Also known as cervical dystonia or a “Wry neck” can now respond to various forms of advanced surgical treatment, as seen here…
The word torticollis is derived from the Latin words ‘tortus’ and ‘collum’, meaning twisted and neck, respectively.
It is a type of dystonic movement disorder most commonly seen in adults age 30 to 40 years of age. Spasmodic torticollis is adult-onset focal or segmental dystonia with a variable combination of neck flexion, extension, rotation, and tilting.
Each individual has a characteristic dystonic posture, which is present at rest, worsens with movement or stress, and improves during sleep.
Torticollis develops in stages, starting with a feeling of tension in the neck muscles, followed by intermittent posturing of the neck with head-turning.
Patient with Twisted Neck
Over a period of time, this becomes constant and fixed, with a little relief during sleep. Symptoms may progress rapidly over several weeks or gradually over several years until it reaches a stage where no more change occurs, which happens 3 to 5 years after the initial manifestation.
Temporary spontaneous remission is almost unusual. But if surgery is done at an early stage, dystonia can be controlled.
Rotation of chin towards the shoulder.
The lateral tilt of the head in the coronal plane with the ear moving towards the shoulder
Forward deviation of the head in the sagittal plane with the chin moving towards the chest
Backward deviation of the head in the sagittal plane thereby elevating the chin and moving the occiput towards the upper back
Torticollis may be either:
Dystonic Torticollis
Non-dystonic or Pseudodystonia
Primary focal dystonia presents as sustained involuntary contractions of the neck muscles leading to abnormal movement and posture of the head.
Further, Dystonic Torticollis is of the following types –
A chronic, progressive form of torticollis. It is thought to be caused due to abnormalities of basal ganglia.
It usually starts with a mild dystonic posture which gradually becomes severe. It manifests as either fixed or intermittent jerky type or a combination of both.
It usually becomes worse during the first 5 years of onset and then eventually stops progressing.
It severely affects activities of daily living and a person’s self-confidence. Spontaneous remission is seen in 20% of patients, but that is usually not complete or prolonged. Also, nearly all patients relapse within 5 years.
This is not so common. It could be due to focal brain lesions of various origins, neurodegenerative disorders, metabolic disorders, drugs, and chemicals that affect the basal ganglia, thalamus, and brainstem and following peripheral injury.
Associated with sustained muscle contractions, due to reflex mechanisms, or example, tilting of the head to improve vision in 4th (trochlear nerve) palsy or in hemianopia.
Non-dystonic torticollis can be divided into:
presents at birth but comes to notice late because head control develops only at 3 months. It is caused due to in utero malposition (breech presentation), birth trauma, malformations of the cervical spine, hypertrophy of cervical muscles, Arnold Chiari malformations.
presents during infancy or early childhood. It is caused due to CNS tumors, syringomyelia, musculoskeletal abnormalities of the cervical spine like atlantoaxial dislocation, cervical spine osteomyelitis, benign paroxysmal torticollis. Psychogenic causes may occur during adolescence.
Clinical features
Symptoms are typically absent early in the morning (honeymoon period), but increase with fatigue and get worsened as the day passes. It is further aggravated by fatigue, stress and anxiety. Watching television, reading or writing are some specific tasks which are reported to worsen torticollis.
Clinical features of Primary or Idiopathic Torticollis
In this, there is abnormal head posture associated with involuntary twisting or jerking of the head and it is not associated with any other neurological sign apart from head/ hand tremor and marked cervical spinal abnormalities. Deviation may occur in any single plane or combinations of directions in which the head moves voluntarily.
The common findings in Idiopathic Torticollis are:
Other Clinical Features in Idiopathic Cervical Dystonia
Clinical features of Secondary Torticollis
Secondary CD should be suspected when patients have additional neurologic, orthopedic, or medical disorders or have a history of drug exposure or trauma prior to onset of CD.
Factors Differentiating Primary from Secondary Dystonia
Following factors are seen in secondary dystonia:
Additional focal neurological signs.
