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Why Do We Have To Work So Damn Hard?
Good question - and so many of
us continue to ask this question over and over again! It seems there
is just something about us that will not let us progress as we would
like to. We exercise, we struggle within our abilities and
disabilities, we try to walk better, to just move better, but don't
seem to have much luck. We take specific instructions from
experts hoping they can help us, but, we also find that is also difficult
and our rewards seem to be few.
We have a good deal of
difficulty just trying to mimic the movements we wish to learn.
And if that isn't bad enough, once we do come to understand how to
make ourselves mimic those movements, invariably we are faced with
yet another obstacle. How many times have you taken a couple days
break to recuperate from your increased clumsiness, which we
encounter because we're having such a difficult time reconditioning
ourselves, and then, when you attempt to repeat what you learned
just a couple days ago, you can't seem to remember what it was
that you learned? This happens to us repeatedly. And
with you, being just like me, you find it so very frustrating.
Ha!
That's really putting it lightly isn't it! Because, the fact
is, we don't just become frustrated. We become frustratingly pissed off at ourselves,
this condition and our plight! Talk
about depressing!
Because of this "problem" we
quickly begin to adopt the belief that we can't do or learn this
stuff... no matter how hard we try... we just can't. We can't
seem to move any differently than what we are stuck with. We cannot
seem to advance like everyone else. And what is worse, and because
they are "normal", the other people just keep right on going without
us. A quick little review of what we all learned last week, yep,
they got it, and they move on to whatever is next. But us... we
can't "move on"... we are left confused, depressed, frustrated,
alone...and the fun is gone. We ask ourselves again, "What the hell
is wrong with me? Why can't I remember this crap?" You just want to
hide in a dark room and cry. Hopefully the following article I
wrote will provide you with some of those answers to these long
burning questions and frustrations. You will find there is a
rationale reason...and there is also a rationale remedy.
SPINOCEREBELLAR ATROPHY
CLINICAL RELAVENCE
Thomas L. Clouse, M.D.
HISTORICAL PRESPECTIVE:
While a vast amount of information has been compiled in regards to
the pathophysiology, genetic perturbations, clinical presentation,
expected progression, and thus, the disabling aspects of
Spinocerebellar Atrophy (SCA), there is yet to be found any
realistic treatment for the afflicted individuals. This leaves the
treating physician in a very precarious position as they are forced
to inform the patient that the patient themselves must learn to
adapt and cope with their degenerative disease process while the
medical research community diligently searches for the elusive
treatment and hopeful cure.
Thus, the individuals suffering with the effects of this
progressively degenerative disease are remanded to witness
themselves being transformed into an increasingly more clumsy and
dependent person. Gradually their mobility, careers, livelihood,
self respect and social interaction will be, and is, stripped from
them. With the knowledge of this inevitable outcome, a select few
of these patients have been afforded a trial of physical therapy in
hopes of providing an opportunity where they might restore some of
their mobility. However, the ultimate restoration of meaningful
motor skills unto these patients has essentially been an illusion
within the reality of their disorder despite the best of efforts.
Fortunately, additional emphasis has been given to this problematic
issue as we witness an increasing number of Movement Disorder and
Balance Centers being established, and these centers have
contributed much valuable information to our understanding of this
disorder and the function of the cerebellum. But these centers are
too few and to date there remains no viable therapeutic model which
provides these patients a venue to seek their illusive
rehabilitation of motor control.
The
medical community as a whole is ill prepared in their understanding
of this disorder, and therefore, greatly restricted in their ability
to properly care for these patients or offer any viable treatment
options. Hence, there is an unfortunate undercurrent which is
harbored by much of the medical community. There is a shared belief
that the ataxic movements and postures which the SCA patient
exhibits are a direct result of the pathophysiology of this disease
process. Thus, it is conjectured that the individual patient really
has no direct control over their ataxic condition, and therefore,
any attempts with physical therapy efforts are relatively mute.
This norm in the mainstream medical practice setting has been very
detrimental for those suffering with SCA. Thus, the majority of
these patients find themselves stranded, and without the
availability of adequate resources for evaluation or therapy
options. Thus, the SCA patient is often left to their own accord to
find resources and methods in hopes of discovering “something” which
will help them cope with their advancing disorder. This inevitable
process promotes a growing frustration for all parties involved and
leads the patient into a deepening depressed mental state, from both
intrinsic and extrinsic sources 3, 24, and subsequently
forces them farther into their impaired degenerative state.
