FROM A PERSONAL AND PROFESSIONAL PERSPECTIVE
TODAY?
            Thomas L. Clouse, M.D.

                       Walking With Ataxia             

                           

TOMORROW!

 Home Page


 TLC to go!


Walking With Ataxia
and
Cerebellar Degeneration

article

 Personal Information
 
   About Me
     My Personal Journey
     Contact Me

 
Basic Overview
 
   Concepts to understand
     Basic problems and
         solutions

    Alert & Danger items
    Points to remember

The nitty gritty (text)
 
   Why do I have to work
          so damn hard?

     You are your enemy.    
     Believe you can.
     Let's begin.
     To move, to walk.
     Stepping out.
     Not shuffling.
     It's not just walking.
     Remember when.
     The dance.

  Comments from
     around the globe

 
    your voice, your story
      supportive letters
      emails
      articles of interest

 Disorder info
  
 detailed information for
     Ataxia defined
     Spinocerebellar Atrophy
   
 Spinocerebellar Atrophy (2)
    Ataxia
            Identified Genetic

         & Biochemical Defects

     Multiple System Atrophy
     Friedreich's Ataxia
     Parkinson's Disease


  click this banner to find information
          on other medical issues
1-1

 
Your personal health
         We can all be healthier
         Basic Nutrition
         Cellular Nutrition
         Oxidative Stress
        
 
Links to other sites

 

       

Walking With Ataxia and Cerebellar Degeneration

Thomas L. Clouse, M.D.
March 1, 2009

 

                A patient with cerebellar degeneration is clumsy; an irrefutable statement of fact.  However, I refute that these patients have to be clumsy, or as clumsy as they have become.  I further refute accepted medical dogma which states, and/or infers, “while physical therapy is beneficial, it has a very limited positive impact on the eventual degenerative progression of the physical manifestations of this disorder.” 13, 15, 21

 

                It is well known that every person afflicted with cerebellar degeneration will witness the demise of their independence secondary to a loss of coordination, balance and ambulatory capabilities as their progressively degenerative disorder continues.  Historically, it has been believed that these noted deficits are simply the physical manifestation of the cerebellar pathophysiology, and are therefore essentially unalterable.  This notion has been further reinforced by the failure rates within this patient population when attempted conventional therapeutic interventions do not achieve the anticipated outcomes of their design.  

 

However, one should argue, that while the rate of degeneration, and the affected area of the cerebellum, is variable depending on the etiology, the acquired abnormal postures, gaits and movements expressed by these patients are surprisingly similar when critically examined.  Thus, it should be postulated that perhaps some of these postures and movements may not be a direct result of the pathophysiology, but rather maladaptive learned behaviors.  From my perspective, the pathophysiology of this collective disorder has been given far too much credit, while in turn alleviating the patient’s responsibility.  To this end, I contend that medicine has failed the patient by “dismissing” half of the equation.  

 

For example, a 45 y.o. healthy male, diagnosed with sporadic Olivopontocerebellar Atrophy, later found to be Spinocerebellar Atrophy 14 (SCA 14), lived “per his pathology” for 8 years post the initial diagnosis while being subjected to accepted medical therapeutic modalities.  And as stated within the literature, his trials of Occupational and Physical Therapy (PT) interventions had little effect on the gradual decline of purposeful movement and neuromuscular control.  Being consistent with the accepted medical descriptors for this disorder, his gait continued to widen, ataxia worsened, falls became more frequent and his social isolation, depression and frustration levels all increased.  Subsequently, he also witnessed the loss of career and marriage, and was advised to begin using a walker for fall prevention measures. 

 

Two years post the recommendation that he begin using a walker, this individual began an unconventional approach in an effort to regain a portion of lost movement skills, and sought the assistance of a professional movement expert at a conventional ballroom dance studio; having had no previous dance experience or dance ambitions.  Without prior working knowledge of any neurodegenerative disease states, or any medical training, the veteran dancer offered basic movement and dance instruction after evaluation of his acquired maladaptive movements and posture, inherent abilities and personal goals. 

