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Spasmodic
Torticollis
Spasmodic
Torticollis, also known as Cervical dystonia, is a focal dystonia in which neck
muscles contract involuntarily, giving rise to abnormal movements and posture of
the head and neck. This term is generally used to describe spasms in any
direction: forward (anterocollis), backwards (retrocollis), and sideways
(torticollis). The movements may be sustained or jerky. Spasms in the muscles or
pinching nerves in the neck can give rise to devastating pain.
Spasmodic
Torticollis should not be confused with other conditions giving rise to a
twisted neck: local orthopaedic or congenital problems of the neck,
ophthalmologic conditions where the head tilts to compensate for double vision,
or a specific manifestation of hiatus hernia in children. Spasmodic Torticollis is due to
abnormal functioning of the basal ganglia, which are situated at the base of the
brain and control all co-ordinated movements. In Spasmodic Torticollis, the
motor program for neck movements is at fault, and the neck muscles contract
involuntary in various combinations. Sustained contractions give rise to
abnormal posture of the head and the neck, while periodic spasms produce jerky
head movements. The severity may vary from mild to severe.
What goes wrong in
the basal ganglia is still unknown. It is generally believed that there is a
disturbance of the delicate balance of various chemical transmitters involved in
conveying messages from one nerve cell to another within the basal ganglia.
Spontaneous recovery
has been reported in about 10% of patients, but this is highly unpredictable.
Usually the torticollis reaches a plateau and remains stable within five years
of onset. This form of focal dystonia is unlikely to spread to become
Generalised Dystonia, though patients with Generalised Dystonia may also have Torticollis.
Patients usually
have no neurological deficit other than Torticollis. About 20%, however, may
have a fine tremor of the hand, head and occasionally the voice. This is called
Essential Tremor.
Treatment in
general
Since the underlying
cause of Spasmodic Torticollis remains unknown, there is at present no cure for
the condition. Treatment is directed towards symptomatic relief, the patients
usually require treatment when they feel the condition is giving rise to
disabling pain or when the abnormal neck posture and movements is causing them
social embarrassment or is threatening to the job. It is often partially
relieved by a gentle touch on the chin or other parts of the face.
Medical treatment
Various medications
are used for treating Spasmodic Torticollis. They have totally different
mechanisms of action and generally produce unpredictable and short lasting
benefits. One drug may work for some patients and not for others. When the
effects of one drug wears off, sometimes the replacement with another drug
helps. There is, therefore, no fixed or best regimen. Establishing a
satisfactory treatment scheme requires patience on the part of both the
physician and the patient.
Some of the
medicines your doctor might consider include:
Artane
(trihexyphenidyl).
Cogentin (benztropine).
Valium (diazepam).
Klonopin (clonazepam).
Lioresal (baclofen).
Tegretol (carbamzepine).
Sinemet or Madopar (levodopa).
Parlodel (bromocriptine).
Symmetrel (amantadine).
Nitoman (tetrabenazine) is often effective, but not always available.
Thorazine (chlorpromazine) and Haldol (haloperidol) and other medicines of the
phenothiazine or butyrophenone groups may help but may produce a side effects
called tardive dyskinesia and should be used with great caution.The list is by no
means complete, and there are many more new drugs being developed. The use of
these medications requires close supervision from a neurologist, and it is
important that the patient does not change the dosage or stop the medications
without the neurologist’s approval.
Surgical
treatment
Surgery is
undertaken to interrupt, at various levels of the nervous system, the pathways
maintaining the abnormal neck movements. Some operations intentionally damage
small regions the thalamus (thalamotomy), globus pallidus (pallidotomy), or
other deep centres in the brain.
Other surgical
approaches include severing one or more of the contracting neck muscles (muscle
resection), cutting nerves going to the nerve roots deep in the neck close to
the spinal cord (anterior cervical rhizonotomy), and removing the nerves at the
point they enter the contracting muscles (selective peripheral denervation).
We do not recommend
most of these procedures. However selective peripheral denervation or
thalamotomy may be considered in very severe cases when other treatment
modalities, including botulinum toxin, have failed and when done by the few
neurosurgeons who have significant experience in these specific operation.
Botulinum toxin
injections
Botulinum toxin is a
toxin produced by bacteria Clostridium botulinum. It paralyses muscles by
blocking the impulse transmitted from the nerve endings to the muscles. In very
large quantities it can produce fatal paralysis of respiratory muscles.
When diluted and
given intramuscularly in extremely small quantities, it can give rise to
selective paralysis of the injected muscle and is safe. In Spasmodic Torticollis, the abnormally overactive neck muscles can be identified by
clinical examination and palpation.
These muscles can be
injected with the toxin. Occasionally, an electroymyogram (EMG) can confirm or
aid in the identification of overactive neck muscle. Studies of this treatment
have been shown to produce significant relief of pain in over 85% of patients
and improvement in the Torticollis in about 70%.
The improvement
following treatment lasts for about three months when patients need to be
re-injected. The injections cause very little discomfort, are well tolerated,
and produce no significant side-effects.
Difficulty
swallowing occasionally occurs. This however, has been mild, transient, and
infrequent. Resistance to the toxin after repeated treatments is rare.
Supportive forms
of treatment
Stress makes all
movement disorders, including Torticollis, worse. Some patients may benefit from
a trained professional in learning stress management.
A physiotherapist
may be able to help patients with Torticollis through an acute episode of pain
and /or spasm through the use of local moist heat, ice or ultrasound. A trained
physiotherapist can suggest exercise and fitness programs suitable for the
disability and can advise on the maintenance of good posture and strength in the
back and shoulder muscles which are often secondarily affected by Torticollis.
Treatment involving
manipulation of the neck is not recommended for Spasmodic Torticollis.
For someone with
Torticollis, an Occupational therapist may be able to help functional disability
with the use of a soft collar. Sometimes a custom fitted soft collar is
necessary. A patient may use a collar in public to prevent unwanted questioning
from strangers. The Occupational Therapist can suggest adaptations in the home
or workplace which will reduce fatigue, promote safety, and improve the mobility
for the patient with Spasmodic Torticollis. Support from family and friends is
important. Thousands of people are experiencing the same symptoms.
Taken from the
Internet via the Dystonia Medical Research
Foundation
,
USA
.
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