From
a patient’s perspective, they are forced to succumb to the
sinusoidal flow of increasing loss. Their losses begin with an
erosion of normal movement abilities; those increasingly
uncoordinated movements and postures which progressively thrusts
them further and further into a dependent, disabling lifestyle
coincident with a birthing of relative social isolation. They begin
to be scrutinized by employers, friends and the public in general.
As their ataxic movements and behaviors become more pronounced they
experience the loss of career, income, independence and self
respect; and are frequently viewed with the stigmata of also being
intellectually challenged3. Embarrassed by their growing
clumsiness, these individuals retreat from activities which have
become challenging, and subsequently, they experience a further
decline in their movement skills from lack of use. They
increasingly avoid more situations where they have found, or
suspect they may find, inherent difficulties in negotiating
through the growing obstacles within our society and are constantly
fearful of falling, injury, humiliation and social ridicule. Their
world fills with the reality of becoming forced into avoiding some
of the daily routines of daily living. Many of the recreational
activities they once enjoyed, and took for granted, become a distant
memory for they no longer have the motor skills to participate.
Their choices become very limited in every aspect of their lives
secondary to the ramifications of this degenerative neurological
disorder.
Moving,
just the act of walking, progressively requires more and more of
their undivided attention as the selective degeneration of their
neurological system progresses. They lose the ability to maneuver
safely within confining spaces, especially those filled with other
people, through obstacles, over changing terrain and steps of any
sort. These disabled individuals quickly become fearful and timid
with their movement activities. Their posture takes on a relative
rigidity, their movements increasingly become more uncoordinated and
they lose the simple ability of normal ambulation, stepping and
turning. It is therefore not surprising to find that these
individuals become relative social isolates, a good degree of which
is self imposed, and that their isolation grows deeper as their
activities steadily become more sedentary. With this increasingly
sedentary lifestyle they become weaker as their muscle tone
diminishes. Thus, they become more ataxic and require more external
walking aides, which causes them to become even more sedentary, and
thus, ever so much weaker. They are on a “slippery slope” for which
almost all of them never recover as this process becomes self
perpetuating and unstoppable.
RECENT
UNDERSTANDING
I refute the belief that the SCA patient’s ataxic movement disorders
and postures are simply a direct effect of the pathopysiology of
SCA. It is my contention that the known neurological changes noted
with this disorder instead have only an indirect role in this
regard. With a growing body of evidence, I believe that it can be
clearly demonstrated that the SCA patient can indeed experience
dramatic results in correcting many of these physical manifestations
with proper physical and psychological therapeutic interventions. I
further argue, that with the proper learning environment,
instruction, guidance and support system, many of these patients can
be effectively rehabilitated to exhibit the renewal of near normal
movement skills; the degree of which appears to be dependent on the
state of their physical conditioning, ambulatory abilities, attitude
and pathological nature of their unique SCA subset upon entry into
these interventional modalities.
How
can I claim to achieve such dramatic results when the literature
seems to predict this not to be a probable outcome? In effect,
previous literature 21, 22, and medical dogma 23,
suggests that while physical therapy is beneficial, it has a very
limited positive impact on the eventual degenerative progression of
the disease process the individual will naturally follow, and with
this being known and accepted, the SCA patient’s motor control and
quality of life is not effectively altered through these attempts.
However, I contend that these findings were skewed in light of the
methods, duration of treatment, understanding of some of the unique
issues and problems which these patient’s experience, and the
expected goals which were in vogue at the time.
The belief that the diminished motor control capabilities these
patients exhibit is largely secondary to the pathophysiological
degenerative process of SCA 21, and these pathological
changes are therefore totally responsible for the increasingly
ataxic movements which the afflicted individual demonstrate, and the
patient is therefore without any control unto themselves, provides
the disease process with too much credit . Arguably, this accepted
belief provides for an enabling of this norm to continue with care
providers, patients and families helplessly viewing the inevitable
outcome. However, I argue that while this degenerative process is
in fact responsible for a great deal of the problematic issues
inherent to this disorder 1, 2, 5, an overlooked major
contributing factor is the de-conditioning of the individual
patient’s physical and mental capabilities, and to this end, the
patient most certainly does harbor some personal control should they
choose to accept it.
THERAPEUTIC
ISSUES
It
is a common occurrence for the SCA patient to be instructed by their
treating physician that there exists no therapy available which has
the ability to offer them any realistic benefit 21, and
at best all they can be offered is palliative care. 23 (In
reality the patient hears, “No help, no hope, no cure, learn to deal
with it”.) In part, the palliative care entails a
referral to a physical or occupational therapy facility to receive
instructional assistance to hopefully improve the patient’s coping
skills relative to their movement disabilities and become fitted
with an assistive device. 23
It
is my contention that these therapeutic interventions are flawed for
a number of reasons. These interventions do not appropriately
address the patient, rather they are directed at the disorder.