 

His ambulatory efforts on first entering the dance studio resembled that of a young toddler struggling with walking.  Thus, the initial sessions were laborious and frustrating, but later became increasingly productive.  Within a couple of months his gait began to narrow as his movement patterns and abilities began to “normalize”.  After six months of bi-weekly private lessons and a mixture of group lessons, he danced his first performance; a simple waltz routine.  Family and friends witnessing the transition applauded, but the medical community he attempted to share his experience with expressed doubt and a relative disbelief. 

 

Let’s interject an important point.  None of the doubting medical community witnessed the entire sequence of events.  Some knew him prior to this unconventional intervention, while others only knew him post the intervention.  In either case, all of the doubters were resistant to exploring this modality for possible general therapeutic applicability to this patient population.  Why?  Was it because this person didn’t fit the definition of reliability within their ranks?  Hardly, for as a competent M.D. himself, one would surmise credibility and believability within his peer group.  In reality however, the findings presented to them were inconsistent with the accepted medical beliefs and literature to which they have been exposed.

 

Medical literature correctly states that SCA, a progressive neurodegenerative condition, has no treatment or cure.  The literature further states that this condition is deemed “untreatable” as per conventional physical therapeutic modalities.  Query any medical professional with a good working knowledge of cerebellar degeneration and they will confirm to you that: “There really isn’t much we can offer these patients.”  So, are the results this patient experienced a fluke?  Perhaps he was misdiagnosed?  How is this possible given our understanding of this disorder? 

 

Perhaps all that is required is a modified form of PT which is yet to be accepted by the medical community?  Recent findings suggest this may be correct, for it is now known that the cerebellum plays a vital role in cognitive function.9, 16, 20  As a result, many patients with cerebellar atrophic degeneration have an acquired learning deficit whereby they demonstrate a Cognitive Sequencing Impairment 6 , as well as a Cognitive Affective Syndrome4, which hinders interpretation, mimicking and the eventual learning of new tasks. 1, 2, 3, 5, 10, 11, 12, 16, 17, 18, 19   Intuitively, this information allows us to rationalize that these patients will require some modifications to any attempted therapeutic intervention to accommodate for their acquired learning deficit. 7, 8, 11, 14, 16  Unfortunately, this valuable bit of information is not yet widely known as the findings are very recent.

 

The success of this patient’s unconventional therapeutic intervention was secondary to the inherent design of that intervention.  Begin with the premise that the instructor was unbiased by previous conceptions, and therefore projected all efforts to “treating the patient” without interjecting any perceived limitations of the “disease state”.  Building from the patient’s personal goal of wanting to regain fluidity of movement, coordinated purposeful movement and a relatively normalized walking pattern, an enabling set of parameters were utilized; and the instructor’s and student’s goals were consistent with the content of the instructions. The instructor adapted to the student’s learning abilities and patiently worked through the inherent frustrations.  Eventually, he was introduced to group activities within the studio and this further enhanced the learning process through positive peer support.  Four very important factors were present from the beginning: enablement, positive encouragement, an unbiased atmosphere conducive to learning and it became an enjoyable activity.

 

With a clear medical history and definitive diagnosis of SCA 14, this patient clearly demonstrates the treatability of cerebellar degeneration in the physical realm.  Further, it is also being shown that other patients with cerebellar degeneration are acquiring the ability to progress from cane or walker dependence to independent ambulation using a staged approach of: 1) addressing maladaptive compensatory behaviors, 2) building a repertoire of basic movements and postures, and 3) overcoming the fundamental motor sequencing impairment seen in cerebellar disease.  These results are yet to be found within medical literature, but they have been witnessed by many as this basic understanding has evolved within the “grass roots” of this patient population.  Achieving a basic understanding of the rationale for the maladaptive postures and movements has led to a basic understanding for formation of corrective techniques and concepts which have shown to be very effective when applied in uncontrolled patient volunteers. 