Orthopedic and neurologic complications due to Torticollis
Passive stretching and proper head positioning : It is very helpful in infants and small children. In passive stretching, a strap is used to hold the head in a certain position to release the tension.
Tummy time: Laying your baby on the stomach for a short period while awake. It helps strengthen neck and shoulder muscles and prepares the baby for crawling.
These modalities often help and bring improvement, but should be started at 3 months of age.
Hot fermentation, traction to the neck and massage may help to relieve pain in the head and neck region.
Stretching exercises and neck braces help with relieving muscle spasms.
Anticholinergic drugs are very effective for the treatment of torticollis. Dose is increased gradually to avoid side effects. Trihexyphenidyl is a central muscarinic antagonist and helps in symptomatic treatment of segmental and generalized dystonia. Benztropine, an anticholinergic, blocks presynaptic dopamine uptake. Side effects such as dry mouth and eyes, constipation, dizziness, confusion, urinary retention, hallucinations, increased heart rate.
Benzodiazepines, such as clonazepam are effective in decreasing the pain as well as anxiety associated dystonia.
Baclofen helps to reduce neck muscle contractions. This is of limited value and can produce drowsiness.
Dopaminergic drugs, for example, Levodopa, and dopamine reducing medications such as tetrabenazine, clozapine, quetiapine. These medications have to be supervised by an expert in the field as sometimes they can also worsen the symptoms.
Botulinum toxin injection, is injected in the abnormally contracting muscles every 3 months, for symptomatic temporary relief. Injection, has to be done in the affected neck muscles. If blindly injected, it would not provide any relief. The neck muscles can be identified either by EMG or Ultrasound. This is supplemented with the clinical knowledge of the dystonic muscles. Unfortunately, the treatment requires huge dose of botulinum toxin and it is quite expensive. After a few sessions, patients develop resistance (develop antibodies) making it difficult to get the same response.
Anti-depressants, role is limited, but helps with psychogenic causes.
There are two forms of surgical treatment. One is cervical rhizotomy and the second is deep brain stimulation.
Indicated in patients with cervical dystonia / torticollis who do not achieve adequate response with medical treatment or repeated botulinum toxin injection. Rhizotomy could be the best option in such patients. About 2/3rd patients achieve long-term improvement.
Selective peripheral denervation is the best surgical option for these patients. It is not indicated in patients who have symptoms like blepharospasm and laryngeal spasm in addition to cervical dystonia.
he procedure is done under general anaesthesia, to avoid any discomfort to the patient.
First nerves supplying the affected muscles of the neck are selectively resected.
A small incision is given at the back of the neck. Nerves outside the spinal canal, which are supplying the affected muscles, are first identified and stimulated under Neurophysiologic guidance and then only, each nerve is carefully identified and resected under the microscope, this way, affected muscles are denervated without damaging any important structures.
The adequacy of denervation is determined with the help of signals recorded from each muscle before and after the resection of the nerves. Once denervation is over, the patient is turned on his back and the front of the neck is prepared.
One of the muscles which is responsible for torticollis is sternocleidomastoid in the neck. Another small incision is made on the neck and the nerve supplying this muscle is resected. Similarly, the nerve supplying the muscle which is responsible for elevation of shoulder in torticollis is carefully resected.
The complete procedure is done under Neurophysiological monitoring, so results are extremely good. The total duration of stay in the hospital is around 7 days. Procedure is performed only at various Select Centres around the word.
It has been pioneered by Professor Taira from Japan, and we are the only centre in India that performs this surgery. One of the biggest advantage of this surgery is that, it is a one time procedure and does not involve any implants.
Thalamotony, ventralis oralis internus (VOI) nucleus is the target for lesioning in Thalamotomy.
Unilateral thalamotomy results in minor improvement in axial and cervical dystonia, whereas bilateral thalamotomy is more effective.
There is a 10%-40% risk of serious complications such as bulbar weakness leading to hypophonia, dysphagia, dysarthria, cognitive impairment and ataxia. Thalamotomy does not give immediate results, but progressive benefit is seen over weeks to months. The surgery is not recommended by most functional neurosurgeons.