Further, the treatment has a systems based approach instead of a
comprehensive approach. In this light, any physical rehabilitative
effort which only addresses a portion of the problem, rather than
the entire patient as a whole, will be lost and forgotten in a very
short time as has been anecdotally noted by the medical community,
families and most notably the patients themselves. Thus, the SCA
patient has been deemed to be untreatable for any rehabilitative
efforts.
For
example, the patient being referred to a physical therapy facility
secondary to increasing episodes of falling is seen by a therapist
who has been given instructions to “evaluate and treat patient’s
gait, assistive devices as required”. Thus, in this setting the
emphasis of the therapist’s treatment efforts will be directed to
this “unit system” of the patient, and likely not enough attention
will be provided to the other physical and mental contributing
factors which all of these patients harbor. Efforts to strengthen
the legs, teach techniques for proper weight transfer, stepping
actions and balance control will certainly be addressed, and most
patients will experience short term improvement, but the therapeutic
intervention ultimately falls short of the hopeful and somewhat
arbitrary goals. As is the rule, the patient’s mobility is aided to
some degree by also fitting them with an assistive device and they
are taught how to move with this foreign object with the hopes they
will demonstrate improvement in their movement abilities. Were
these patients provided with the therapy they required? In
yesterdays world of understanding, yes they were; but not today’s.
These
patients ultimately lost any new abilities they gained through
therapy because they did not properly learn how to use the
information and techniques. It has been my experience that the SCA
patient requires a total body approach when attempting to teach them
movement skills to gain the best results. Basically, this is
secondary to the total body de-conditioning they all experience to
some degree secondary to the learned compensatory movements which
have become the perceived normal mannerism they adopt in an effort
to attain their individual rendition of functional movement patterns
in dealing with their level of ataxia. Further, they are
embarrassed by their lack of motor control and easily humiliated and
withdrawn if the therapy sessions are not within an environment
where they feel comfortable; and this factor cannot be overlooked or
under emphasized. In the setting were this is not brought
into the treatment equation only mediocre results will be achieved
at best.
Patients
with Spinocerebellar Atrophy have unique problems. One of their
recently recognized deficits is that they have developed a cognitive
sequencing impairment 7. Thus, they often have
difficulty understanding how to interpret and mimic a demonstrated
movement 1, 2, 4, 6, 18, 20 which necessitates that the
task must be deliberately repeated ad nausuem before the SCA
patient is able to mimic and learn it. During this process the
patient easily becomes frustrated and the therapist routinely cannot
comprehend why the patient “is not getting it”. Therefore, these
individuals must have an experienced therapist who has the ability
to recognize not only this learning impairment, but also the many
other issues these patients are attempting to cope with.
As
opposed to earlier assumptions 22 it is now well known
that the cerebellum is required for cognitive function and
sequencing, as well as learning and motor control, 9, 17, 19
and accounts for their cognitive sequencing impairment 7.
Therefore, as the cerebellum undergoes the characteristic
degenerative changes, the SCA patient will indeed experience
learning difficulties and require more time to acquire new skills,
as compared to persons without cerebellar problems 6, 7, 8, 10,
11, 13, 15, 20. Thus, these patients must be afforded a
longer duration of time in the learning process for them to
comprehend, learn and execute the material being presented to them.
This is a portion of the reason why the SCA patient lives within a
reactionary world. The reactionary world they have been forced to
learn and become accustomed, to react to other stimuli around and
within them as their ataxic movements become more pronounced, and
thus lose the ability to anticipate their movements. The other
portion of the credit resides within the psyche of the patient.
These patients no longer trust their failing movement skills.
Constantly they are attempting to correct internal perturbations
which they themselves induce and to which their resultant posture
becomes stiffened in an effort to stabilize themselves. Within
their world they fail to realize that all of these adopted behaviors
have a drastic negative effect on their movement abilities - for
they have come to believe they are actually “stabilizing” themselves
in an effort to avoid falling.
The
SCA patient progressively loses the ability to perform in an
anticipatory capacity. This is secondary to two interdependent
factors. First, these capabilities are blunted secondary to their
ataxic postural (mis)adjustments and subsequent learned behaviors.