 

The importance of this is paramount to this patient population who has been without hope of any kind.  Clearly, this “understanding” must be brought into the mainstream of medicine, scrutinized through controlled studies to provide validation, and disseminated as quickly as our system will allow so the desperate individuals suffering with cerebellar degeneration, as well as other disorders which may benefit, may finally hear their physician say, “There is something we can offer you that will help!”

 

References:

 

1.       Lang, CA, Bastian, AJ.  Learning to predict the future: the cerebellum adapts feed forward 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.       Maschke, Gomez, Ebner, Konczak.  Hereditary cerebellar ataxia progressively impairs force adaptation during goal-directed arm movements.  J Neurophysiol, 2004 Jan:91(1): 230-238.

 

4.       Schmahmann, JD.  Disorders of the cerebellum: ataxia, dysmetria of thought, and the cerebellar cognitive affective syndrome.  J Neuropsychiatry Clin Neurosci 16:3, summer 2004.

 

 5.       Sanes, Dimitrov, Hallett.  Motor learning in patients with cerebellar dysfunction.  Brain, Vol. 113, No. 1, 103-120, 1990.

6.       Leggio, Tedesco, Chiricozzi, Clausi, Orsini, Molinari.  Cognitive sequencing impairment in patients with focal or atrophic cerebellar damage.  Brain 2008 131(5): 1332-1343.

7.       Boyden, Katoh, Raymond.  Cerebellum-dependent learning: the role of multiple plasticity mechanisms.  Annual Review of Neuroscience Vol. 27: 581-609, 2004.

8.   Morton MM, Bastian AJ.  Cerebellar contributions to locomotor adaptations during splitbelt treadmill walking.  The Journal of Neuroscience 26(36): 9107-9116, Sep 2006.

9.   Steinlin, M.  The cerebellum in cognitive processes: supporting studies in children.  Cerebellum 2007:6(3): 237-241.

10.   Porrill J, Dean P.  Cerebellar motor learning: When is cortical plasticity not enough?  Comput Biol  2007 Oct: 3(10): e197.

11.   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.

12.   Lang CE, Bastian AJ.  Cerebellar subjects show impaired adaptation of anticipatory EMG during catching.  J Neurophysiol. 82: 2108-2119, 1999.

13.   Cerebellar Disorders.  The Merck Manual, eighteenth edition, 2006; 1886-1887.

14.   Leonard CT.  The Neuroscience of Human Movement, 1998; 228.

15.   Arshad, Syed T, Kari, Kalpana,  Mehndiratta, Yash.
Olivopontocerebellar Atrophy.  eMedicine from WebMD,  Jan. 17, 2007. Dartmouth Hitchcock Medical Center; Veterans Affairs Medical Center, Georgetown University; Howard University Hospital. 


16.  Krakauer, Shadmehr. Consolidation of Motor Memory. Trends Neurosci. 2006 January; 29(1): 58-64.

 

17.  Ebner, Pasalar. Cerebellum Predicts the Future Motor State. Cerebellum. 2008 Oct 1 1

 

18.  McGibben, Krebs, Wagenaar. Stepping stability: effects of sensory perturbation. J NeuroEngineering and Rehab. 2005, 2: 9

 

19.  Ilg, Golla, Their, Giese. Specific influences of cerebellar dysfunctions on gait. Brain. 2007 Mar; 130(Pt 3): 786-98.  Epub 2007 Feb 7.

 

20.  Shadmehr, Krakauer.   A Computational Neuroanatomy for Motor Control.  Exp. Brain Res. 2008 March: 185(3): 359-381.


21. 
Azevedo, Christina J,  Berman, Stephen A.  Olivopontocerebellar Atrophy;  eMedicine, Department of Neurology, Dartmouth-Hitchcock Medical Center, White River Junction Veterans Medical Center.  Updated: Feb 12, 2009