Ataxia, and a bit of Myoclonus.

I have a midterm tomorrow in my movement disorders class, I thought that you might like to hear a bit about ATAXIA.

It is a neurological disorder characterized by clumsiness (lack of coordination) in voluntary movement resulting from dysfunction of the cerebellum or cerebellar pathways.

Common clinical manifestations of ataxia are:

1. Stance and gait: staggering, clumsy walking with a wide-based stance. Patients have the most trouble with turning and stopping movements (basically, they look like they are drunk, in fact, acute and chronic alcohol abuse actually results in ataxia)

2. Limb coordination: 3 main problems here;

     a. asynergia = movements broken down into steps with no flow or                                  coordination

                    b. dysdiodochokinesis = problems with rapidly alternating movements

          c. dysmetria = abnormal movement excursions and difficulties in reaching a                  specific target

3. Tremor: tremor is associated with voluntary movements only

4. Muscle tone: hypertonia, which is a decreased resistance to passive limb displacement

5. Speech: dysarthria, which is clumsy, slurring, poorly modulated speech

6. Ocular motor function: fixation instability and involuntary saccades

Causes of ataxia vary; most cases are chronic progressive (appearing in infancy/adolescence or in late adulthood). But there are other causes:

Sporadic Ataxia

1. Degenerative: often caused my multiple system atrophy (MSA)

2. Stroke, tumor, or infection

3. Toxic/Metabolic: chronic or acute alcohol abuse or celiac disease (well, if people with celiac disease eat gluten, then they can get ataxia)

4. Autoimmune: MS, encephalomyelitis, or Guillian-Barre

Genetic Ataxia

1. Dominant

2. Recessive

3. X-linked

There is NO treatment for ataxia. All you can do is treat the symptoms through physical therapy, occupational therapy, or speech therapy. Freidreich Ataxia, which is the most common form of ataxia, is recessive and affects 1 in every 50 000 people.

Another fun fact (if you made it this far), which is not on my midterm, but that I learned in this class, is about a movement disorder called myoclonus.

Have you ever had this happen to you? 

You are drifting off to sleep, when all of the sudden, your leg kicks so violently that it wakes you up...

That is a movement disorder. It is called Physiological Myoclonus. However, it is nothing to worry about, it occurs in healthy individuals. It causes sleep jerks (called hypnic jerks), hiccoughs, and anxiety or exercise induced twitches/jerks.

Cool, hey?