Secondly, it has been discovered that “predictive feed forward
adaptations require cerebellar control and the subjects studied with
cerebellar damage had reduced and sometimes absent adaptation “to a
new task they had been asked to perform”. 11, 18
Clearly these patients require additional time to allow cerebellar
plasticity 12, 16, 17 to occur and thus allow for the
acquisition of new motor skills. 18, 19
In
order to offer the best care to the SCA patient, the medical
community charged with their care must recognize and control for
this new information. Additionally, they must have the ability to
understand how and why the patient has developed their unique
movements and postures, how these movements are transmitted,
reinforced and supported by other muscle groups (which many times
are contrary to normal movement patterns), what modifications will
be required which will enable the ataxic individual to learn the
task being taught (i.e., the therapist must be able to recognize and
anticipate these aberrant movement patterns and be knowledgeable of
their meaning in relation to what purpose they serve the patient),
how to best convey this information to the patient to instill a good
learning environment, have an understanding of the SCA patients
learning deficits, have a patience level with the patient which is
conducive with the task, maintain a supportive attitude, maintain
the therapy session in a manner which is not demeaning to the
patient, is enjoyable, and also supports the patient’s goals. These
sessions can be very daunting for all that is required to enable the
staff and the patient to continue in a positive environment while
preserving the slow progression of the patient’s abilities in an
effort to fulfill their collective goals.
A
REALISTIC APPROACH
I
have recognized and understand these factors, and have pioneered an
approach which is better suited to the ataxic individual; better
suited in that it addresses their particular problems. With my
understanding of these individual’s unique problem areas, the
solution has become understandable and the remedy achievable. To
some my approach seems novel, yet remains very simplistic. And upon
that recognition they ask, “Why hasn’t someone told us about this
before? Why don’t my doctors and therapists know about this?” The
answer to this question is again rather simplistic. They don’t
understand us. Yes, I said us. I also have spinocerebellar atrophy
(SCA 14) and live within the ataxic world. I share the plight. I
share the frustrations. I share the shame. I’ve also searched for
the “something” to help, some sort of therapy, found it, refined it,
and now share it with the others like myself.
My
approach achieves positive results – and these begin to appear
fairly quickly. How quickly and to what degree is dependent on the
individual. It is dependent on their level of physical conditioning
and ambulation. There are no external devices used other than
possibly a cane or walker. There are no pills, potions or
apparatuses. There are no high tech pieces of equipment, computer
aides or balancing devices ever used. The only things we use are
what the ataxic person has within themselves. We begin with the act
of simple observation then progress through the basics – to which
these ataxic individuals respond very well. Simply put, they amaze
themselves. Much of what they have been told they cannot do – they
find they can. Much of what they have been told cannot be corrected
– they find that it can.
With
the acquisition of new motor skills these individuals slowly became
more independent in movement, and thought, as they experience a
euphoria which has long been suppressed. As they experience
physical obstacles beginning to diminish, they also experience
psychological obstacles beginning to erode as well. In principal,
as these individuals become more adept with their increasing
independence of motor control, so do they begin to exhibit an
identical transition of thought, well being and desire to further
their achievements. It is clearly evident that as these individuals
begin to regain lost motor control, they also are developing an
“enabling” psyche which further allows them to give themselves
permission to not only continue, but to also make better strides to
learn additional skills. Thus, within this process they are also
beginning to experience a form of re-socialization and an improved
quality of life. In short, provide the ataxic individual with more
independent mobility, safer and effective movement skills, give them
the power to get some of their “normal” back, and they will fill in
much of the rest on their own.
My
approach is found within the information contained within my website
www.walkingwithataxia.com.
While the content of this site does not contain the approach in its
entirety, the beginning basics can be found there. It is our hope,
the people suffering with SCA and similar diagnoses, and the many
other individuals suffering with ataxic movement disorders, that the
medical community will begin to recognize and adopt what we have
found to be true in this regard. Further, as we the affected
individuals have found this simple approach to be so beneficial,
having the potential towards rehabilitative efforts, that it be
adopted and approved as such by the medical insurance entities. Can
you imagine the newly diagnosed individual with this disorder who is
simultaneously channeled into an effective movement skills program
which has the ability to prevent them from developing much of what
we now see these patients develop? Can you imagine what impact this
would have on their lives? I can. And it is so simple – yet so
elusive. Help us change medical dogma. There is a treatment for
SCA in the realm of movement skills.
References
1.
Lang, CA, Bastian, AJ. Learning to predict the future: the
cerebellum adapts feedforward movement control. Current opinion in
Neurobiology, 16:645-649, 2006.
2. Lang, CA, Bastian, AJ. Cerebellar damage impairs
automaticity of a recently practiced movement. J Neruophysiol, 87:
1336-1347, 2002.
