An 85-year-old woman presented with right arm twitching. She had a history of Alzheimer's dementia and is a resident at a skilled nursing facility. That morning at breakfast, the nursing home staff noticed rhythmic twitching of her right upper extremity while she tried to hold her fork. She had a pacemaker implanted for sick sinus syndrome 2 months before this visit (Figure 1). She denied complaints. She specifically denied any problems related to her pacemaker. An ECG showed normal sinus rhythm without any active pacing.
Figure 1. Chest radiograph immediately after pacemaker placement showing normal position of pacemaker leads.
Diagnosis
Twiddler's syndrome. Twiddler's syndrome occurs when the patient deliberately or subconsciously spins the pacemaker generator within its pocket. The pacemaker leads are subsequently dislodged from the myocardium and ventricular pacing ceases. As the leads are continually reeled around the pulse generator, unintended pacing of nerves may occur (Figure 2). The phrenic nerve may be stimulated, resulting in diaphragmatic pacing and the sensation of abdominal pulsations. With further twirling, the brachial plexus may be stimulated, as in this case, causing rhythmic arm twitching.1 Risk factors include obesity, older age, female sex, and especially dementia.2 Treatment involves creating a smaller pocket for the pacemaker, suturing the device to the fascia, and placement of the pacemaker deep in the pectoralis muscle.3
Figure 2. Chest radiograph 2 months after placement showing the pacemaker leads reeled around pulse generator. Used with permission of Benjamin P. Davis, MD, Department of Emergency Medicine, Carle Foundation Hospital, University of Illinois College of Medicine, Urbana, IL.
References
1. 1Nicholson WJ, Tuohy KA, Tilkemeier P. Twiddler's syndrome. N Engl J Med. 2003;34:1726–1727.
2. 2Harel G, Georgeta E, Copperman Y. Twiddler's syndrome: a rare cause of pacemaker failure. Isr Med Assoc J. 2008;10:160–161.
3. 3Riezebos RK, de Ruiter GS. Twiddler's syndrome: an unusual cause of pacemaker dysfunction. Am J Geriatr Cardiol. 2008;17:53–54.
CrossRef
Department of Emergency Medicine, Carle Foundation Hospital, University of Illinois College of Medicine, Urbana, IL
For the diagnosis and teaching points, see page 483.
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