of some authors, TS-although rare-may affect approximately 1% of implanted patients (2)(3)(4)(5)(6)(7)(8)(9). 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 (2-10).…”

“…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.…”

“…We can speculate that TS would be more common when IPGs with a single anchoring hole would be used more commonly. Nevertheless, it is hard to estimate the exact prevalence of TS because most authors report not the exact number of DBS electrodes implantations but mention that this complication was first encountered after roughly over 200 procedures in their series (3,8). The articles presented in chronological order describe small case series or case reports and are summarized in Table 1.…”

“…Although the number of TS cases remains limited, some predisposing factors of the syndrome's development have been recognized. Most authors suggest that these factors include advanced age at the time of surgery with more loose subcutaneous tissue, adipose patients with thick adipose tissue layer precluding proper immobilization of the IPG in the subcutaneous pocket (2)(3)(4)(5)(6)(7)(8)(9). Additional predisposing factors, such as creating an excessively large pocket or a patient with compulsive-obsessive behavior may also play an important role in the development of this hardware-related complication (6)(7)(8).…”

“…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.…”