By the time you’ve worked in the Emergency Department for more than a week it’s highly likely you’ll have seen a patient who has a bit of a sore neck after a relatively minor bump in their car. A little less often we see patients presenting with a fixed torticollis, their head held over to one side with pain associated with attempts to return it to a normal position. Torticollis as a presenting symptom can represent a number of pathologies, some of which are related to dystonia. The word torticollis comes from the Latin tortus (twisted) and collum (neck).
The torticollis can occur in a variety of directions:
Simple torticollis where there is no rotation of the head but uneven muscle tone present
Rotational torticollis where the face is turned towards the shoulder
Lateral torticollis where the head is tilted, ear to shoulder
While this is often benign, you’ll be pleased to know that it’s yet another seemingly innocuous condition which can sometimes represent some important and sinister pathology and as such it’s worth thinking about carefully, both in the context of immediate management and appropriate safety-netting, review and follow-up.
“A Car Drove Into The Back Of Mine!”
In those with a history of trauma, cervical dystonia can occur relatively quickly after trauma (immediately to a few days afterwards) or quite some time afterwards (delays of a few months have been reported although I wonder how reliably these can be attributed back to initiating trauma, particularly for minor injuries).
The trauma is usually associated with immediate pain and followed by a reduced range of movement and abnormal head posturing. If the patient has a history of trauma and meets criteria for imaging then radiographs may be helpful as dystonia can occur in the presence of bony injury. While movements should be minimised, please don’t try to force these patients into a rigid collar!
Just be careful and thorough with these patients. Perform a neurological examination and check the history carefully to make sure there are no features which might point you to possible sinister causes – think fever, weight loss, night sweats, reduced appetite…
In patients with a significant mechanism of injury, torticollis is an ominous sign. It is often associated with unifacet dislocation or fracture of the occipital condyle (which follows a high-energy injury). These patients frequently have associated neurological findings and often need cervical spine CT and subsequent MR scan – a chat with your friendly radiology colleague is required!
“Doctor, That Vomiting Girl in Bed 6 Looks a Bit Funny…”
Certain medications can precipitate acute dystonic reactions. For dystonic reactions developing acutely in the ED the most likely culprit is metoclopramide and acute oculogyric crises are most commonly seen with metoclopramide in young women, although patients may present in a similar way after taking their own phenytoin, carbamazepine or antipsychotics. Patients often have speech disturbance or staring. The recommended treatment in the UK is intravenous procyclidine 5-10mg for acute dystonia and it is usually effective in 5-10mins.
The video below shows a frightening reaction to metoclopramide; it’s easy to see why patients will need a good deal of explanation afterwards!
“I Just Woke Up Like This, Doc.”
Most often (around 85% of cases) there is no clear history of trauma; the patient has simply woken up like this in the morning. Acute idiopathic torticollis is the most common presentation – there is no history of trauma, the adult wakes with the neck stuck in a particular position and the vast majority of cases will resolve spontaneously within 1-2 weeks. There was an RCT of benztropine for the relief of acute non-traumatic neck pain (not FOAM) in the EMJ in 2014 which failed to demonstrate superiority.
“My Neck Hurts and I Don’t Feel Well!”
While acute idiopathic torticollis can present in children as well as in adults, it can also be a sign of some underlying nastiness and this is more true of the paediatric population (though it can occur in adults as well).
Torticollis can be associated with infection of the structures of the neck: think pharyngitis, tonsillitis, retropharyngeal abscess, otitis media, osteomyelitis, sinusitis, adenitis and even upper lobe pneumonia. Look for sickness in these patients who may not automatically have their vital signs recorded at triage. In the presence of fever or tachycardia, or in a patient who also reports constitutional symptoms, examine fully and investigate for possible infections.
We do occasionally see torticollis as a first presentation of a neoplastic cause; tumours in the cerebellopontine angle/posterior fossa cause a compensatory torticollis, so neurological examination of upper limbs, lower limbs, gait, co-ordination and cranial nerves is also important (you might alternatively identify the congenital strabismus or nystagmus which has led to the compensatory torticollis).
Finally, children can have atlantoaxial subluxation (C1 on C2) which may be related to juvenile arthritis or result from ligamentous laxity following infection in the neck (Grisel’s syndrome). This free electronic poster below outlines three typical paediatric clinical cases of non-resolving torticollis and shows the importance of follow-up – click the image to see a bigger version in PDF format.
These conditions may not be apparent at first presentation and since most are idiopathic and self resolving, follow-up is key; particularly in paediatric patients. If the symptoms are persisting, imaging of the brain and cervical spine is appropriate (MR scan is probably better than CT).
“My Baby is Making Weird Movements!”
Congenital torticollis is rare and usually related to in-utero positioning or birth trauma causing abnormal neck positioning, presenting in the first few weeks of life. Ultrasound can confirm the diagnosis and the muscles involved (most commonly ipsilateral sternocleidomastoid).
Sandifer syndrome may also cause paroxysmal dystonia with opisthotonic posturing: this is associated with reflux in babies (the diagnosis is more likely if episodes are associated with feeding) and can be very frightening for parents who think their baby is having a seizure.
So in summary:
- Most are atraumatic and spontaneous and will resolve spontaneously: analgesia (and possibly benzodiazepines) constitute first-line treatment
- If there is a history of a traumatic mechanism of injury, imaging can be helpful
- Thorough assessment for potential infection and neurological signs is very important (the key here is thorough history-taking and examination)
- Those which are not resolving should be reassessed; consider planned follow-up for kids and clear safety netting for all patients
Natalie
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Congenital torticollis in my first born, important to note its improvement and resolution both of its own acord associated gentle physiotherapy.
Still may be a reason for presentation..
Prochlorperazine and dystonia, still rares its head- a large muscle I said, a large muscle!