Deep Brain Stimulation of Globus pallidus interna (GPi) for all types of dystonia, especially dystonia associated with Parkinson’s disease and generalized dystonia.
This is the most common surgical procedures performed around the word. The detailed description of deep brain stimulation is given elsewhere on this website. It can be offered for a stand alone focal cervical dystonia or cervical dystonia associated with other disorders like generalised dystonia, secondary dystonia etc.
The success rate of this surgery is pretty high and around 70% to 80% of the Patient’s confirmed adequate suppression of symptoms and resumption of the activities of daily living. This is a relatively safe procedure and has been tried and applied to large number of patients around the world.
Improvement in symptoms is gradual and may take weeks to months post-surgery.
The advantage of DBS is that is has the additional benefit of titrability. We usually recommend DBS for patients having associated symptoms of dystonia in the upper limb or likely to develop dystonia or other body parts. In all other pure cervical dystonia patients, we recommend Cervical Rhizotomy.
For details of the procedure click on – Deep Brain stimulation
If you are diagnosed with Torticollis, there are few remedies to try at home that may help you to relieve symptoms.
Symptoms often fade during sleep, so you can take a break to lie down for some time and take rest.
Physical healing exercises for adults with torticollis may comprise graded (step-by-step) neck exercises. Initially you may start by trying to gently move your head a little further in each direction each time. Then, try to keep your head in the final position for longer periods of time. If you cannot complete these exercises on your own, you may have another person to support you with tender passive movements.
Try to find out the reasons behind stress, anxiety and work on the same. Meditation and yoga has shown improvements in patients. The signs and symptoms of cervical dystonia tend to worsen when you are stressed, so learning stress management techniques is also important.
Patients of idiopathic spasmodic Torticollis may benefit from the sensory tricks. The best way to know about this is by trying several tricks and sees which one reduces the dystonia.
– Touching the chin, back of the head, neck. face or cheek gently can help decrease neck dystonia
– The touch is usually (but not always) more effective on the side of the head with the dystonia.
– Reclining or sitting with head support.
– Try to rest the head against a wall.
– Using transportable back or neck supports.
– Using a body suit to assist posture.
– Looking at a fixed point or into a mirror.
Heat packs and massage can help relax your neck and shoulder muscles. Exercises that improve neck strength and flexibility may also be helpful.
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It is indicated in patients with cervical dystonia/torticollis who do not achieve adequate response with medical treatment or repeated botulinum injections or those who are non-responders to botulinum injections. Selective peripheral denervation is now accepted as the best surgical option for these patients. Overall, about one to two-thirds of patients achieve long-term improvement. It is not indicated in patients who have symptoms like blepharospasm and laryngeal spasm in addition to cervical dystonia.
In this procedure, nerves supplying the affected muscles of the neck are selectively cut. In peripheral denervation for torticollis, a small incision is given at back of neck. Nerves outside the spinal canal, which are going to the affected muscles, are first identified and stimulated under Neurophysiologic guidance and then they are cut. Each nerve is carefully identified and resected under microscope; in this way only the affected muscles are denervated without damaging any important structures. We confirm the adequacy of denervation with the help of signals recorded from each muscle before and after the resection of the nerves.
Selective denervation under microscope
This procedure is done under general anesthesia and hence there is no discomfort to the patient. Once denervated, the patient is turned on his back and the front of the neck is prepared. One of the muscles which is responsible for torticollis is sternocleidomastoid in the neck. So, one more small incision is taken in neck and nerve going to this muscle is selectively cut. Similarly, the nerve supplying the muscle which is responsible for elevation of shoulder in torticollis is selectively cut. As the whole procedure is done under Neurophysiological monitoring, the results are extremely good. The total duration of hospital stay is around 6-7 days.
After selective peripheral denervation for spasmodic torticollis, it is very important that patients do posture exercises to regain a sense of midline and to improve the range of movement. Although a denervation procedure for torticollis is not a curative treatment the overall improvement of the symptoms is generally 80–90%.
Patient undergone cervical rhizotomy ( Pre & Postop) Note the neck turning and rt shoulder elevation before surgery
