From the Editor
There are a lot of reports
on the Internet of people with dystonia who have managed to reduce their
spasms or change the pattern of their spasms through one form or another
of corrective exercises. Some are able to correct deviations of the head,
for example, by concentrating on a good body posture at all time. Others
have gone through intensive physiotherapy and found that they are able
to regain a certain control over their affected muscles. Others still,
using biofeedback or visualization techniques are now able to do things
they were not able to do before, such as moving their head in all directions
with ease or walking without it being an unpleasant experience. One of
our group member, working with his neurologist, tried various methods to
immobilize the neck for a prolonged period of time, with the result that
the spasms have decreased slightly and some of the twitches have disappeared
We are told that this has
to do with the "plasticity" of the brain – some parts of the brain that
are affected by dystonia can somehow be remolded or remapped. So we have
asked Rosalie Labelle, who has a Master's degree in Neuroscience and has
recently joined our group of volunteers, to look into this and tell us
what science has to say on this matter. As you can see in this article
she wrote for us, it turns out that recent findings regarding brain plasticity
and dystonia shed a new light on our movement disorder and point to encouraging
prospects for future therapies that may help improve, if not cure some
forms of dystonia.
Of course this is simply
part of our ongoing effort to try to understand dystonia better and support
our members in their attempts to improve their situation when everything
else fails. For the time being, Botox and medications remain the most effective
treatments for dystonia, but many are looking at other ways to decrease
their spasms. For some, Botox no longer works and for others medications
have become too difficult to live with.
On behalf of the group I
would like to thank Rosalie for this excellent report.
Dystonia and Brain Plasticity
by Rosalie Labelle
(for the Newsletter of
the Ottawa Area Dystonia Support Group)
The brain is plastic. Connections
in the brain can organize and reorganize in response to experience or sensory
stimulation (Kolb and Whishaw, 1998). While the brains' plastic nature
is positive and adaptive, it can also be maladaptive. The ability of the
brain to remap itself is of interest to researchers studying dystonia.
The somatosensory cortex of the brain
receives information regarding bodily sensation such as touch. The inputs
from different body parts are laid out in an orderly arrangement that segregates
information from adjacent areas (ie the hand and wrist) in adjacent parts
of the cortex (Holmes, 1999). A similar topography exists in the motor
cortex, where nearby brain areas control neighbouring muscle groups (Holmes,
Researchers believe that there is
a disorder of sensory function in dystonia (Hallett, 2000, and Byl et.
al., 2000). A role of the sensory system is to drive the motor system;
hence disordered sensation can lead to disordered movement (Hallett , 2000).
Byl et. al. (2000) report degradation in hand representation (ie disorganized
electrical activity) in the somatosensory cortex of a flutist with focal
hand dystonia when compared to the hand representation of a healthy flutist.
Other studies in humans support these findings (Elbert et. al., 1998 and
Bara-Jimenez et. al. 2000).
on monkeys indicate that brain changes can be pathological and lead to
motor dysfunction. Monkeys developed movement control disorders following
completion of training that required them to carry out rapid, repetitive,
highly stereotypic hand movements (Byl et. al., 1996). Degraded hand representations
were found in the somatosensory cortex of the monkeys (enlarged, overlapping
receptive fields). This study showed that the brain is constantly refining
maps and may confuse the information when repeated rapid movements happen
simultaneously, leading to diminished control over hand movements. Merzenich
and colleagues found rewiring in the brain's sensory body map in monkeys
following training that required them to use their hands repetitively.
After training it was found that the usually precise map in the sensory
cortex had blurred so that a single neuron might respond no matter which
finger they touched, indicating that the brain had rewired and lost resolution
Maladaptive brain changes have been
reported by Nudo et. al. (1996 ) in the motor cortex of monkeys following
rapid, stereotyped, learned hand movement. The muscles the monkeys used
responded to stimulation over a larger patch of the motor cortex. Before
training each spot on the cortex had controlled just one muscle. After
training some spots controlled two or more muscles that the monkeys had
used to simultaneously perform the task that they were trained to do. The
cortex now treated the compound muscle movements as a single stereotyped
action, displaying overlapping regions that now controlled muscles of the
wrist and fingers (Holmes, 1999). The muscles were no longer able to act
with precision and movement control disturbances resulted.
Byl et. al. (1996) proposed that
human focal hand dystonias could be treated by making use of the brain's
ability to make positive, adaptive changes. According to Byl in order to
change the nervous system a task has to be highly attended and repetitive.
