Clinical Image Case: Triple “E” Syndrome
Triple “E” Syndrome: An Unusual Presentation of Severe Bilateral Arm Pain
A 68-year-old male with a past medical history of HTN, HLD presents reporting severe bilateral upper extremity pain. Patient has trouble providing more history due to pain, but states he woke up in bed with the pain and denies any trauma.
BP 129/68 | Pulse 64 | Temp 97.3 °F (36.3 °C) | Resp 18 | SpO2 93%
You note uncomfortable appearing man, mild confusion, minimal bilateral shoulder deformity, proximal bilateral arm tenderness, decreased range of motion of bilateral shoulders, soft compartments, distal upper extremities neurovascularly intact.
You also note two other notable exam findings that aid in your diagnosis: dried urine on the patient’s pajama pants and a small amount of dried blood at the corner of his mouth.
You obtain x-rays including the following:
Bilateral posterior shoulder dislocation with proximal humerus fractures, likely secondary to seizure.
Patient’s wife subsequently provided corroborating history reporting that she woke up early this morning to find her husband “flailing around” in the bed shaking uncontrollably. When he stopped, he was confused at first and then reported severe pain. She confirms he has no previous history of seizure and that he had no recent trauma and had no arm pain or injuries when he went to sleep the night before.
Shoulder dislocations account for approximately 50% of joint dislocations, with anterior dislocation making up 95% of shoulder dislocations, posterior shoulder dislocations accounting for ~4%, and the remaining ~0.5% of shoulder dislocations due to inferior dislocations (luxatio erecta).  When posterior shoulder dislocation does occur, they are usually secondary to seizure, electric shock, or severe trauma. Brackstone et al (2001) proposed the “Triple E Syndrome” referring to the three causes of bilateral posterior shoulder dislocation: epilepsy, electrocution, extreme trauma.  A 1989 study found that of 2800 patients admitted to the hospital with a diagnosis of seizure, 1.1 % (30/2800) of these patients sustained fractures, with 0.3% (7/2800) of patients sustaining fractures due to seizure alone (no associated trauma). 
Bilateral posterior fracture-dislocation of the shoulder, first described in 1902 by Herman Mynter is even more rare and has been suggested to be pathognomonic of seizure in absence of trauma.  Furthermore, given the rarity of this presentation and the associated x-ray findings, diagnosis of posterior shoulder dislocation is often missed or there is a delay in diagnosis on initial examination.  As an emergency medicine physician, it is important to have a high clinical suspicion for posterior shoulder dislocation when patients report shoulder pain and have decreased range of motion after seizure.
Mechanistically, posterior dislocations are the result of severe internal rotation and adduction. During a seizure this may occur due to internal rotator muscles of the shoulder contracting with greater force than the external rotators, resulting in the humeral head moving superiorly and posteriorly.  If the seizure endures after dislocation the refractory muscle contraction can lead to humerus fracture, as in the patient in this case.
Shoulder dislocations without fracture may be reduced by the ED physician, however, given the complexity of proximal humeral head fractures that occur in setting of dislocation, operative repair is often required.
Possible first x-ray taken of posterior dislocation:
Neurology Case Resolution:
Patient had an unrevealing first-time seizure work up including a CT brain, electrolytes, finger stick glucose, infectious work up, urinalysis, and toxicology screen. During his inpatient stay, neurology was consulted and an inpatient EEG and MRI were obtained. The plan upon discharge was for neurology follow-up and to avoid driving for 6 months.
Orthopedic case resolution:
Orthopedic surgery was consulted given the fracture dislocation. Closed reduction was attempted in the ER but was unsuccessful. The patient was therefore taken to the operating room and ultimately required bilateral total shoulder arthroplasty. Patient was discharged to rehab facility with plan for physical therapy.
Author: Derek Lubetkin, MD is a fourth year emergency medicine resident at Brown Emergency Medicine Residency.
Faculty reviewer: Jared Anderson, MD is an attending physician at Brown Emergency Medicine
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