Spasmodic torticollis is a rare condition characterized by involuntary movement of the head. It is classified as a focal or segmental dystonia and occurs in approximately 3 in 10,000 people. It begins in early adult life.
The three varieties of spasmodic torticollis are as follows:
- • Tonic, which involves involuntary turning of the head to one side
- • Clonic, which involves involuntary shaking of the head
- • Tonic/clonic, which involves both types of involuntary movement
Spasmodic torticollis also can be subclassified based on the specific movement of the head: (1) rotation, which involves the turning of the head to the side; (2) laterocollis, which involves the leaning of the head against the shoulder; (3) retrocollis, which involves the leaning of the head toward the back; and (4) anterocollis, which involves the leaning of the head toward the chest.
The disease occurs more commonly in women and often is initially diagnosed as a hysterical reaction or tic.
Thought to be due to dysfunction centrally, rather than a disease of the affected muscles, spasmodic torticollis often begins as a subtle involuntary movement of the head.
Early in the disease, the dystonia is often intermittent.
As the disease progresses, the symptoms become more severe and harder for the patient to hide. The dystonic movements may become more sustained and associated with constant, aching pain in the affected muscles.
The pain often becomes the primary reason for the patient to seek medical attention, with the patient almost indifferent to the dystonic movements.
The dystonia often disappears with sleep and becomes less pronounced on first awakening, with the dystonic movements and pain worsening as the day progresses.
Spontaneous recovery has been reported, but, overall, treatment is difficult and of limited success.
What are the Symptoms of Spasmodic Torticollis
A patient with spasmodic torticollis exhibits involuntary, dystonic movements of the head. In extreme cases, the dystonia is continuous and the laterocollis so marked that the patient’s ear rests on the ipsilateral shoulder. Pain may be a predominant feature of the syndrome, and spasms of the cervical paraspinous musculature, the strap muscles of the neck, and the sternocleidomastoid are often present. Hypertrophy of the affected muscles may occur occasionally.
Other than the dystonic movements, the neurological examination is normal. As mentioned previously, the patient may seem indifferent to the abnormal head movements or position. Touching the opposite side of the face or chin often causes the dystonia to cease momentarily.
How is Spasmodic Torticollis diagnosed?
Magnetic resonance imaging (MRI) of the brain and brainstem should be performed in all patients thought to have spasmodic torticollis.
MRI of the brain provides the best information regarding the cranial vault and its contents.
MRI is highly accurate and helps identify abnormalities that may put the patient at risk for neurological disasters secondary to intracranial and brainstem pathological conditions, including tumors and demyelinating disease.
Magnetic resonance angiography (MRA) may be useful in identifying aneurysms responsible for neurological symptoms.
In patients who cannot undergo MRI, such as patients with pacemakers, computed tomography (CT) is a reasonable second choice.
Clinical laboratory tests consisting of a complete blood cell count, automated chemistry profile, and erythrocyte sedimentation rate are indicated to rule out infection and malignancy.
Spasmodic torticollis is generally a straightforward clinical diagnosis that can be made on the basis of a targeted history and physical examination.
The involuntary nature of this movement disorder is the hallmark of the disease and helps distinguish it from tics and habit spasms that are voluntary and worsen when the patient is tense. Tics and habit spasms resemble volitional movement.
Behavioral abnormalities, such as hysterical conversion reactions, also must be considered. Acute spasm and pain of the muscles of the neck or wry neck can mimic spasmodic torticollis, but its onset is acute, and the symptoms usually resolve within days to a week.
Occasionally, patients with clonic spasmodic torticollis are initially diagnosed as having Parkinson disease.
In general, the treatment of spasmodic torticollis is disappointing. Physical interventions including biofeedback and patient self-performed geste antagoniste, for example, touching one’s chin, may provide some symptomatic relief.
Pharmacological treatment with skeletal muscle relaxants, including drugs that act at the spinal cord level, such as baclofen, and centrally acting drugs, such as the anticonvulsants and levodopa, may provide some symptomatic relief in mild cases. Trihexyphenidyl, dopamine agonists, and diazepam also have been advocated.
In patients for whom pharmacological treatment fails, injection of the affected muscles with botulinum toxin is a reasonable next step.
Frequent injections may result in the development of antibodies against the toxin, which makes the toxin less effective. By changing to different subtypes of toxin, efficacy may be restored.
For intractable cases, microvascular decompression of the spinal accessory nerve, neurodestruction of the spinal accessory nerve, deep brain stimulation, and bilateral thalamotomy has been advocated. The results of treatments are variable at best.
Although spasmodic torticollis is usually a straightforward clinical diagnosis, the clinician must always rule out other pathological processes involving the central nervous system.
Treatment of this syndrome is difficult, and treatment of concurrent depression is often required.
Spasmodic torticollis is a devastating disease that responds poorly to treatment.
Injection of the affected muscles with botulinum toxin to effect chemodenervation is probably the best therapeutic option for most patients.
The diagnosis of the disease is straightforward. MRI of the brain is indicated in all patients thought to have spasmodic torticollis.