3. Berent, Giordani, Gilman, Junck, Lehtinen, Markel, Boivin,
Kluin, Parks, Koeppe. Neuropsychological changes in
olivopontocerebellar atrophy. Arch Neurol, 1990 Sep:47(9): 997-1001
4. Maschke, Gomez, Ebner, Konczak. Hereditary cerebellar
ataxia progressively impairs force adaptation during goal-directed
arm movements. J Neurophysiol, 2004 Jan:91(1): 230-238.
5. Schmahmann, JD. Disorders of the cerebellum: ataxia,
dysmetria of thought, and the cerebellar cognitive affective
syndrome. J Neuropsychiatry Clin Neurosci 16:3, summer 2004.
6. Sanes, Dimitrov, Hallett. Motor learning in patients with
cerebellar dysfunction. Brain, Vol. 113, No. 1, 103-120, 1990.
7. Leggio, Tedesco, Chiricozzi, Clausi, Orsini, Molinari.
Cognitive sequencing impairment in patients with focal or atrophic
cerebellar damage. Brain 2008 131(5): 1332-1343.
8. Petrosini, L. “Do what I do: and “Do how I do”: Different
components of imitative learning are mediated by different neural
structures. The Neuroscientist, Vol. 13, No. 4, 335-348, 2007.
9. Boyden, Katoh, Raymond. Cerebellum-dependent learning: the
role of multiple plasticity mechanisms. Annual Review of
Neuroscience Vol. 27: 581-609, 2004.
10. Earhart GM, Bastian AJ. Selection and coordination of human
locomotor forms following cerebellar damage. J Neurophsyiol 85:
759-769, 2001.
11. Morton MM, Bastian AJ. Cerebellar contributions to locomotor
adaptations during splitbelt treadmill walking. The Journal of
Neuroscience 26(36): 9107-9116, Sep 2006.
12. Steinmetz JE. Psychological functions of the cerebellum.
Behavioral and Cognitive Neuroscience Reviews, Vol. 1, No.3,
229-241, 2002.
13. Steinlin, M. The cerebellum in cognitive processes:
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14. Ienaga, Mitoma, Kubota, Morita, Mizusawa. Dynamic imbalance
in gait ataxia. Characteristics of planter pressure measurements.
J Neurol Sci 2006 Jul 15;246(1-2): 53-57.
15. Topka, Valls-Sole, Massaquoi, Hallett. Deficit in classical
conditioning in patients with cerebellar degeneration. Brain 1993
Aug:116(part4): 961-969.
16. Porrill J, Dean P. Cerebellar motor learning: When is
cortical plasticity not enough? Comput Biol 2007 Oct: 3(10): e197.
17. Wolpaw JR, Chen XY. The cerebellum in maintenance of a motor
skill: A hierarchy of brain and spinal cord plasticity underlies
H-reflex conditioning. Learn Mem. 2006 March; 13(2): 208-215.
18. Ciedrichsen J, Criscimagna-Hemminger SE, Shadmehr R.
Dissociating timing and coordination as functions of the
cerebellum. J Neurosci. 2007 June 6: 27(23): 6291-6301.
19. Doyon, Song, Karni, Lalonde, Adams, Ungerleider.
Experience-dependent changes in cerebellar contributions to motor
sequence learning. Porc Natl Acad Sci U S A. 2002 January 22;
99(2): 1017-1022.
20. Lang CE, Bastian AJ. Cerebellar subjects show impaired
adaptation of anticipatory EMG during catching. J Neurophysiol. 82:
2108-2119, 1999.
21. Cerebellar Disorders. The Merck Manual, eighteenth edition,
2006; 1886-1887.
22. Leonard CT. The Neuroscience of Human Movement, 1998; 228.
23. Syed T Arshad, MD, Department of Neurology, Dartmouth
Hitchcock Medical Center; Kalpana Kari, MD, Department of Neurology,
Veterans Affairs Medical Center, Georgetown University; Yash
Mehndiratta, MD, Department of Neurology, Howard University
Hospital. Olivopontocerebellar Atrophy. eMedicine from WebMD,
Jan. 17, 2007.
24. Konarski, McIntyre, Grupp, Kennedy. J Psychiatry Neurosci.
2005 May; 30(3): 178-186.
Author’s
email:
tlcdoc@comcast.net
Author resides in
Florida, USA, is a former general surgeon, emergency room physician
and general practitioner. He was diagnosed with
Olivopontocerebellar Atropy in 1997 and subsequently found to have
the marker for Spinocerebellar Atrophy 14. In an effort to educate
those stricken with ataxia, from this and other diseases, he
provides information on his website
www.walkingwithataxia.com,
as well as providing workshops to this population base.
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