While tasks that are highly attended, very ritualistic, repetitive and
near simultaneous will lead to degradation, tasks that are highly attended,
repetitive and variable lead to improved performance (Ryl, 1999). Byl's
training exercises (ie to play dominoes by feel) forced patients to make
more delicate discriminations with their fingers in order to help them
to relearn fine distinctions between neighbouring finger representations
in the cortex. Following their training patients displayed lasting improvements
in function. Brain scans of one patient before and after therapy showed
that the sensory map was moving towards a more normal arrangement (Holmes,
1999). Recent reports support Byl's findings. Zeuner et. al. (2002) reported
that 60% of patients with focal hand dystonias, who were trained in Braille
reading, required shortened times to write a standard paragraph.
Byl's results are promising and suggest
that traditional treatments for focal hand dystonia should be reassessed.
Byl's retraining requires high levels of repetition, and thus it may not
be the type of task that all patients are willing to perform (Rayl, 1999).
Some authors believe immobilization and rehabilitation might boost each
other, leading to improved therapy (Priori et al., 2001). Priori and colleagues
(2001) state that limb immobilization for the treatment of severe dystonia
of the hand and forearm, probably acts by prompting inactivity dependent
plastic changes, reversing the functional abnormalities present at the
cortical level. In nondystonic subjects prolonged limb immobilization causes
the motor cortical representation of the immobilized limb to shrink. Priori
believes that prolonged immobilization would normalize the abnormally enlarged
cortical representation of dystonic muscles. Priori et al state that their
observations fit with the hypothesis of activity dependent neuroplasticity.
In their protocol inactivity dependent neuroplasticity (a sort of detraining)
would be beneficial to focal dystonia sufferers because prolonged immobilization
would normalize the abnormally enlarged cortical representation of dystonic
experiments have focused on focal hand dystonia, thus the consideration
of the application of Byl's principals for the treatment of other forms
of dystonia would be of interest. Retraining methods have been used in
the treatment of spasmodic torticollis. De Benedittis (1996) found that
hypnosis and biofeedback could be used successfully to retrain neck muscles
to reduce symptoms in four patients with spasmodic torticollis.
Retraining studies that make use
of the brains’ plastic nature have been successful at reducing the symptomology
of focal hand dystonias in humans. These studies indicate that traditional
methods for the treatment of focal hand dystonias require re- evaluation
in light of new findings.
Bara-Jimenez, W., Shelton, P. and Hallett, M. 2000.Neurology.
Spatial discrimination is abnormal in focal hand dystonia. V. 55 (12) pp
Byl, N.N., McKenzie, A. and Nagarajan, S.S. 2000. Journal
of Hand Therapy. Differences in somatosensory hand organization in a healthy
flutist and a flutist with focal hand dystonia: a case report. V. 13 (4)
Byl, N.N., Merzenich, M.M. and Jenkins, W.M. 1996. Neurology.
A primate genesis model of focal dystonia and repetitive strain injury:
I. Learning induced dedifferentiation of the representation of the hand
in the primary somatosensory cortex in adult monkeys .V. 47 (2) pp 508-520.
De Benedittis, G. 1996. International Journal of Clinical
and Experimental Hypnosis. Hypnosis and spasmodic torticollis--report of
four cases: a brief communication. V. 44 (4) pp 292-306.
Elbert, T., Candia, V., Altenmuller, E., Rau, H., Sterr,
A., Rockstroh, B., Pantev C. and Taub, E. 1998. Neuroreport. Alteration
of digital representation in somatosensory cortex in focal hand dystonia.
V. 9 (16) pp 3571-3575.
Groves, P.M. and Rebec, G.V. 1988. Introduction to Biological
Psychology. Wm. C. Brown Publishers. USA.
Hallett, M. 2000. Brain. Disorder of movement preparation
in dystonia. Vol. 123, No. 9. pp 1765-1766.
Holmes, B. 1999. New Scientist. The strain is in the brain.
V. 162, issue 2181. p26.
Kolb, B. and Whishaw, I.Q. 1998. Annual Review of Psychology.
Brain plasticity and behavior. V. 49 pp 43-64
Nudo, R.J.et. al. 1996. Journal of Neuroscience. Use dependent
alterations of movement representation in the primary motor cortex of adult
squirrel monkeys. V. 16. pp 785-807.
Priori, A., Pesenti, A., Cappellari, A., Scarlato, G.
and Barbieri, S. 2001. Neurology. Limb immobilization for the treatment
of focal occupational dystonia. V. 57 (1 of 2) pp 405-409.
Rayl, A.J.S. 1999. The Scientist. Research: Recent Findings
Lead to New Understanding of Dystonia. V. 13 (3): 14.
Zeuner, K.E., Bara-Jimenez, W., Noguchi, P.S., Goldstein,
S.R., Dambrosia, J.M. and Hallett, M. 2002. Annals of Neurology. Sensory
training for patients with focal hand dystonia. V. 51 (5): 593-598.
* Rosalie Labelle is a volunteer with the Ottawa Area Dystonia Support
Group and has a Master's Degree in Neuroscience.
For information: Yvon
Breton, Hull, Quebec