Spasmodic Torticollis

Dystfndt at aol.com Dystfndt at aol.com
Wed Apr 19 16:27:31 EST 1995


What is Spasmodic Torticollis?
Spasmodic torticollis, also known as cervical dystonia, is a focal dystonia
in which neck muscles contract involuntary, giving rise to abnormal movements
and posture of the head and neck.  This term is generally 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 orthopedic 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.

Torticollis is due to abnormal functioning of the basal ganglia, which are
situated at the base of the brain and control all coordinated movements.  In
spasmodic torticollis, the motor program for neck movement is at fault, and
the neck muscles contract involuntary in various combinations.  Sustained
contractions give rise to abnormal posture of the head and 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.  Though a history of head or neck injury
may be obtained in some patients, there is yet no clear evidence to support
the theory that torticollis is directly related to trauma.

Prognosis of Spasmodic Torticollis
Spontaneous recovery has been reported in about 10 percent 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 generalized dystonia, though patients with
generalized dystonia my also have torticollis.  Occasionally, there may be
associated focal dystonia of the eyelids, face, and hand.

Patients usually have no neurological deficit other than torticollis.  About
20 percent, however, may have a fine tremor of the hand, head, and
occasionally the voice.  This is called essential tremor.

Torticollis is sometimes hereditary.

Since the underlying cause of spasmodic torticollis remains unknown, there is
as present no cure for the condition.  Treatment is directed towards
symptomatic relief, the patients usually requiring treatment when they feel
the condition is giving rise to disabling pain or when the abnormal neck
posture and movement 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 (Pharmacological) 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.

The drugs that are frequently used include:
Some of the medicines your doctor might consider include: Artane
(trihexyphenidyl), Cogentin (benztropine), Valium (diazepam), Klonapin
(clonazepam), Lioresal (baclofen), Tegretol (carbamzepine), Sinemet or
Madopar (levodopa), Parlodel (bromocriptine), Symmetrel (amantadine).
 Nitoman (tetrabenazine) is often effective, but it is not available in the
United States.  Thorazine (chlorpromazine) and Haldol (haloperidol) and other
medicines of the phenothiazine or butyrophenone groups may help but may
produce a side effect 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, ant it is important that the patient 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 of the thalamus (thalamotomy), globus
pallidus (pallidotomy), or other deep centers 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 be
the few neurosurgeons who have significant experience in these specific
operations.

Botulinum Toxin Injections
Botulinum toxin is a toxin produced by bacteria Clostridium botulinum.  It
paralyzes 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 elctromyogram (EMG) can confirm or
aid in the identification of overactive neck muscles.  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 treatment
is rare, though circulating antibodies have been detected in about 10 percent
of patients.  The significance of this remains to be studied.

Based on this experience, botulinum toxin injection appears to be the best
treatment for spasmodic torticollis.

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.  Thousand of person are
experiencing the same symptoms.  The Dystonia Medical Research Foundation has
numerous support groups where sharing the experience will reassure a patient
and his/her family.

For more information write the Dystonia Medical Research Foundation, One East
Wacker Dr., Suite 2430, Chicago, IL 60601-2001, Phone 312-755-0198, Fax:
312-803-0138, e-mail: dystfndt at aol.com




More information about the Bioforum mailing list